CARDIOVASCULAR

SYSTEM

High-Yield
NOTES
by
Medicalstudyzone.com

PATHOLOGY
Table of contents
Acute Coronary Syndromes 1
Myocardial Infarction (MI) 2
Prinzmetal’s Angina 4
Unstable Angina 5
Acyanotic Defects 6
Atrial Septal Defects (ASD) 7
Coarctation of Aorta 9
Patent Ductus Arteriosus (PDA) 11
Ventricular Septal Defect (VSD) 13
Aneurysms & Dissection 15
Aneurysms 15
Aortic Dissection 19
Bradycardia & Heart Block 21
Atrioventricular Block 22
Bundle Branch Block 24
Sick Sinus Syndrome 29
Cardiac Tumors 29
Atrial Myxoma 30
Rhabdomyoma 31
Cardiomyopathy 33
Dilated Cardiomyopathy 34
Hypertrophic Cardiomyopathy (HCM) 36
Restrictive Cardiomyopathy 38
Cardiomyopathy Types 40
Stable Angina Pectoris 41
Cyanotic Defects 43
Hypoplastic Left Heart Syndrome 44
Persistant Truncus Arteriosus 45
Tetralogy of Fallot 48
Total Anamolus Pulmonary Venous 50
Transposition of The Great Vessels 51
Cyanotic Heart Defect Overview 54
Heart Failure 55
Classification of Heart Failure 56
Cor Pulmonale 58
Diastolic Heart Failure 59
Left Heart Failure 60
Right Heart Failure 62
Heart Valve Disease 64
Aortic Insufficiency 65
Aortic Stenosis 66
Mitral Insufficiency 68
Mitral Stenosis 70
Mitral Valve Prolapse 72
Pulmonary Insufficiency 73
Plumonary Stenosis 74
Tricuspid Insufficiency 75
Tricuspid Stenosis 76
Valvular Murmus Chars. 78
Hypertension & Hypotension 79
Hypertension 80
Hypertensive Emergency 83
Hypotension 84
Infections & Inflammation of the Heart 85
Infective Endocarditis 86
Libman-Sacks Endocarditis 89
Myocarditis 90
Rheumatic Fever 92
Lymphatic Dysfunction 95
Lymphedema 95
Pericardial Disease 97
Acute Pericarditis 98
Cardiac Tamponade 100
Constructive Pericarditis 102
Dressler’s Syndrome 103
Pericardial Effusion 104
Peripheral Artery Disease 105
Arteriosclerosis 107
Atherosclerosis 108
PAD vs. PVD 110
Table of contents
Pre-excitation Disorders 111
AV Reentrant Tachycardia (AVRT) 112
Wolff-Parkinson-White Syndrome 114
Premature Contraction 117
Premature Atrial Contraction 118
Premature Ventricular Contraction 121
Shock 122
Sudden Cardiac Death 127
Brugada Syndrome 127
Pulseless Electric Activity 129
Ventricular Fibrillation 130
Supraventricular Tachycardia 133
Atrial Fibrillation 134
Atrial Flutter 136
Atriventricular Nodal Reentrant Tachycardia
(AVNRT) 139
Vascular Compression Syndromes 141
Nutcracker Syndrome 142
Superior Mesenteric Artery Syndrome 143
Vasculitis 162
Vasculitides Overview 163
Bechcet’s Disease 164
Buerger’s Disease 165
Churg-Strauss Symdrome 166
Giant Cell Arteritis 167
Granulomatosis with Polyangitis 168
Henoch-Schönlein purpura (HSP) 170
Kawasaki Disease 171
Microscopic Polyangiitis 172
Polyarteritis Nodosa 173
Takayasu Arteritis 174
Venous Dysfunction 176
Chronic Venous Insufficiency (CVI) 177
Deep Vein Thrombosis (DVT) 179
Well’s Score 180
Thrombophlebitis 181
Varicose Veins 183
Ventricular Tachycardia 185
Long QT Syndrome (LQTS) 187
Torsades des Pointes (TdP) 190
Ventricular Tachycardia 192

Vascular Malformations 145

Arteriovenous Malformation 146
Cavernous Hemangioma 147
Hemangioma 149
Lymphangioma 150

Vascular Steal Syndromes 153

Coronary Steal Syndrome 154
Subclavian Steal Syndrome 155

Vascular Tumors 157

Angiosarcoma 157
Glomus Tumor 159
Kaposi Sarcoma 160
 NOTES

NOTES
• ACUTE CORONARY SYNDROMES

GENERALLY, WHAT ARE THEY?

( PATHOLOGY & CAUSES ) ( T_R_EA_:T'_M_EN_T
__ )
• Signs and symptoms caused by decreased MEDICATIONS
blood flow in the coronary arteries to the • Oxygen as needed
extent that the muscle cannot function • Pain control with nitrates and/or morphine
properly, or even dies
• Antiplatelets
• Acute coronary syndromes are almost
• Anticoagulants
always due to atherosclerosis
• Nitrates: decreases preload by vasodilation
• Beta blockers: reduces cardiac demand by
(__ SI_G_NS_&_S_Y_M_PT_O_M_s_) decreasing heart rate, BP and contractility
(first-line choice: metoprolol)
• See individual disorders • Statins: HMG-CoA reductase inhibitor
that reduces production of cholesterol to
improve lipid profile
( D_IA_G_N_OS_IS)
OTHER INTERVENTIONS
• See individual disorders
• Hospital admission with continuous
monitoring
• Reestablish blood flow via catheterization I
rev a scu I a ri zatio n
• Lifestyle changes: improve diet (reduce
intake of saturated fat), smoking cessation,
control blood pressure, strict management
of diabetes mellitus, increase exercise,
weight loss, improve lipid profile

PAV-111\\.
l I
I TP-A~SMVJ.AL \WfMt<.T
1SugE.wDOLA~O\~l
\ t-JFA~l"T ST£M \" ST-se.5~e.r\~ e.\e.vG.~0"'
..iSlllll\ \ ,._ 'no"' -CF.l -S~~e ... 1 ~\e110.l.:io"' 'M '!oc.on.l.10.\ \Y'fa,c.t1'0"'-
""~oloro-!u\ 1~1.1rtti OV\

tLu

Figure 1.1 Illustration depicting ST depression and ST elevation seen in myocardial infarctions .

1
 afratafreeh.com exclusive

MYOCARDIAL INFARCTION (Ml)

osms.i"l/ m14oeo.Tdio.l-info.Te-lion
( PATHOLOGY & CAUSES
• Death of heart muscle cells due to lack of
oxygen-rich blood flow
• Plaque buildup (fat, cholesterol, proteins,
calcium, white blood cells) takes years to
form in lumen
• Blood platelets adhere to plaque and
enhance clotting process, creating
blockage
• Necrosis of myocardial cells follows series
of events
0 < 24 hrs: early coagulativenecrosis, cell
debris in blood, edema, wavy fibers and
hemorrhage
0 1-3 days: extensive necrosis, tissue has
acute inflammation with neutrophils
0 3-14 days: macrophages and
granulation tissue in margins
0 > 14 days: contracted scar forms
COMPLICATIONS
) • Heart failure: heart muscle fails to
compensate for damage; risk related to
size/territory of infarct and individual's
baseline cardiac function
• Cardiac arrhythmia: may be seen
within minutes after Ml or years later. If
undiagnosed Ml, may be cause of death
• Left ventricular (LV) failure and pulmonary
edema: happens after left ventricular
infarction, free wall rupture, ventricular
septal defect, papillary muscle rupture with
mitral regurgitation
• Postinfarction pericarditis, papillary muscle
rupture (might lead to acute, severe mitral
regurgitation), interventricular septal
rupture, ventricular pseudoaneurysm
formation, ventricular free wall rupture
(might lead to ventricular free wall rupture
leading to pericardia! tamponade/ventricular
pseudoaneurysm), true ventricular
aneurysm, Dressler syndrome

TYPES
\ MNEMONIC.: DARTH VADER
ST segment elevation myocardial infarction
(STEMI) Complication
s of Ml

• Coronary artery completely blocked; full Death

thickness of myocardial wall involved Arrythmia
• ECG shows ST elevation, possible Q waves Rupture (free ventricular wall/
ventricular septum/papillary
Non-ST segment elevation Ml (NSTEMI) muscles)
• Coronary artery not completely blocked, Tamponade
subendocardium may be especially Heart failure (acute or chronic)
vulnerable to ischemia
Valve disease
• ECG shows ST depression Aneurysm of ventricles
Dressler's syndrome
RISI( FACTORS thromboEmbolism (mural
• Modifiable risk factors: older age, smoking, thrombus)
high blood pressure, diabetes mellitus, high Recurrence/ mitral
cholesterol, low levels of physical activity, Reg u rg itatio n
obesity, excessive alcohol use, illegal drug
use (e.g., cocaine, amphetamines), chronic
stress
• Non-modifiable risk factors: family history,
biological male

2
(__ SI_G_NS_&_S_Y_M_PT_O_M_s
) ( T_R_E~_iT_M_EN_T__
• Acute chest pain lasting > 20 min,
radiating to arm/jaw
• Uncomfortable chest/back/neck/
jaw/stomach pain, dyspnea, fatigue,
diaphoresis, and/or nausea
• Feeling of fullness/indigestion
)
• STEMI first priority: emergent reperfusion
via percutaneous coronary intervention (e.g.
catheterization)/throm bolysis
O Very time sensitive
• NSTEMI: reperfusion via percutaneous
coronary intervention (not thrombolysis)
O Less time sensitive than in STEMI

( D_IA_GN_O_s,_s) MEDICATIONS
• Heparin, aspirin + clopidogrel, beta
LAB RESULTS
blockers, ACE inhibitors, statins
• Usually detected with diagnostic laboratory
• Control symptoms with morphine and
work for cardiac enzymes
nitrog lyce ri n
O Troponin I, troponin T most specific,
sensitive markers: rise apparent within
2-4 hrs, peaking -24 hrs
° CK-MB can detect reinfarction after
initial Ml: levels increased 4 hrs after
infarction, peak at 24 hrs, return to
normal after 48 hrs

OTHER DIAGNOSTICS
ECG
• Can confirm diagnosis; time sensitive, not
accurate after 6 hours
• < 30 min: ST segment elevation
, Only seen in STEM ls Figure 1.3 Gross pathology of a ruptured
, ST depression/no ST segment deviation papillary muscle, a serious complication of
would be seen in NSTEMls myocardial infarction.
• < 24 hrs: T wave inversion
• > 24 hrs: Q waves appear

Figure 1.4 Histological

appearance of the
myocardium following a myocardial infarct.
Figure 1.2 A pathology pot containing a heart
exhibiting an anterior myocardial infarction,
usually the result of left anterior descending
artery disease.

3
 PRINZMETAL'S ANGINA
osms.i"l/ Tinzme-1:o.ls-o.ngino.

( PATHOLOGY & CAUSES ) ( T_R_EA_~_M_EN_T

)
• AKA vasospastic angina/variant angina MEDICATIONS
Calcium channel blockers
CAUSES
• Coronary artery vasospasms; occur Vasodilators
spontaneously even at rest • Increases coronary blood flow to heart
• Vasospasms: constriction of the smooth
muscle surrounding the artery, reducing
blood flow through the vessel
° Cause of vasospasms not
well understood; likely due to
LOCATION OF
vasoconstrictors such as platelet STEMI IN ECG
thromboxane A2
° Coronary artery vasospasms cause INFARCT LEADS WITH ST
ischemia throughout all of the heart ELEVATION OR
LOCATION Q WAVES
layers

ANTEROSEPTAL Vl -V2
(..___SI_G_NS_&_SY_M_PT_O_M_s
) (LAD)

• Same as stable angina, except pain may ANTEROAPIC.AL V3 -V4

occur at rest (DISTAL LAD)
• Pain described as pressure, squeezing,
burning, or tightness; can radiate to the ANTEROLATERAL VS-V6
either/both arms, jaw, shoulders, and back; (LAD o" LC.X)
lasts less than 20 minutes
• Other symptoms: Levine's sign (a clenched LATERAL(LC.X) I - aVL
fist held over the chest), dyspnea,
diaphoresis, fatigue, nausea, and epigastric INFERIOR(LC.X) II, Ill, aVL
pain
V7-V9,
ST depression in
( D_IA_GN_o_s,_s) POSTERIOR(PDA) Vl-V3 with tall
R waves
DIAGNOSTIC IMAGING
• Transient ST segment elevation
• Illustrates transmural ischemia

4
 UNSTABLE ANGINA
osms.i"l/unno.ble-o.ngino.

( PATHOLOGY & CAUSES ) ( D_IA_GN_O_s,_s

)
• Episodic chest pain that either LAB RESULTS
O Is new in onset • Serial troponins measured for individuals
O Occurs at rest unpredictably with unstable angina to rule out myocardial
infarction
O Rapidly worsens over time
• Troponin released after injury to myocytes
O Occurs within 48 hrs after acute Ml
is a marker for myocardial injury
• Usually caused by ruptured atherosclerotic
plaque - formation of thrombosis on top
of plaque - almost complete blockage in OTHER DIAGNOSTICS
blood vessel - ischemia - pain
ECG
• Medical emergency: high risk of
progression to Ml • Can present with ST segment depression
O Angina: myocytes still alive , May also present with T wave
inversions
O Myocardial infarction: death of
myocytes , Illustrates subendocardial ischemia

(__ S_IG_N_S_&_S_YM_P_T_O_M_s__.) ( T_R_E~_~_M_EN_T

)
• Unstable angina. NSTEMls are
• Same as stable angina, except pain may
indistinguishable at initial evaluation
occur at rest
• Elevated troponins indicating myocardial
• Pain described as pressure, squeezing,
infarction not detectable for hours
burning, or tightness; can radiate to the
either/both arms, jaw, shoulders, and back; • Initial management identical
lasts less than 20 minutes
• Other symptoms: Levine's sign (a clenched MEDICATIONS
fist held over the chest), dyspnea,
diaphoresis, fatigue, nausea, and epigastric Clopidogrel
pain
Low-molecular-weight-heparin (LMWH)
• Prevents clot formation

Enoxaparin
• Drug of choice based on empirical evidence

5
NOTES

- NOTES
•
-
ACYANOTIC DEFECTS
GENERALLY, WHAT ARE THEY?
Eisenmenger syndrome
( PATHOLOGY & CAUSES ) • At rest: asymptomatic
• With exertion: cyanosis, palpitations
• Heart defects presenting without cyanosis
dyspnea, chest pain, syncope
(blue-tinged skin)
• Caused by fetal heart malformation, can
lead to heart failure
(..____ D_IA_GN_o_s,_s )
• ASD, PDA, and VSD
O All three cause left-to right shunt e- DIAGNOSTIC IMAGING
oxygenated blood flows redundantly • Heart imaging to identify defect type
through pulmonary clrculatton=-.
becomes Eisenmenger syndrome over
time (..____ T_R_EA_~_M_EN_T
)
SURGERY
(__ s,_G_NS_&_SY_M_PT_O_M_s ) • Rarely

• Sometimes asymptomatic, but can lead to

heart failure, Eisenmenger syndrome MNEMONIC: P(C)AV
Acyanotic defects
Heart failure
Patent ductus arteriosus
• Infants: poor feeding/failure to thrive,
fluid retention, pulmonary congestion, (Coarctation of the aorta): no
hepatomegaly, respiratory distress, shunt
elevated jugular venous pressure Atrial septal defect
Ventricular septal defect

6
 Oi!i~eN\a,4.eo..
f;...... &\.000
~(OM
BOO'# l.U)J~~
III\Ur4St.c.A.
8LaoS> Vo\.Ua. l:.
i
Pu.l~O NA~ \hPE:tT~MIO~
4 Hl~Hl~ p1cs<:>vve
L ElSEtJM~tJC':al~
S'ft-.JO~o..._~
SMa.\l V5D
L YVIO'ct- 'o, o.S?p+CHMQtJ(.

LQ<jt-...- V~Ds
L C,.~ptoill!> mO(t SCWC.ft.
L ?<t~V\t e.arl1e.r

Figure 2.1 Illustration of blood flow through a ventricular septa I defect.

ATRIAL SEPTAL DEFECT (ASD)

osmsJl/ e1-lrie1l-sep-le1l-cJ.ef ee-l

( PATHOLOGY & CAUSES )

• A hole in the heart wall dividing left/right
atria (left-to-right shunt)
• Blood passes through pulmonary
circulation redundantly

(__ SI_G_NS_&_S_Y_M_PT_O_M_s_)
• Fixed, split S2 and pulmonic ejection
murmur (louder with age)
• Infants and children
O Respiratory infections
° Failure to thrive
• Adults (before 40) Figure 2.2 CT scan in the axial plane showing
O Palpitations, exercise intolerance, an atrial septa I defect. Note the faint contrast
dyspnea, fatigue plume as blood flows from the high pressure
left system to the low pressure right system.
RA; right atrium. LA; left atrium. RV; right
ventricle. LV; left ventricle.
7
(..____ D_IA_GN_O_SI_S
)
DIAGNOSTIC IMAGING
Chest X-ray
• Right heart dilation
• Prominent pulmonary vascularity

Transesophageal echocardiography
• Visualize size & location accurately

SURGERY
Figure 2.3 lntraoperative view of multiple,
Right heart catheterization pinhole atrial septal defects.
• Increased oxygen saturation in:
O Right atrium
O Right ventricle ( T_R_EA_:l"_M_EN_T
)
O Pulmonary artery
SURGERY
• Percutaneous surgical closure
• Adults: surgery in cases of
, Right ventricular enlargement,
paradoxical embolism, right-to-left
shunt

Atf2.\AL S&PlAl
OtfE.CT (PiSO)
AC.YAtJOl''-
J ~'t>\u.t.
t
~ B\..ooD
(olto1,,uu.+td.)""
(ol't\plicatl'o~:
to2.~~o~ e..iMloo\ \SM
• v-13-.,t "'~' ilAV\11 (",;A..,.r. ~, ...')
• vi~'<lt vevltflc..\e.
• ~l)..\MOYIO~

"'~
~+ro. ElOOO
i
clt\Qtd. \)IA\l'W\OW\it
'1o\11t Gl~
(s?~~ S1 sou.rid..)

Figure 2.4 Illustration depecting blood shunting from left to right atrium in atrial septa I defect.

8
 COARCTATIONOF THE AORTA
(CoA)
osms.i"l/eoo.,-e-lo.-lion-of--l he-o.o,--lo.
compared to lower extremities
( PATHOLOGY & CAUSES ) • Secondary hypertension
• Severe heart failure, shock if/when PDA
• Narrowed segment of aorta
closes
• Upstream issues
• Other symptoms may more apparent with
O Blood flow increases into aortic
age
branches before coarctation - blood
• Chest pain, cold extremities, claudication
flow, pressure increases in upper
on exertion
extremities, head
• Left ventricular impulse palpable,
• Downstream issues
sustained
O Decreased blood flow, decreased
• Pulsations felt in intercostal spaces
pressure in lower extremities
° Kidneys receive less blood - activate Adults
renin-angiotensin-aldosterone system • Hypertension (most common)
(RAAS) - secondary hypertension • Hypotension in lower extremities
• Preductal coarctation • Bilateral lower extremity claudication
, Associated with Turner syndrome, PDA • Dyspnea on exertion
, May go unnoticed unless severe. • Delayed/weak femoral pulses
Presents as postductal coarctation
• Postductal coarctation
, Distal to ligamentum arteriosum ( D_IA_GN_O_SI_S
__ )
, Presents in adulthood
, Blood pressure higher upstream. lower DIAGNOSTIC IMAGING
downstream
Angiogram
, Autoregulatory vasoconstriction/
• Visualize narrowing in aorta, anatomy &
vasodilation preserves regional blood
severity
flow
Chest X-ray
• Rib notching: 3-sign (narrowed aorta
(__ s,_G_NS_&_S_Y_M_PT_O_M_s_)
resembles notch of number 3 due to
prestenotic of aortic arch & postenotic of
• Depends on presence/severity of PDA descending aorta dilatation)
• Systolic murmur
O Systole: diamond-shaped murmur Echocardiograph
O Diastole: high-pitched decrescendo • Visualize location, size, blood turbulance
murmur
OTHER DIAGNOSTICS
Infants
• Lower extremity cyanosis ECG
• Absent or delayed femoral pulse • Left ventricular hypertrophy, left atrial
• Failure to thrive/poor feeding enlargement
• Blood pressure higher in upper extremities

9
 afratafreeh.com exclusive

( T_R_EA_:T_M_EN_T__ )
MEDICATIONS
Prostaglandin E
• Increases flow to lower extremities

SURGERY
• Resection with end-to-end anastomosis
O If unfeasible, bypass graft across area of
coarctation
• Long-segment coarctation: subclavian
aortoplasty Figure 2.5 Illustration showing narrowing of
• Prosthetic patch aortoplasty (rarely) aorta lumen.
• Balloon angioplasty with possible stent

Figure 2.7 A chest radiograph demonstrating

the figure of three sign seen in coarctation of
the aorta.
Figure 2.6 CT scan in the sagittal plane
demonstrating coarctation of the aorta.

10
 PATENT DUCTUS ARTERIOSUS
(PDA)
osms.i"l/ o.-le n-l-due-lus-c11·-leTiosus

( PATHOLOGY & CAUSES ) ( D_IA_GN_o_s,_s __ )

• Ductus arteriosus remains open after birth DIAGNOSTIC IMAGING
• Left-to-right shunt between atria
Echocardiograph
• Sometimes presents with congenital
• 20 suprasternal echocardiogram
defects (congenital rubella syndrome)
Chest X-ray
CAUSES • Normal/cardiomegaly

Congenital rubella
• Mother-fetal transmission of rubella in first
OTHER DIAGNOSTICS
tnmester=- cytopathic damage to blood ECG
vessels, ischemia to organs
• Left ventricular hypertrophy, left atrial
• Prematurity enlargement
• Perinatal distress, hypoxia

( T_R_E~_~_M_EN_T
__ )
(__ SI_G_NS_&_S_Y_M_PT_O_M_s
)
• Small asymptomatic PDA: monitor
Depend on size of PDA
• Smaller
MEDICATIONS
O Usually asymptomatic
O Neonates: holosystolic "machine-line" Neonates (10-14 days)
murmur on auscultation • Close PDA using prostaglandin inhibitor
O Infants, children, adults: continuous
Symptomatic moderate/large PDA
murmur
• During heart failure
• Moderate
• Digoxin, furosemide
O Exercise intolerance
° Continuous murmur
O Wide systemic pulse pressure SURGERY
O Displaced ventricular apex Symptomatic moderate/large PDA
• Larger • Closure recommended for symptoms of
O Infants: leads to heart failure left-to-right shunting, left-sided volume
° Children: shortness of breath, overload, reversible pulmonary arterial
fatigability, Eisenmenger syndrome hypertension
° Children< 5kg/111bs: surgical ligation
0 > 5kg/111bs (including adolescents/
adults): percutaneous occlusion, surgical
ligation for large PDA

11
 P~iE.tJf OUCTUS A~Tt.~\OSlJS

Ao'2TA
L O.Oftil

Figure 2.8 Illustration depicting location of a patent ductus arteriosus.

Figure 2.9 Volume-rendered CT scan of the

heart and great vessels showing a patent
ductus arteriosus.

12
 VENTRICULAR SEPTAL DEFECT
(VSD)
osmsJl/ ven-lTieulo.T-sep-lo.1-defee-l

( PATHOLOGY & CAUSES ) (..____ D_IA_GN_o_s,_s __ )

• Left-to-right shunt between ventricles DIAGNOSTIC IMAGING
• Most common congenital heart disease
Chest X-ray
• Left-to-right shunt between ventricles
• Unreliable; may indicate left atrial
• Often presents with other defects (e.g. enlargement, right ventricular hypertrophy,
tetralogy of Fallot) left ventricular hypertrophy, or pulmonary
artery enlargement
Size of defect
• Small: restrictive Echocardiogram
O Normal pressure maintained between • Determines location and size
ventricles
• Moderate or large: non-restrictive MRI
O No pressure difference between • Use if echo does not diagnose
ventricles
SURGERY

( SIGNS & SYMPTOMS ) Cardiac catheterization

• Used if echo and MRI did not diagnose, but
individual still has pulmonary hypertension
• Asymptomatic in utero
• Holosystolic murmur (loud, high-pitched)
located at lower left sternal border OTHER INTERVENTIONS
Size of defect ECG
• Small: asymptomatic, murmur • Left ventricular hypertrophy

• Moderate-large: sweating, poor feeding/ • May see right ventricular hypertrophy;

failure to thrive, respiratory infections. left, right atrial enlargement (may see
Murmur plus thrill, and diastolic rumble in Katz-Wachtel phenomenon)
mitral area
O Signs of congestive heart failure
(dyspnea, persistent cough, pulmonary
vascular resistance)
O Eisenmenger's syndrome

13
(..____ T_R_EA_:l'_M_EN_T
)
• Most small VSDs close on their own

SURGERY
• Repair larger shunts by age 2 to prevent
pulmonary hypertension

Surgical repair
• Patch closure over ventricular septal defect
(preferred treatment)

Transcatheter closure
• Mesh to close VSD (higher risk)

Hybrid procedure
Figure 2.10 View of the right side of the heart
showing multiple ventricular septal defects.

Figure 2.11 Doppler ultrasound scan demonstrating flow of blood across the interventricular
septum in a VSD.

14

- NOTES
•
~
ANEURYSMS & DISSECTION
ANEURYSMS
osmsJl/ o.neu,-14ms
( PATHOLOGY & CAUSES
• Abnormal dilations in blood vessel; 1.5x
larger than normal vessel diameter (> 3.0
cm in aortic and thoracic)
• Frequently occurs in areas of high blood
pressure: aorta, femoral, iliac, popliteal,
and cerebral arteries; can occur in veins
(uncommon). Pressure on blood vessel
walls increases with diameter of vessel
lumen (LaPlace's law)
• 60% of true aortic aneurysms occur in
abdominal aorta, 40% in thoracic aorta;
most between renal artery branch and
aortic bifurcation due to less collagen in this
area of aorta
Locations
• Can occur in any blood vessel; particularly
life-threatening in the following locations
• Abdominal aortic aneurysm (AAA)
O Localized in abdominal aorta (diameter
> 3cm/1.12in or> 50% larger than
normal)
° Caused by atherosclerosis, infection.
trauma, arteritis, cystic medial necrosis
• Thoracic aortic aneurysm
O Localized in thoracic aorta. Less
common than abdominal aortic
aneurysm
• Cerebral aneurysms
O Located in brain; particularly threatening
in circle of Willis
NOTES
TYPES
)
True aneurysms
• All layers of blood vessel wall dilate
° Fusiform aneurysms: blood vessel walls
dilate symmetrically
O Saccular (berry) aneurysms:
asymmetrical ballooning of blood vessel
walls due to increased blood pressure
on one side of blood vessel wall
Pseudoaneurysms
• Small hole in blood vessel wall---> blood
leaks out, pools; resembles fusiform/
saccular aneurysm
CAUSES
lschemia
• lschemia of arteries with vasa vasorum:
hyaline arteriolosclerosis decreases blood
to large artery walls; decreases smooth
muscle in arterial tunica media
• lschemia of arteries without vasa vasorum:
plaque from atherosclerosis blocks blood
vessel walls from receiving oxygen
Infection
• Tertiary syphilis: causes inflammation of
tunica inti ma of vasa vasorum, decreasing
blood to arterial wall in thoracic artery
(endarteritis obliterans)
• Mycotic aneurysms: secondary infection in
intracranial arteries/visceral arteries/arteries
of extremities (bacteria enters vessel wall,
weakening it)
O Pathogens include: Bacteroides fragilis,
Pseudomonas aeruginosa, Salmonella
15
 "FtiLSE"
~
T(tU£ ANEUltYS""
•••••Y•••• ,,,,,..,....,,. f'su£00AtJEU~'ISM
,/;'-ml ICljers~
t,\JLGt

I
~~~l"lrtlft'tto\
SACCULAP. (or 'l>E.M-V)
Figure 3.1 Illustration depicting differences between types of aneurysms.

species, Aspergillus, Candida, aorta, inferior vena cava/duodenum,

Mucor (also an infective endocarditis respectively)
complication) • Thoracic aortic aneurysm
, Dissection, rupture (bleeding into
Genetic
thoracic cavity)
• Connective tissue disorders: Marfan's
• Cerebral aneurysm
syndrome, Ehlers-Danlos syndrome
, Rupture (leads to hemorrhagic stroke or
subarachnoid hemorrhage)
RISI( FACTORS , If large, aneurysm can place pressure
• White biologically-male individuals on surrounding cerebral tissue, causing
of European descent, advanced age, neurological symptoms
smoking, hyperlipidemia, hypertension,
family history, Ehlers-Danlos syndrome,
Marfan syndrome, syphilis, cystic medial (__ SI_G_NS_&_S_Y_M_PT_O_M_
)
degeneration, bicuspid aortic valve
• Asymptomatic until rupture: severe pain
COMPLICATIONS in specific location (abdomen, chest, lower
back, groin), pulsating mass, hypotension,
• High mortality rates
syncope
• Rupture: internal exsanguination; increased
intracranial pressure (if in brain) • Abdominal aortic aneurysm
• Compression to surrounding structures:
O On rupture: pain in abdomen/back,
superior vena cava syndrome, aortic pulsating sensation in abdomen, low
insufficiency blood pressure, syncope

• Thrombosis/emboli: stagnant blood in extra

O Large aneurysms felt by pushing on
lumen space abdomen

• Abdominal aortic aneurysm

O Rupture (bleeding can be retroperitoneal
or into abdominal cavity), acute aortic
occlusion, aortocaval/aortoduodenal
fistulae (connections between

16
 • Repair methods
( D_IA_GN_O_s,_s) O Surgical dipping: aneurysm clipped at
base
DIAGNOSTIC IMAGING O Endovascular coiling: platinum wires
Ultrasound promote blood clotting, decrease blood
• Confirms presence, location, size; monitors flow through aneurysm
growth O Endovascular stenting: wire stent
inside aneurysm allows blood to bypass
CT scan aneurysm
• Accurately measures; used pre-surgery

CTA scan OTHER INTERVENTIONS

• CT scan + injecting contrast dye shows • Goals, initially
blood flow; used for surgery O Prevent aneurysm rupture with regular
ultrasound monitoring
• Goals for individuals receiving surgery for
OTHER DIAGNOSTICS aneurysm
ECG O Maintain tissue perfusion, motor and
• Rules out myocardial infarction sensory function, prevent complications,
i.e. infection/thrombosis
• Goals for post-operative individuals
( T_R_E~_;,-_M_EN_T
) O Maintain blood pressure/perfusion,
especially renal perfusion
MEDICATIONS O Monitor urine output, peripheral
• Pharmaceutical treatments for blood pulses, capillary refill, skin temperature,
pressure management abdominal girth, intra abdominal
pressure, limb sensation and movement
• Monitor stent/graft patency
SURGERY
• Indications: aneurysms> 5cm/1.96in,
0.5cm/0.2in growth in six months,
individual symptomatic

C.AllSE
,.,,,,,,..,..,,.& .. - we.o.\o,ass in ves~\ wo.11
~ foSIT\VE fEE06f'Gll

LMLI\C£5\
I

r f . ~ Loop
LAW '-1.. t DI AME.TE.P. = t PJESSOP.E
BALLOON
~

Figure 3.2 Illustration depicting Laplace's law. Increasing diameter increases pressure on the
walls of blood vessel. Similar to how a balloon becomes easier to fill with air as it inflates.

17
Figure 3.3 A CT scan of the head in the left
parasagittal plane demonstrating a saccular
aneurysm of the internal carotid artery.
Figure 3.5 A CT scan of the chest in the
coronal plane demonstrating a massive
thoracic aortic aneurysm involving the
ascending aorta. The aortic valve is faintly
visible.

Figure 3.4 Abdominal CT scan in the axial plane demonstrating a ruptured abdominal aortic
aneurysm.

18
 AORTIC DISSECTION
osmsJl/ e10T-l.ie_dissee-l.ion
( PATHOLOGY & CAUSES )
PATHOLOGY
• Tearing/widening of artery's internal layer,
followed by blood entering vessel wall,
causing pain
O Typically affects aorta
• Tear forms in tunica inti ma of aorta -
high pressure blood flows between tunica
intima/tunica media - layer separation -
false lumen - dilate aorta
• Most aneurysms develop in first 10 cm of
aorta
• Can present acutely/chronically
TYPES
Stanford classification
• Type A: dissection involves ascending aorta
and/or aortic arch, sometimes descending
aorta
• Type B: dissection involves descending
aorta/aortic arch without involvement of
ascending aorta
• Blood flow tears tunica media/tunica
externa - severe internal bleeding -
death
• Blood flow tears tunica intima again, return
to true lumen (not severe)
• Obstruction of arterial branches off aorta.
leading to ischemia of individual organs
• Blood tunnels, creates false lumen that
extends to aortic branch - obstruction
(_~S_IG_NS_&~SY_M_P_TO_M_S~)
• Sudden. intense, tearing chest pain
radiating to back, nausea. vomiting,
diaphoresis
• Chronic dissections painless
• Decreased peripheral pulses, asymmetric
pulses
• Hypertension/hypotension depending on
location of dissection
• Diastolic decrescendo murmur: ascending
aortic dissections - aortic regurgitation
• Neurological deficits: stroke, hemiplegia,
syncope

CAUSES
• Weakness in vessel wall due to chronic
hypertension. blood vessel coarctation,
connective tissue disorders, aneurysms

RISI( FACTORS
• Pregnancy, previous open heart surgery,
vasculitis, trauma, family history of aortic
dissection. Turner's syndrome. cocaine use
• Cystic medial necrosis: familial inherited
disorder causing degenerative breakdown
of collagen. elastin, smooth muscle; wall
weakens. predisposing individual to
aneurysm/dissection

COMPLICATIONS
• Pericardial tamponade: most common Figure 3.6 Gross pathology of an aortic
cause of death dissection.

19
 afratafreeh.com exclusive
OTHER DIAGNOSTICS
(..____ D_IA_GN_o_s,_s)
ECG
DIAGNOSTIC IMAGING • Helps rule out alternative diagnostic
possibilities, e.g. myocardial infarction
Chest X-ray
• Widening of mediastinum consistent with
dissection, but inadequate as sole evidence C..____ T_R_EA_:l"_M_EN_T )
for diagnosis

Transesophageal echocardiogram
MEDICATIONS
• Stanford Type 8: lower heart rate, blood
• Best for hemodynamically-unstable
pressure
individuals
, First line: beta-blockers
• High sensitivity for identifying dissection,
complications like aortic regurgitation, , Second line: calcium channel blockers
cardiac tamponade, involvement of , Pain management for acute dissection
coronary arteries

CT angiography SURGERY
• Stanford type A: medical emergency,
• Best for hemodynamically-stable
surgical repair indicated
individuals
• Stanford Type 8: surgical repair indicated
• High sensitivity for identifying dissection,
when dissection acute, complications arise,
can provide anatomic information useful
medication ineffective
in planning surgical repair; visualize/locate
dissection

Figure 3.7 Abdominal CT scan in the axial plane demonstrating an aortic dissection of the
descending aorta. Note the media, dissected from the wall of the aorta, demarcating the true
and the false lumen.

20
 NOTES

- NOTES
•
-
BRADYCARDIA & HEART BLOCk

GENERALLY,WHAT ARE THEY?

( PATHOLOGY & CAUSES ) (__ s,_G_NS_&_S_Y_M_PT_O_M_s_)
• Delay or complete blockage in the electrical • If symptomatic, may present as
conduction system of the heart -e abnormal lightheadedness, headache, syncope,
heart rhythm; primarily, bradycardia palpitations, Stokes-Adams attacks,
fatigue, dyspnoea etc.

CAUSES
• Can be caused by defect in ( D_IA_GN_o_s,_s __ )
O Atrioventricular node
O Bundle branches • ECG-based; see individual disorders
o Sinoatrial node
• Idiopathic or secondary to
O Myocardial ischemia
( T_R_E~_~_M_EN_T__ )
° Fibrosis
• May not require treatment
o Infections
° Congenital heart disease
MEDICATIONS
° Cardiomyopathies
• E.g. atropine
O Iatrogenic (e.g. medication. post-
surgery)
OTHER INTERVENTIONS
COMPLICATIONS • Transcutaneous pacing
• Pacemaker implantation
• May progress to fatal arrhythmias. heart
failure, and/or sudden cardiac death

21
 ATRIOVENTRICULARBLQC,I(
osms.i"l/ o.-lTioven-lTieulo.T-\>loek
( PATHOLOGY & CAUSES
• Blockage/delay in electrical signal
stimulating contraction between atria,
ventricles
TYPES
• First degree atrioventricular (AV) block
• Second degree atrioventricular block
O Type 1/Mobitz 1/Wenckebach
o Type 11/Mobitz II
• Third degree atrioventricular block/complete
heart block
CAUSES
Congenital heart disease
Heart damage
• Infiltrative/dilated cardiomyopathies,
muscular dystrophy, lyme disease,
myocardial ischemia, myocarditis,
endocarditis with abscess, hyperkalemia,
high vagal tone
Iatrogenic causes
• Medication (beta blockers, calcium channel
blockers, cardiac glycosides). post-cardiac
surgery, post-catheter ablation, post-
transcatheter aortic valve implantation
Lev's disease/Lenegre-Lev syndrome
• Idiopathic fibrosis and calcification of
heart's electrical conduction system, most
common in elderly
COMPLICATIONS
• Heart failure secondary to bradycardia;
third degree AV block risk for sudden
cardiac death
) (__ s,_G_NS_&_SY_M_PT_O_M_s_)
• Presence/severity depends on ventricular
rate
, Lightheadedness, syncope, fatigue,
dyspnea
( D_IA_GN_o_s,_s __ )
OTHER DIAGNOSTICS
ECG
• First-degree AV block
, Signal delayed; continues to ventricles
, PR interval > 200ms due to delayed
ventricular contraction
• Second degree AV block
, Type 1/Mobitz 1/Wenckebach: PR
interval lengthens with each beat until
blocked completely (e.g. progressive PR
intervals : 200ms----. 260ms ----. 300ms
----. dropped beat; no QRS). Ventricular
escape beat: if ventricle does not receive
signal from atrioventricular node after
short time, latent pacemaker cells within
bundle of His/ventricle kick in, begin
pacing heart at slower than normal rate
(-20-50bpm)
, Type 11/Mobitz II: prolonged PR interval
(> 200ms). Block commonly in bundle
of His+-s QRS usually wide (> llOms).
intermittent dropped beats (no QRS).
Happens randomly; no progressive
lengthening of PR interval; every second
P wave blocked. may progress to third
degree AV block
• Third degree AV block/complete heart block
, Signal completely blocked every time
, Eg. ventricles contract at lower rate
than atria (ventricular pacemaker cells
establish rate)
, No association between P waves, QRS
complexes
22
 MNEMONIC
( T_R_E~_~_M_EN_T
__ ) AV blocks
If the R is far from P, then you
• Depends on type/severity
have a First Degree.
= For all: identify electrolyte imbalances/
Longe~longe~ longe~
medication-induced causes
drop! Then you have a
• No treatment: Wenckebach.
= First degree AV block, asymptomatic If some P's don't get through,
type I second degree then you have Mobitz II.
If P's and Q's don't agree, then
MEDICATIONS you have a Third Degree.
• Atropine: second degree, third degree

OTHER INTERVENTIONS
Permanent pacemaker
• Asymptomatic: type II second degree, third
degree
• Symptomatic: type I & II second degree,
third degree

Transcutaneous pacing
• Symptomatic: type I & II second degree,
third degree

~I. . . . . --f
n n
f I f I _ _.._..... __ f I

Figure 4.1 ECG (lead II) demonstrating first degree atrioventricular block.

n V1

Figure 4.2 ECG {lead V1) demonstrating Mobitz I (Wenckebach) second degree atrioventiricular
block.

23
n
Figure 4.3 ECG (lead V1) demonstrating Mobitz II second degree atrioventricular block.

n V1

Figure 4.4 ECG (lead V1) demonstrating third degree (complete) atrioventricular block.

BUNDLE BRANCH BLQC,I(

osms.i-l/\,undle-\>Te1neh-\>loek
from complete heart block and may lead to
( PATHOLOGY & CAUSES ) ventricular asystole

• Electrical signal for contraction of left/right Intermittent bundle branch block

ventricle completely blocked or delayed • Occasional block, unrelated to heart rate

Rate-related bundle branch block

TYPES
• Block occurs when heart rate is relatively
• Either right or left bundle branch blocks can
fast, temporarily resolves once heart rate
be complete or incomplete
slows down
° Complete: total blockage of signal
transmission
O Incomplete: slowed signal transmission CAUSES
• Fibrosis/scarring, formed acutely/chronically
Right bundle branch block (RBBB)
• Signal blocked in right bundle branch Acute
O Left ventricle contracts first-« signal • lschemia, myocardial infarction. myocarditis
carried to right side via Purkinje fibers ----> • Sudden increase in right ventricular
right ventricle contracts pressure----> pulmonary embolism
• Iatrogenic: right heart catheterization/
Left bundle branch block (LBBB) ethanol ablation of basal ventricular septum
• Signal blocked in left bundle branch
O Right ventricle contracts ----> left ventricle Chronic
contracts • Gradual remodelling of heart muscle
O
Hypertension. coronary artery disease,
Bilateral bundle branch block cardiomyopathies
• Caused by disease involving both right/left O Pulmonary hypertension
bundle branches; on ECG, indistinguishable
n Congenital heart disease

24
RISI( FACTORS • LBBB only
• Increasing age, associated with underlying O Negative V1, positive V6 (away from V1
or advancing heart disease towards V6)
O V1: QS, or "little r"-rS complex. W shape
O V6: large, notched R wave. M shape
(__ s,_G_NS_&_S_Y_M_PT_O_M_s_) • RBBB only
O Positive V1,negative V6
RBBB
O V1: large terminal R wave. M shape
• Asymptomatic; wide splitting on
auscultation O V6: slurred S wave, W shape

LBBB
• Asymptomatic; reversed splitting on (...____ T_R_EA_:t"_M_EN_T )
auscultation
• No treatment

( D_IA_GN_O_s,_s __ )
MNEMONIC: WilliaM
OTHER DIAGNOSTICS MaRRoW
ECG ECG of Left BBB
• LBBB and RBBB W-shape in Vl Left BBB
O Lead II (limb lead) shows long QRS Left BBB has V6 M-shape
complex> 120ms (normal: 80-120ms)
O Longer QRS complex because ECG of Right BBB
depolarization starts on time but ends M-shape in Vl Right BBB
later due to depolarization delay in one Right BBB has V6 W-shape
ventricle

~T!'f ~olt ~'S"t ~olt 1l13"'t ~olt

\oya"""' "'°' "- ... VI wa,ic.\,, \,lo<\'. "' VI \oy0,ic.\,, \,lo<\'. "- V{o

Figure 4.5 Illustration depicting mnemonic "WiLLiaM MaRRoW."

25
 •• V4

~
I

Figure 4.6 ECG demonstrating left bundle branch block.

~~j~
~~

V,~~r1t;Vt~~
II t
V'\J1/\J\/"4r\fv./V(/Ji/,V'-A/'4/'-V\v\Jv·-4/\JVVt/'0/~rV\)VVi/viJVvr'Al'-4/\
Figure 4.7 ECG demonstrating right bundle branch block.

26
 LE.AO 11 "' I 11'1,,'b lcrul.

Figure 4.8 Illustration depicting wide QRS in bundle branch block.

~j,_LVI NOil-MA.i.. Vt.

-y l-
\.lVf 1',......stir
._. • .,&,.\,,,\,\• \'.. VI ~~!\,,,~~ V<.

w .r:
fl_t~1ott
v
~,n. ll131o+
M
~die
VI \.ya"<-"' l,lod'- "- V(o

-~·1
..,,,
.. .., ....,"'•d'- "'

f-~"
"1
M
-~sJ
\111.'N'c4

Figure 4.9 Illustration depicting M-shape and W-shape in bundle branch blocks.

27
 SIC,I( SINUS SYNDROME
osms.i-l/ siek-sinus-s14nd Tome

( PATHOLOGY & CAUSES ) (__ s,_G_NS_&_S_Y_M_PT_O_M_

• Malfunction in sinoatrial node (SA node)
characterized by persistent spontaneous
sinus bradycardia, alternating sinus
bradycardia and tachyarrhythmia
(sometimes called tachycardia-bradycardia
syndrome)
• Stokes-Adams attacks (fainting due to
asystole/ventricular fibrillation). syncope,
palpitations, chest pain, dyspnea. fatigue,
headache, nausea
• Variable ECG findings

CAUSES (......___ D_IA_GN_O_s,_s)

• Disorders causing scarring/degeneration/
damage to SA node
DIAGNOSTIC IMAGING
O Sarcoidosis. amyloidosis, ECG
hemochromatosis, Chagas disease,
cardiomyopathies
OTHER DIAGNOSTICS
• Can be caused/worsened by certain
• Tilt table testing
medications
• Holter monitor
O Digoxin, calcium channel blockers, beta
blockers. anti-arrhythmics
• Congenital
O Mutations
(......___ T_R_E~_~_M_EN_T )
of SCN5A gene encoding
alpha subunit of sodium ion channel OTHER INTERVENTIONS
Pacemaker implantation
RISI( FACTORS
• For hemodynamically stable individuals,
• Elderly
tachycardia can be treated with medication;
• Coronary artery disease
can be combined with pacemaker in some
• High blood pressure cases
• Aortic, mitral valve diseases • For hemodynamically unstable individuals.
definitive therapy requires pacemaker
COMPLICATIONS implantation; medication plays limited role
• Sinus arrest. sinus node exit block, sinus
bradycardia
O May be associated with tachycardia
(characterized by long pause after
tachycardia), e.g. atrial tachycardia, atrial
fibrillation
O Associated with azygos continuation of
interrupted inferior vena cava

28
 NOTES

- NOTES
•
-
CARDIAC TUMORS
GENERALLY,WHAT ARE THEY?
( PATHOLOGY & CAUSES ) (__ SI_G_NS_&_S_Y_M_PT_O_M_s_)
• Abnormal cell growth forms mass in heart • Asymptomatic
, Incidental finding on echocardiogram,
TYPES MRI, CT scan
• Dyspnea
Primary , Most common symptom
• Rare , Can progress to orthopnea, paroxysmal
o Adults: myxoma nocturnal dyspnea
° Children: rhabdomyoma • "Tumor plop" sound upon auscultation with
left atrial myxoma
Secondary
• Syncope, presyncope, dizziness, chest pain/
• More common than primary tightness
• Metastases from cancer in other areas (lung
cancer, lymphoma, breast cancer, leukemia,
melanoma, hepatocellular carcinoma, colon ( D_IA_GN_O_SI_S
__ )
cancer)
O Lymphogenous/hematogenous DIAGNOSTIC IMAGING
dissemination • MRI, CT scan, ultrasound
, Incidental finding
COMPLICATIONS , See individual disorders
• Impaired left ventricular structure, filling, • 20 echocardiogram preferred procedure
ejection caused by tumor
• Arrhythmias: tumor disrupts normal nodal/ LAB RESULTS
septal electrical conduction
• Histology conducted on biopsy via surgical
• Heart failure: tumor obstructs inflow/
excision/fine needle aspiration
outflow
• Recurrence of tumor after excision (if tumor
not completely removed)
• Embolism. sudden cardiac death,
( TREATMENT )
myocardial infarction
--------------~
SURGERY
• Symptomatic: surgical resection

OTHER INTERVENTIONS
• Asymptomatic: monitor

29
 ATRIAL MYXOMA
osms.i-1:/a-l:,-ial-m14xoma

( PATHOLOGY & CAUSES ) (__ SI_G_NS_&_S_Y_M_PT_O_M_

)
• A benign heart tumor • Asymptomatic: incidental finding on
• Most common primary cardiac tumor in echocardiogram, MRI, CT scan
adults • Dyspnea: most common symptom
• Arises in heart's mesenchymal connective , Can progress to orthopnea, paroxysmal
tissue nocturnal dyspnea
• Most common in left atrium. may cause • "Tumor plop" sound upon auscultation with
syncope left atrial myxoma
O Tumor in left atrturn=-. obstructs mitral • Syncope, presyncope, dizziness, chest pain/
valve tightness
• Histology
O Pedunculated (attached to tissue stalk)
O Gelatinous due to abundance of ground
(..____ D_IA_GN_O_SI_S
)
substance
LAB RESULTS
RISI( FACTORS Histology
• Age 40-60 • Stellate myxoma cells in myxoid stroma of
glycosaminoglycans
• More common in biological females
O Less pronounced in familial atrial
myxoma
( T_R_EA_~_M_EN_T
)
• Genetic disease
SURGERY
• Symptomatic: resection

OTHER INTERVENTIONS
• Asymptomatic: monitor

Figure 5.1 Surgically excised atrial myxoma. A

small piece of myocardium marks the point of
attachment.

30
 • , ': • • 41 ,· •• ~6PINO~E!,CELLS, · •

. ..
. '"; . .. .... .
. , ,., . . ' .-\ . . ~ : ~.,
. .
,; '. f: ~
·'" . :,
.
,,,,.. •
.
. ... . ,. }>·
I
·
• I
/.' "
t
" f) I I . ,· •." ',.:
f
• •.,
.....

· · 1··
c •
. .·. , •· •
•
.. , .

_J'..L .... ·L . . ._ ,' \_- .. ~ . . ., . .-

Ih ~UC.010 MATRIX ·.[ o ~ • . ~ : , . ~ -, J
. .
...: -. ", ·-"'- ~

Figure 5.2 Histological appearance of a

myxoma with abundant mucoid matrix (pink
background) and scanty, bland spindle cells
with low mitotic activity.

Figure 5.3 A sagittal CT scan demonstrating

a myxoma in the left atrium.

RHABDOMYOMA
osms.i"l/Tho.\,dom14omo.
• Associated with nevoid basal cell
( PATHOLOGY & CAUSES ) carcinoma syndrome
• Genetic disease
• Benign tumor of striated muscle
• Most common primary cardiac tumor in
infants/children ( D_IA_GN_o_s,_s __ )
• Arises in ventricles
• Presents congenitally DIAGNOSTIC IMAGING
• Benign hamartoma (abnormal tissue
MRI, CT scan, ultrasound
formation)
• Incidental finding
• Association between rhabdomyoma/
tuberous sclerosis about 30-50% Ultrasound, MRI
• Often regresses spontaneously • Visualize tumor
• Shrink with age

LAB RESULTS
RISI( FACTORS
• More common in children Histology
• More common in biological males (2.4:1 • Hamartomatous growths surrounded in a
male-female ratio) glycogen-rich eosinophilic cytoplasm
• Average presentation age is four years old

31
(__ SI_G_NS_&_SY_M_PT_O_M_s
)
• Usually present at birth
• Tender, painful, benign, slow-growing
nodules
° Common in neck/mouth/larynx, may
cause breathing difficulties

( T_R_E~_iT_M_EN_T
)
SURGERY Figure 5.4 A surgically excised
• Symptomatic: surgical resection rhabdomyoma.

OTHER INTERVENTIONS
• Asymptomatic: monitor

Figure 5.5 Histological appearance of a rhabdomyoma composed of plump, pink skeletal muscle
cells.

32
 NOTES

- NOTES
•
-
CARDIOMYOPATHY
GENERALLY.WHAT IS IT?
( PATHOLOGY & CAUSES ) ( D_IA_GN_O_s,_s)
• Broad term, describes any issue resulting DIAGNOSTIC IMAGING
from disease of myocardium • Chest X-ray
• Primary cardiomyopathy: issue develops of • Echocardiogram/cardiac MRI
its own accord
• Secondary cardiomyopathy: issue develops
OTHER DIAGNOSTICS
as compensation for another underlying
• ECG
disease

RISI( FACTORS ( T_R_E~_~_M_EN_T

)
• Positive family history
MEDICATIONS
• See individual diseases
COMPLICATIONS
O Heart failure, arrhythmias, sudden
cardiac death SURGERY
• Implantable cardioverter-defibrillator (ICD)
• Heart transplant
(__ SI_G_NS_&_S_Y_M_PT_O_M_s_)
• Can be asymptomatic OTHER INTERVENTIONS
• Heart failure signs, symptoms • Lifestyle changes
• Heart murmurs

33
 DILATED CARDIOMYOPATHY
osms.i"l/dilo.-led-em
, Related to pregnancy-associated
( PATHOLOGY & CAUSES ) hypertension
, Half of individuals recover following
• Dilation of all four chambers of heart
pregnancy
• Most common type of cardiomyopathy
O New sarcomeres added in series, Sarcoidosis
creates larger chambers with relatively • Growth of granulomas in heart - dilation
weak walls, less muscle for contraction
- low systolic function
RISI( FACTORS
° Chambers stretch - valves stretch -
• Alcoholism, past family history of diseases
blood regurgitates back into atria
implicated in DMD

CAUSES COMPLIC ATIONS

• Primary dilated cardiomyopathy most often
• Systolic heart failure
idiopathic
, Valve regurgitation: as chambers
Genetic mutations/conditions stretch, so do valves
• Duchenne muscular dystrophy (DMD), • Arrhythmias: stretching muscle irritates
hemochromatosis conduction system

Myocarditis
• Can progress from myocarditis to dilated MNEMONIC: ID SIG MAPS
cardiomyopathy Causes of Dilated
Cardiomyopathy
Infection
Idiopathic
• Coxsackievirus 8: leads to myocarditis,
Drugs/Doxorubicin (and
heart muscle inflammation
cocaine)
• Chagas disease: protozoa! infection
Beriberi (wet)
Linked to alcoholism Infection
• Alcohol, metabolites have direct toxic effect Genetic
on heart muscle Myocarditis
Alcoholism
Linked to certain drugs
Peripartum cardiomyopathy
• Chemotherapy: doxorubicin, daunorubicin
Sarcoidosis
• Cocaine

Wet beriberi
• Beriberi: illness caused by thiamine (vitamin
Bl) deficiency
• Wet beriberi: affects hearr ] thiamine
levels impair myocardium energy
production

Peripartum cardiomyopathy
• Can develop in third trimester of pregnancy/
weeks after delivery

34
 (
(__ SI_G_NS_&_S_Y_M_PT_O_M_s
) T_R_E~_~_M_EN_T
)
• Fatigue, dyspnea MEDICATIONS
• Lateral displaced point of maximum • Angiotensin-converting-enzyme (ACE)
impulse (PMI) inhibitor, angiotensin receptor blocker, beta
• Chest pain on exertion blocker
• Holosystolic murmur (mitral valve , Slows disease progression
regurgitation during systole)
• S3 sound (blood rushing into, slamming SURGERY
into dilated ventricular wall during diastole) • Heart transplant (extreme cases)

( D_IA_GN_O_s,_s) OTHER INTERVENTIONS

• Left ventricular assist device (LVAD):
DIAGNOSTIC IMAGING mechanical pump assists heart in delivering
blood to body
X-ray
• Cardiomegaly, pulmonary edema, pleural
effusion

OTHER DIAGNOSTICS
ECG
• Shows ventricular dilation, reduced ejection
fraction

Figure 6.2 A chest radiograph demonstrating

enlargement of the heart due to dilated
cardiomyopathy. The heart occupies more
than half the width of the chest.
Figure 6.1 Gross pathology of dilated
cardiomyopathy. Note the large ventricles
and thin ventricular walls.

35
HYPERTROPHIC CARDIOMYOPATHY
(HCM)
osmsJl/h14peT-lTophie-em
COMPLIC ATIONS
( PATHOLOGY & CAUSES ) • Arrhythmias, sudden cardiac death

• Myocardium becomes thick, heavy,

hypercontractile
• Myocytes become disorganized, new
sarcomeres added in parallel to existing
ones
• Left ventricle most often affected
O Muscle growth asymmetrical -
interventricular septum grows larger
relative to free wall
• Hypertrophy - walls taking up more
space, ! blood fills ventricle
O Walls become stiff, less compliant -
less filling - low stroke volume -
dysfunction in diastolic filling of left
ventricle - diastolic heart failure
• Arrhythmias: larger muscles require more
oxygen, coupled with heart having difficulty
delivering blood to tissues - ischemia -
arrhythmias
CAUSES
RISI( FACTORS
• Positive family history of HCM/conditions
known to be associated with HCM (e.g.
Fried reich's ataxia)
( SIGNS & SYMPTOMS )
• Many individuals asymptomatic
• Auscultation: crescendo-decrescendo
murmur
, l intensity with ! venous return
(Valsalva, standing), ! in intensity with I
venous return (handgrip, squatting)
• Symptoms arise as complications arise
O Dyspnea: left ventricle stiffening, atrium
increasing back pressure into lungs -
interstitial lung congestion
n Fatigue
Exertional chest pain: ischemia
O

O Syncope with exertion: brain receiving

Genetic missense mutation, inherited as low oxygen
autosomal dominant trait O Palpitations: ischemia, arrhythmias
• Different genetic mutations affect different O
Sudden cardiac death
sarcomere proteins • May exhibit bifid pulse: two pulses felt
• Friedreich's ataxia: autosomal recessive Mitral valve moves toward outflow tract
O

neurodegenerative disease
- l obstruction mid-systole
Hypertrophic obstructive cardiomyopat
hy
(subtype)
• lnterventricular septum growth blocks left
ventricular outflow tract during systole
- blood must flow quickly through small
opening, ! pressure in this area (Venturi
effect) - low pressure pulls anterior leaflet
of mitral valve toward septum - further
mitral valve obstruction towards septum -
further obstruction overall

36
 Disopyramide
( D_IA_GN_O_s,_s ) • Can be used for its negative inotropic
properties
DIAGNOSTIC IMAGING
Digoxin contraindicated
Echocardiography
/cardiac MRI
• l force of contraction, can t obstruction
• Enlarged heart charnbers/] ejection fraction

Chest X-ray SURGERY

• j ratio of distance between heart, thoracic • Implantable cardioverter-defibrillator
cage
Surgical septal myectomy
• Involves removing portion of
LAB RESULTS
interventricular septum, ! obstruction
Genetic testing
Septal ablation
• Cardiomegaly-implicated gene mutations
• Chemical myomectomy to partially ablate
septum
OTHER DIAGNOSTICS
Heart transplant
ECG • If unresponsive to all other forms of
• Detectable electrical changes, such as left treatment
ventricular hypertrophy

OTHER INTERVENTIONS
( T_R_E~_~_M_EN_T ) Lifestyle change
• Cessation of high-intensity athletics
MEDICATIONS
Beta blockers
• ! heart rate, contractile force
Calcium channel blockers
• If beta blockers not tolerated
• Slows down heart rate

Figure 6.4 The histological appearance of

the myocardium in a case of hypertrophic
cardiomyopathy. There is complete myocyte
disarray. The myocytes display bizarre forms
with side to side branching and are arranged
in a whorled configuration.

Figure 6.3 Gross pathology of hypertrophic

cardiomyopathy. The myocardium has
become so enlarged that both ventricles are
almost entirely obliterated.

37
 \-l't'PE:fi:!T!l-oPH IC."'- CAttOIOM~OP~T\-\'1'
(_ogsrl4.l<.'T\VE.)

ossnz.u,T~D
L.6FT V£._,T~I C.UL.A~
ou-n:Lov..1 naAc:..T

t
Bwot>1'HIU>uc.-i"
SMAU.. D?~"''"'"
i
C~S.~~-0£t~(H.1t>o]
~u~~l2
S, S
Al<,o S£h-J 1u
~~It S1'EJJ0S1~
I ,dlJ,11fJl
<VW'Wr " I ~
141
i
Figure 6.5 Illustration showing the Venturi effect: low blood pressure pulls the anterior
leaflet of the mitral valve towards the septum, creating an obstruction. Blood can't get
through the small opening, leading to a crescendo-descrescendo heart murmur.

RESTRICTIVE CARDIOMYOPATHY
osms.i-l/ Tes-1:Tie-live-em
heart tissue
( PATHOLOGY & CAUSES )
Endocardial fibroelastosis
• Cardiomyopathy: heart wall is rigid, has • Fibrosis develops in endocardium (inner
difficulty stretching, pumping lining of heart) and subendocardium (layer
• Ventricles restrict filling, ! cardiac output underneath endocardium)

Loffler syndrome
CAUSES • Eosinophils accumulate in lung tissue
• Infiltrative diseases, storage diseases,
• Loeffler endocarditis/Loetfler
endomyocardial diseases.
endomyocarditis: eosinophils also
accumulate in endocardial layer of heart
Amyloidosis
tissue - inflammation, endocardial fibrosis
• Amyloids are misfolded proteins -
- restrict heart tissue
insoluble - deposit in tissues, organs -
organs less compliant Hemochromatosis
• Familial amyloid cardiomyopathy O Iron deposits in heart tissue, contributes
• Mutant transthyretin (TTR) protein; to restricted tissue
misfolded deposits preferentially in heart
tissue Other causes
• Senile cardiac amyloidosis; TIR protein/ • Heart tissue radiation
wild type TTR deposits in heart over time , Radiation generates reactive oxygen
species - inflammation over time
Sarcoidosis - myocardial fibrosis - tissue stiff,
• Immune cell collections form granulomas in
38
 restrictive

COMPLICATIONS
• Can - diastolic heart failure
O Stiff ventricles - cannot stretch - less
filling - low cardiac output - heart
failure

\ MNEMONIC: LASHER
Causes of Restrictive
Figure 6.6 Histological appearance of the
cardiomyopathy
myocardium in a case of cardiac amyloidosis;
Loffler syndrome
a cause of restrictive cardiomyopathy. Cardiac
Amyloidosis myocytes (dark purple) are surrounded by
Sarcoidosis amyloid deposits (light pink).
Hemochromatosis
Endocardial fibroelastosis
post-Radiation
( T_R_E~_~_M_EN_T
__ )
MEDICATIONS
(__ SI_G_NS_&_S_Y_M_PT_O_M_s_) Loop diuretics
• ! systemic, pulmonary congestion
• Auscultation: stiff ventricle - S4 heart
sound Beta-blocker, calcium channel blocker, an-
• Presents as congestive heart failure: giotensin converting enzyme inhibitors
dyspnea; paroxysmal nocturnal dyspnea; • Slows heart rate, j ventricular-filling time
orthopnea; crackles; intraalveolar
hemorrhage; fatigue; inability to exercise;
appetite loss; abdomen swelling; swelling SURGERY
of feet, ankles; uneven/rapid pulse; chest • Heart transplant
pain; low urine output; nocturia

( D_IA_GN_o_s,_s
__ )
OTHER DIAGNOSTICS
ECG
• Low-amplitude signals: peak to nadir
measurement of QRS complex being<
5mm (limb leadsj/« lOmm (precordial
leads). Low voltage produced due to loss of
viable myocardium
• Small QRS complexes: QRS complexes
represent ventricular contraction, restricted
tissue - weaker contraction

39
 afratafreeh.com exclusive

Genetic. infectious,
Genetic storage diseases;
alcoholic. peripartum
endomyocardial disease

Asymmetric myocardial
Large chambers, growth; markedly thick Rigid, noncompliant
relatively weak wall walls, overgrowth of heart wall resists filing
septum

Impaired diastolic
Impaired diastolic
Impaired systolic relaxation;
relaxation; normal
contraction hyperdynamic systolic
systolic function
function

53 54 54

AV valves; especially
Mitral valves AV valves
mitral

Atrial fibrillation, Low amplitude signals;

Atrial fibrillation;
ventricular small QR5 signals; AV
ventricular fibrillation
tachycardia block

Enlarged cardiac
silhouette (ballooned); Normal to enlarged Usually normal cardiac
pulmonary venous cardiac silhouette silhouette
congestion

Hypertrophied LV; LV Enlarged right atrium;

Decreased LV wall outflow obstruction; LV ejection fraction
thickness; LV ejection mitral regurgitation; LV decreased; infiltrative
fraction decreased ejection fraction normal disorders cause
to increased "speckled" appearance

Increased risk of
May lead to sudden JVD may manifest as
thromboembolic
death in young athletes Kussmaul's sign
events

40

- NOTES
• STABLE ANGINA PECTORIS
-
CORONARY ARTERY DISEASE
osmsJl/ s-1:o.\>le-o.ngino.
( PATHOLOGY & CAUSES
• Episodic chest pain because of inadequate
oxygen supply to the heart, most often due
to obstruction in the coronary arteries
• Most common type of angina
O Angina pectoris refers to a specific
type of chest pain caused by lack of
blood flow to the heart muscle. Pain
often presents as pressure, fullness,
squeezing pain in the center of the chest
O In angina - ischemia in the
subendocardium - triggers release of
adenosine and bradykinin - pain
CAUSES
Atherosclerosis
• Causes plaque buildup in vessel; blood flow
to heart muscle limited
O As plaque becomes larger and blocks
more flow, pain arises with lower levels
of exertion
Tachyarrhythmias
• Increased heart rate increases heart's
demand for oxygen that cannot be
delivered, thus causing pain
Pulmonary hypertension
• Left main coronary artery can become
compressed by enlarged pulmonary artery
trunk, leading to reduced perfusion and
pain
Increased myocardial oxygen demand
• Consequence of increased left ventricular
NOTES
mass secondary to concentric hypertrophy,
) and an increased afterload due to the fixed
obstruction to left ventricular outflow
Genetic hypertrophic cardiomyopathy
• Cardiac hypertrophy leads to increased
oxygen demand of heart muscle. In times
of high exertion, the heart cannot supply
enough oxygen and ischemia results
RISI( FACTORS
• Smoking, hypertension, diabetes,
dyslipidemia (high LDL, low HDL, and high
triglycerides), obesity, family history of
coronary artery disease
Protective factors
• Modest alcohol consumption (-one drink/
day), healthy diet (e.g., lots of vegetables,
grains, and nuts), and regular exercise
(__ s,_G_NS_&_S_Y_M_PT_O_M_s_)
• Chest pain coinciding with increased
exertion or stress. Pain described as
pressure, squeezing, burning, or tightness;
can radiate to the either/both arms, jaw,
shoulders, and back
• Pain usually lasts less than 20 minutes
• Other symptoms: Levine's sign (a clenched
fist held over the chest), dyspnea,
diaphoresis, fatigue, nausea, and epigastric
pain
O Angina equivalent: These "other
symptoms" felt without chest pain
during periods of coronary ischemia
(more common in diabetics, biologically-
female individuals, elderly)
41
( D_IA_G_N_O_SI_S)
• Primarily based on signs/symptoms;
"clinical diagnosis"
OTHER DIAGNOSTICS
Electrocardiogram (ECG)
• Stable angina can present with ST segment
depression
o May also present with T wave
inversions
o Illustrates subendocardial ischemia
o Dynamic ECG changes: changes in ECG
are seen for duration of symptoms, but
can reduce in intensity or disappear
when symptoms abate (e.g. individual
may start with 2mm ST depressions
with no chest pain, but then get 4mm
ST depressions during an episode of
unstable angina at rest, which revert to
2mm ST depressions once episode is
concluded)
• Other tests may be required to obtain more
specific information
( TR_E_A_~M_E_N_T
)
MEDICATIONS
Sublingual nitroglycerin
• The classic regimen for stable angina; as
needed, daily aspirin, beta blockers and
stat in
Nitrates and Vasodilator
• Nitrates treat immediate pain
• Vasodilator causes venodilation, which
decreases preload, reducing oxygen
demand of the heart
• Nitroglycerin
, Used to treat immediate pain on as-
needed basis
• lsosorbide mononitrate
, Used for prevention
Beta blockers
• Reduce myocardial oxygen demand by
reducing heart rate and contractility
• First line medication for stable angina
Statins
• Preventative. Lowers low density
lipoproteins, improves dyslipidemia
Aspirin
• Preventative. Prevents thrombosis by
blocking platelet activation, reduces chance
of stable angina ----. unstable angina
42
 NOTES

- NOTES
•
~

CYANOTIC DEFECTS
GENERALLY, WHAT ARE THEY?

c PATHOLOGY & CAUSES ) c DIAGNOSIS )

• Heart defects with cyanotic presentation: DIAGNOSTIC IMAGING
blue discoloration of skin/mucous • Prenatal ultrasound
membranes, typically seen at fingertips, • Echocardiography
lips, extremities
• Chest X-ray
• Develop in utero
• Persistent truncus arteriosus, hypoplastic
left heart syndrome, transposition of great OTHER DIAGNOSTICS
vessels can lead to heart failure • ECG
• Persistent truncus arteriosus, tetra logy of
Fallot can lead to Eisenmenger syndrome
c TREATMENT )
c SIGNS & SYMPTOMS ) MEDICATIONS
• See individual disorders
• Cyanosis
• See individual disorders SURGERY
• Definitive treatment

OTHER INTERVENTIONS
• Lifestyle changes

DEOXYGENATED OXYGENATED
BLOOD from BODY BLOOD from LUNGS

LEFT ATRIUM

PULMONARY
ARTERY

Figure 8.1 Illustration depicting blood flow in hypoplastic left heart syndrome .

43
 HYPOPLASTIC LEFT HEART
SYNDROME
osmsJl/h14poplas-f:le-lef-f:-hea,-{-s14nd
,-ome

( PATHOLOGY & CAUSES ) (__ s,_G_NS_&_S_Y_M_PT_O_M_

)
• Congenital underdevelopment of left heart • Respiratory distress, poor feeding/failure to
thrive, left-sided heart failure

CAUSES
• Unknown: primary congenital heart defect (.._____ D_IA_GN_O_s,_s)
may reduce flow through left ventricle/
left outflow tract, affect other heart DIAGNOSTIC IMAGING
ma !formations
• Underdeveloped left ventricle, ascending Prenatal ultrasound
aorta
Chest X-ray
O Aortidmitral valves may also be
affected, narrow, or absent (atresia) • Cardiomegaly

• If untreated: left-sided heart failure -

cardiogenic shock - death OTHER DIAGNOSTICS
Atrial septal defect (ASD) and Patent duc- ECG
tus arteriosus (PDA) • Right ventricular hypertrophy
• ASD/PDA required for post-natal survival in • After birth
hypoplastic left heart syndrome
• With ASD, PDA: right heart function
present but impaired; sometimes ( T_R_EA_~_M_EN_T
)
asymptomatic at birth
O Oxygenated blood in left atrium flows MEDICATIONS
into right atrium through ASD - • Prostaglandin El keeps ductus arteriosus
pulmonary artery - PDA - aorta - open until surgery can be performed
body
O Within one day: ductus arteriosus

begins closing - cyanosis

SURGERY
• Surgical repair/heart transplant based on
• Without ASD, PDA: heart not capable of
complexity
sustaining life outside womb
O Right heart functions normally -
oxygenated blood enters left atrium -
flow backs up due to small mitral valve,
small left ventricle - high pressure in
left atrium. blood circulated ineffectively
by left ventricle

44
 PERSISTENT TRUNCUS
ARTERIOSUS
osms.i"l/truneus_o.rteriosus
associated with genetic disorders
( PATHOLOGY & CAUSES ) (DiGeorge syndrome)

• Truncus arteriosus fails to divide into aorta/

pulmonary artery COMPLIC ATIONS
• Single giant artery branching off from • Cardiomegaly
right, left ventricles which splits into aorta, • Pulmonary hypertension, can progress to
pulmonary artery permanent lung damage
• Oxygenated, deoxygenated blood mix • Respiratory problems
• Deoxygenated blood mixes into systemic • Arrhythmia
circulation - cyanosis • Valve regurgitation

CAUSES
• Associated with DiGeorge
( s,_G_NS_&_SY_M_PT_O_M_s_)
syndrome/22q 11.2 deletion syndrome
• Difficulty breathing, pounding heart,
(abnormal tissue formation during
weak pulse, poor feeding/failure to thrive,
development)
lethargy
• Before birth, deoxygenated blood sent to
• With physical exertion (severity varies)
mother, oxygenated blood arrives from
mother
O Dizziness, fatigue, palpitations, dyspnea
° Fetal heart sends blood through • Impaired growth
foramen ovale • Auscultation
O Loud systolic murmur along left sternal
O Oxygenated, deoxygenated blood mix
in truncus arteriosus. Both circulations border due to increased flow through
get same amount of oxygenated, mitral valve
deoxygenated blood ° Constant ejection click before S2
O Otherwise normal fetal development (closure of aortic, pulmonic valves)
• After birth, the baby relies on own lungs - O Diastolic flow murmur at apex when
foramen ovale closes pulmonary blood flow increases
O Deoxygenated, oxygenated blood still
mixed - cyanosis
O Excess blood shunted to pulmonary ( D_IA_GN_O_s,_s
__ )
circuit, as pressure in pulmonary circuit
is less than pressure of systemic circuit DIAGNOSTIC IMAGING
X-ray
RISI( FACTORS • Shows heart size, lung abnormalities,
• Combination of genes, maternal possible presence of excess fluid in lungs
environment
• Smoking, excessive alcohol intake, Echocardiogram
teratogenic medications during pregnancy; • Single large vessel arising from left, right
gestational diabetes; viral illness during ventricles
pregnancy (e.g. German measles); • Abnormalities of valves between large

45
 vessel and ventricle it arises from
• May show abnormal blood movement
between right, left ventricle, and volume of
blood flow to lungs
lnotropic agents
• Strengthens cardiac contractions (e.g.
Digoxin, treats congestive heart failure,
slows down heart rate, increases force of
contractions)

OTHER DIAGNOSTICS Prophylaxis

• Newborn pulse oximetry screening • Antibiotics during dental/other surgical
O Low oxygen saturation procedures to avoid infections
O Diagnose before symptoms develop

ECG SURGERY
• Atrial enlargement (notching of P waves/P • Goal: restore normal blood flow through
mitrale) heart

• Ventricular hypertrophy • Procedures vary depending on individual

anatomy
• Abnormal T waves
, Close hole between right/left ventricles
• Right axis deviation
, Separate large vessel into pulmonary
artery, aorta
(.____ T_R_EA_:T_M_EN_T__ ) , Reconstruct single large vessel into
new, complete aorta
• In rare cases, babies may survive into , Implant new tube, valve to connect right
adulthood without surgical repair ventricle with upper part of pulmonary
artery, creating new, complete
pulmonary artery
MEDICATIONS
Diuretics OTHER INTERVENTIONS
• Gets rid of excess fluid (e.g. chlorothiazide) • Lifestyle: possible limitation on intense
physical activity
• Lifelong monitoring

TRUNCUS ATERIOSUS (TA)

resislance &

pmsure ~UNG~
BODY
PULMONARY
ARTERY AORTA

MIGM PRESSURE ~WPRESSURE

lESS SLOOD ' MORE SLOOD

Figure 8.2 llustration depicting blood flow through the heart in persistent truncus arteriosus.

46
Figure 8.3 Gross pathology of a persistent truncus arteriosus. Both the left and right ventricles
pump blood to both the aorta and pulmonary artery through a quadricuspid truncus valve.

•
y4·;~.!,
1.~

!' ~,. ··/· ...- -·.. ~

.,
- .

. ")-· .- ·-
.. .. .
w· . . 0
'
r.~
Figure 8.4 Gross pathology of a persistent
truncus arteriosus. Both the left and right
ventricles pump blood to both the aorta and
pulmonary artery through a quadricuspid
truncus valve.

47
 TETRALOGYOF FALLOT
osms.tl/-le-lTe1log14-of-fe1llo-l
( PATHOLOGY & CAUSES ) (__ s,_G_NS_&_SY_M_PT_O_M_s
)
• Combination of four congenital heart • Depend upon severity of stenosis
abnormalities • Less severe right ventricular outflow
• Right ventricular outflow tract stenosis obstruction often asymptomatic
(pulmonic stenosis): obstructs pulmonary • Cyanosis around lips, fingernails ("blue
circulation baby syndrome" )
• Right ventricular hypertrophy: • Poor feeding/failure to thrive
compensates for right ventricular outflow • Harsh holosystolic murmur at left upper
tract stenosis sternal border - sounds like pulmonary
• Ventricular septal defect (VSD): hole in wall stenosis
between ventricles. High pressure in right O Right ventricular heave
ventricle - blood shunts from right to left
• Older infants, children
- deoxygenated blood to body
O Clubbed fingers, toes after a few
• Aorta overrides ventricular septal defect:
months
aorta in abnormal position. Variable
presentations
O Exertional dyspnea
O Hypercyanotic episode (tet spell): on
exertion, infant's oxygen demands
CAUSES increase - sudden decrease in oxygen
• Arises during cardiovascular development saturation - cyanosis
• Most common cause of cyanotic congenital
heart defects
• Four abnormalities together cause ( D_IA_GN_o_s,_s __ )
O Mixing oxygenated, deoxygenated
blood DIAGNOSTIC IMAGING
O Narrowed vessels/valves that increase Echocardiography
cardiac workload
• Pre/postnatal
• Severity of stenosis affects blood flow,
changing pressure differentials Chest X-ray
O Mild stenosis: left-right shunt - • "Boot-shaped" heart
oxygenated blood simply goes through
pulmonary circulation again
O Severe stenosis: right-left shunt - MNEMONIC: PROVe
deoxygenated blood enters body Components of tetra logy of
circulation - less oxygen to tissues Fallot
• Leads to pulmonic regurgitation: blood Pulmonary infundibular
flows backwards into RV. right heart stenosis
overloads, can cause right-sided heart
Right ventricle hypertrophy
failure
Overriding aorta
• Associated with alcohol exposure in utero,
Ventricular septal defect
maternal age 40+ years, poor nutrition or
viral illness during pregnancy (e.g. rubella),
Down syndrome or DiGeorge syndrome,
positive family history of tetralogy of Fallot

48
OTHER DIAGNOSTICS
ECG
• Right ventricular hypertrophy, right atrial
enlargement

( T_R_E~_~_M_EN_T
)
MEDICATIONS
Prostaglandin El analogs (alprostadil)
• Severe cases Figure 8.5 Digital clubbing in an adult with
• Keep ductus arteriosus open ----. improve tetra logy of Fallot.
cyanosis until surgery

OTHER INTERVENTIONS
• Treat tet spell
O Infants squat to reduce cyanosis: kinks
femoral artertes=- increases vascular
resistance ----. increases systemic
pressure----. increases pressure in left
ventricle to greater than pressure in
right ventncle c- reverse shunt to left-
right----. resolve cyanosis

SURGERY Figure 8.6 A chest radiograph of an infant

• Cardiac repair surgery (first year of life)
demonstrating the classic boot-shaped heart
O VSD patch closure (only oxygenated seen in tetralogy of Fallot.
blood flows from left ventricle into aorta)
O Right ventricular outflow tract enlarged

TETRALOGYofFALLOT TOF
* foul' MEART ABNORMALITIES * RIGHT-+ LEFT

* CD STENOSIS ~ nanowing
L ,-191,l venfrleulal'
*
ouHlow frael
l.ESS OBSTRUCTION: leH-lo-,-19hl
MORE 08STRUCTION:._l"l9hl-~lef~
less oxljgenatlon
of l(55U&S

@ RIGHTHYPERTROPHY
VENTRICULAR

m VENTRICULAR SEPTAL
DEFECT

@AORTA OVERRIDES SEPTAL

DEFECT

Figure 8.7 Illustration depicting blood flow through a heart with Tetralogy of Fallot.

49
 TOTAL ANOMALOUS PULMONARY
VENOUS RETURN
osms.i-l/ nnomnlous-pulmonnT1J-Venous

( PATHOLOGY & CAUSES ) (__ SI_G_NS_&_SY_M_PT_O_M_s_)

• Congenital heart defect characterized by
anomalous connection of the pulmonary
veins and the heart
• Occurs during first eight weeks of fetal
development; cause unknown
TYPES
• Severity of symptoms depend upon
presence/degree of obstruction
• Cyanosis, tachypnea, tachycardia, dyspnea,
failure to thrive, recurrent respiratory
infections
• In case of infradiaphragmatic variant: liver
enlargement

Supracardiac variant
• Most common ( D_IA_GN_o_s,_s
__ )
• Pulmonary veins open into brachiocephalic
veins/superior vena cava (SVC) DIAGNOSTIC IMAGING
Cardiac variant Chest X-ray

• Pulmonary veins open into coronary sinus/

O Snowman sign (figure of 8): dilated
right atrium SVC, pulmonary vein, brachiocephalic
artery formshead; dilated right atrium
lnfradiaphragmatic variant forms snowman's body
• Pulmonary veins open into portal/hepatic
Echocardiograp hy
veins
• Right ventricular and pulmonary artery
Mixed variant volume loading
• Oxygenated blood travels through • Might show left atrium with no connecting
pulmonary veins to right atrium/veins----> veins
blood does not leave pulmonary circulation
----> no systemic circulation LAB RESULTS
• Incompatible with life unless foramen • Assess oxygenation and acid-base status:
ova le/patent ductus arteriosus present decreased values
----> oxygenated and deoxygenated blood
rnix-» established connection between
pulmonary and systemic circulation OTHER DIAGNOSTICS
• Anomalous connections often accompanied
ECG
by pulmonary vein obstruction ---->
pulmonary venous hypertension, severe • Right ventricular hypertrophy
cyanosis Auscultation
• Systolic ejection murmur
COMPLICATIONS • Increased pulmonary component of S2
• Recurrent pulmonary vein stenosis • Split S2
• S3 gallop

50
 O If present, pulmonary venous
(..____ T_R_E~_~_M_EN_T
__ ) obstruction must be identified and
treated promptly
SURGERY
• Surgery to establish blood flow from the
right atrium to left atrium OTHER INTERVENTIONS
• Cardiac catheterization

TRANSPOSITION OF THE
GREAT VESSELS (TGA)
osmsJl/-l,-ansposl"lion_of _g,..ea-l_ vessels

RISI( FACTORS
( PATHOLOGY & CAUSES ) • During pregnancy: diabetes, rubella, poor
nutrition, consumption of alcohol, > 40
• Abnormal development causes aorta to years old
arise from right ventricle, pulmonary artery
to arise from left ventricle
• Transposition creates two small circuits of
blood flow rather than one large
( SIGNS & SYMPTOMS )
O Right side: right ventricle ----. aorta ----. • In utero: asymptomatic
body----. right atrium ----. right ventricle • d-TGA:
(blood never oxygenated)
• Cyanosis, unchanged with supplemental
O Left side: left ventricle e- pulmonary oxygen (less severe if VSD present)
artery----. lungs----. pulmonary veins e-
• Tachypnea
left atrium ----. left ventricle (blood never
deoxygenated) • Acidosis
• 1-TGA:
• After birth ----. lungs used for oxygen ----.
foramen ovale, ductus arteriosus close O Asymptomatic
----. no exchange between two circuits ----.
cyanosis, death
• Sometimes, foramen ovale or ductus (....___ D_IA_GN_O_s,_s
__ )
arteriosus stay open, or baby has
ventricular septa I defect (VSD); allows DIAGNOSTIC IMAGING
blood to circulate Echocardiogram
• Different levels of severity of transposition
• Evaluate heart function, structure
of the great arteries (TGA)
O d-TGA: dextro-TGA/complete TGA Chest X-ray
(dextro = aorta on right) • Classic triad
0 1-TGA: levo-TGA/congenitally corrected O Heart appears as egg on its side/"egg
TGA (levo = aorta on left). Ventricles. on a string" appearance
valves switched. Great vessels in normal O Lung congestion
orientation, but connected to wrong
, Cardiomegaly
ventricle. Normal blood flow circuits
preserved Angiogram
• Pre-surgery

51
 SURGERY
( T_R_E~_~_M_EN_T
) • Balloon atrial septostomy: short-term
solution. Hole created in atrial septum
MEDICATIONS
• Surgically switch great vessels
• Prostaglandin E: short-term solution. Keeps
, Five year survival rate> 80%
ductus arteriosus open
, No treatment: one year survival rate
10%

Figure 8.8 Chest radiograph in both a lateral (L) and frontal (R) view, demonstrating the "egg on
a string" sign of transposition of the great vessels.

52
 GOMPlETE TRANSPOSITIONof -lhe GREAT ARTERIES
switch places J (cJ.exh-o- TGA)
- C",-1ght"
HEART

"'-NEVER NEVER :i\

gels gels/
OXYGENATED DEOXYGENATED
BODY LUNGS
AORTA PULMONARY

-
aTleTIJ

Figure 8.9 Illustration depicting blood flow through a heart with dextro transposition of the great
arteries.

levo-TGA -v "congenlto.lllJcorrected TGA" N ADULTS at risk of

~YANOTIC HEART FAILURE
notT C\,tue HEART

TIUC.vo.lve
USPID0
+
RIGHT
BODY VENUlc.LE LUNGS
\,utlt foT \,uilt foT
HIGH PRESSURE lOW PRESSURE

AORTA

Figure 8.10 Illustration depicting blood flow through a heart with levo transposition of the great
arteries.

53
Underdeveloped left side of the heart;
associated with ASO and ECG: Right ventricular hypertrophy
PDA for survival
Prostaglandin E required until surgery

Auscultate: Systolic murmur, ejection click

No division causes one giant artery; Pulse Oximetry: Low

associated with OiGeorge syndrome
ECG: Atrial enlargement, ventricular
hypertrophy, right axis deviation

Auscultate: Harsh holosystolic murmur LUSB

Pulmonic stenosis, right ventricular

CXR: "Boot-shaped" heart
hypertrophy, overriding aorta. VSO

Exertional cyanosis

Cyanosis persists with oxygen supplementation

Aorta arises from right ventricle:
pulmonary artery from left ventricle CXR: "Egg on a string"Prostaglandin E required
until surgery

Pulmonary veins do not connect

property to heart; associated with
Present
patent foramcn ovate or PDA
for survival

54

- NOTES
•
-
HEART FAILURE
GENERALLY.WHAT IS IT?
( PATHOLOGY & CAUSES
• A complex clinical syndrome characterized
by the heart's inability to effectively fill and/
or eject (pump) blood
• Stroke volume (SV): volume (ml) of blood
pumped by heart per contraction
• Cardiac output (CO): volume of blood
pumped by heart per minute (L/min)
° CO= SV X heart rate
• Preload: amount of blood in left ventricle
before contraction
• Afterload: stress on the ventricular wall
during systole
l systemic resistance, l blood viscosity,
O
aortic valve stenosis, ventricular dilation
----. i afterload
• lnotropy: cardiac contractility
• Ejection fraction (EF): % of blood leaving
heart during each contraction
O E= stroke volume ) pulmonary arterial hypertension
( end diastolic volume xlOO
• Frank-Starling mechanism: loading
ventricle with blood during diastole,
stretching out cardiac rnuscles=- more
forceful contraction; j SV during systole
Heart failure (HF) with reduced ejection
fraction (HFrEF)
• Systolic HF; "pump dysfunction"
• Causes: ! contractility/force of contraction
(e.g. myocardial infarction. myocarditis),
! blood supply to the heart (e.g.
coronary artery disease). j afterload
(e.g. hypertension). impaired mechanical
function (e.g. valve disease)
NOTES
• Normal preload, ! contractility (inotropy;
) force of contraction) ----. inadequate
emptying of ventricles during systole ----.
! EF s 40 (HFrEF); often also have some
degree of diastolic dysfunction
HF with preservedejection fraction
(HFpEF)
• Diastolic HF; "filling dysfunction"
• Causes: restrictive cardiomyopathy (e.g.
amyloidosis, sarcoidosis). valve disease,
hypertension
• Ventricles noncom pliant and unable to
fill during diastole ----. j filling pressures !
preload, normal contractilitv=-. ! SV----.
preserved EF ~ 50 (HFpEF)
TYPES
• Biventricular heart failure
O Left, right failure; systoliddiastolic
• Cor pulmonale
O Heart failure secondary to any cause of
• Left-sided heart failure
OImpaired ability of the left ventricle
to maintain adequate cardiac output
without an increase in left-sided filling
pressures
• Right-sided heart failure
O Impaired ability of the right ventricle to
deliver of blood flow to the pulmonary
circulation and l right atrial pressure
• Classification based on structure and
symptoms
O ACC/AHA HF Stages. NYHA Classes
(see table)
55
 A. At risk for HF without structural heart disease or
symptoms

B. Structural heart disease but without HF I. Asymptomatic

II. Symptomatic with moderate exertion

C. Structural heart disease with prior or current HF
symptoms
Ill. Symptomatic with minimal exertion

0. Refractory HF requiring advanced interventions (e.g.

implantable cardioverter defibrillator, biventricular IV. Symptomatic at rest
pacing, left ventricular assist device)

ACC - American College of Cardiology

AHA - American Heart Association
NYHA - New York Hospital Association

RISI( FACTORS • Arrhythmias

• Cardiac disorders: ischemic heart disease, • End organ damage: due to lack of perfusion
valvular heart disease, hypertension, LV • Liver damage (congestive hepatopathy)
hypertrophy, peripartum cardiomyopathy,
• Exacerbation
myocarditis, congenital heart disease,
= See mnemonic
chronic tachyarrhythmias
= Certain drugs may exacerbate HF;
• Other chronic diseases: hypertension,
e.g. NSAIDs, excessive doses of beta
diabetes, obesity, chronic lung disease,
blockers, calcium channel blockers,
infiltrative diseases (e.g. amyloidosis)
cyclophosphamide
• Toxins: cigarette smoking, ethanol,
cardiotoxic medications (e.g. doxorubicin,
amphotericin B); illicit drugs (e.g. MNEMONIC: FAILURE
amphetamines, cocaine)
Exacerbation of Heart failure
• High-output states: thyrotoxicosis, anemia
Forgot medication
• j age
Arrhythmia/Anemia
lschemia/lnfarction/lnfection
COMPLICATIONS Lifestyle (e.g. too much salt)
• Cardiogenic shock Upregulation of CO (e.g.
• Biventricular heart failure pregnancy, hyperthyroidism)
O Left/right-sided HF precursor/ Renal failure
complication of each other Embolism (e.g. pulmonary)

56
 OTHER DIAGNOSTICS
(__ s,_G_NS_&
__SY_M_PT_O_M_s
) • History and physical examination
identifying characteristic symptoms,
• High filling pressures: pulmonary edema, evidence of fluid retention and/or
dyspnea, orthopnea, exercise intolerance, hypoperfusion and functional impairment
paroxysmal nocturnal dyspnea (PND), due to cardiac dysfunction
basilar crackles, tachypnea, jugular venous
distention (JVD), hypoxemia, fatigue, ECG
peripheral edema, hepatomegaly, S3 • Identifies contributing rhythm disturbances
• Low cardiac output: tachycardia,
hypotension, cool extremities, I pulse
pressure, I urine output. I appetite

(..____ D_IA_GN_O_s,_s)
DIAGNOSTIC IMAGING
Warm&Wet
Chest X-ray (Compensated) (Congested)
• Detects cardiomegaly, chamber and vessel PCWP normal PCWP elevated
enlargement, pulmonary congestion, Cl normal Cl normal
presence of pericardia I and pleural
Cold & Dry Cold & Wet
effusions (Low flow state) (Decompensated)
PCWP low/normal PCWPelevated
Doppler echocardiography Cl decreased Cl decreased
• Evaluates hemodynamics related to in
valvular and biventricular function

Right heart (pulmonary artery) catheter-

ization (..____ T_R_E~_~_M_EN_T__ )
• Measures CO (cardiac index), filling
pressures, pulmonary capillary wedge MEDICATIONS
pressure (PCWP) • Individualized in accordance with New
York Heart Association (NYHA) class, EF,
MRI
comorbidities
• Visualizes ventricular volumes, mass,
• Angiotensin converting enzyme (ACE)
presence of myocardial remodeling
inhibitor or angiotensin II receptor blockers
(ARB)
LAB RESULTS • Beta-blocker (carvedilol, bisoprolol,
• j 8-type natriuretic peptide (BNP) and/or metoprolol ER)
N-terminal pro-BNP • Aldosterone agonist
• j serum creatinine and blood urea nitrogen • Mineralocorticoid receptor antagonist
(BUN) indicates glomerular filtration rate ! (HFpEF)
GFR due to hypoperfusion
• Acute decompensation
• j serum total bilirubin and aminotransferase
= See mnemonic
indicates congestive hepatopathy from
right-sided HF
• j serum lactate if cardiogenic shock
• Exercise testing: six-minute walk test
and/or a cardiopulmonary exercise test
measuring oxygen uptake (Vo2) evaluates
exercise capacity

57
 MNEMONIC: POND OTHER INTERVENTIONS
Acute decompensation • Lifestyle modifications
Position (upright) +I- positive , Low dietary salt, exercise as tolerated,
pressure ventilation (e.g. smoking cessation, minimize alcohol
Bi PAP) intake
Oxygen • Ventricular assist device (VAD)
Nitrates • Implanted defibrillator
Diuretics • Biventricular pacemaker for
resynchronization

SURGERY
• Heart transplant
° Considered in NYHA class of Ill or
IV despite maximized medical and
resynchronization therapy

COR PULMONALE
osms.i"l/eer _pulmone1le
• Recent surgery, hypercoagulable states (i
( PATHOLOGY & CAUSES ) risk of pulmonary embolism)

• Right ventricular hypertrophy, dilation,

and/or dysfunction due to pulmonary COMPLICATIONS
hypertension secondary to pulmonary • RVfailure
disease (e.g. chronic obstructive pulmonary • Liver dysfunction
disease (COPD). pulmonary fibrosis),
upper airway obstruction (e.g. obstructive
sleep apnea, obesity-hypoventilation
syndrome), or chest wall irregularities (e.g.
( SIGNS & SYMPTOMS )
kyphoscoliosis) • Dyspnea, chest pain, peripheral edema,
• Acute cor pulmonale develops in the jugular venous distension, hepatomegaly
setting of a sudden volume and/or pressure
overload in the right side of the heart; e.g.
massive pulmonary embolism ( D_IA_GN_o_s,_s )
• j pulmonary vascular resistance e- j
pulmonary circuit afterload ----. j right DIAGNOSTIC IMAGING
ventricular workload ----. right ventricular
Chest X-ray
hypertrophy or dilatation ----. impaired right
ventricular function and failure ----. j right • Visualizes right ventricular hypertrophy,
atrial pressure ----. fluid back-up into venous distended pulmonary vasculature,
circulation ----. peripheral edema pulmonary edema

Echocardiograph
y
RISI( FACTORS • Detects structural and functional changes
• Presence of parenchymal or vascular lung of right ventricle; estimates right ventricular
disease, chronic airway obstruction systolic pressures
• Smoking

58
MRI SURGERY
• Visualizes right ventricular hypertrophy, • Heart-lung transplant for resistant cor
right atrial enlargement, tricuspid valve pulmonale
dysfunction regurgitation, retrograde flow

Cardiac catheterization OTHER INTERVENTIONS

• j elevated central venous pressure, j • Treat underlying disease process
right ventricular, end-diastolic pressure, • Lifestyle
evidence of underlying pulmonary disease • Low dietary salt, exercise as tolerated,
smoking cessation

(..____ T_R_E~_iT_M_EN_T )
MEDICATIONS
• Supplemental oxygen
• Loop diuretic

DIASTOLIC HEART FAILURE

osmsJl/ dio.s-lolie-heo.T-l-fo.iluTe

( PATHOLOGY & CAUSES ) (__ s,_G_NS_&_S_Y_M_PT_O_M_s_)

• A clinical syndrome characterized by failure
of the heart to pump sufficient blood to
meet the metabolic needs of the body due
to ! ventricular filling
• HF with preserved ejection fraction
(HFpEF)
• Filling dysfunction
O Stiff, non-compliant ventricle - !
ventricular relaxation - r end diastolic
pressure - j resistance to filling -
! preload - EF ~ 50, ! SV, ! CO -
pulmonary congestion
RISI( FACTORS
• j age, restrictive cardiomyopathy (e.g.
amyloidosis, sarcoidosis); hypertrophic
cardiomyopathy, long-standing
hypertension, valve disease (especially
aortic stenosis), CAD, diabetes, obesity
COMPLICATIONS
• Arrhythmias, pulmonary embolism,
pulmonary hypertension, right ventricular
failure
• Fatigue, dyspnea, orthopnea, exercise
intolerance, pulmonary rales, JVD
( D_IA_GN_o_s,_s __ )
DIAGNOSTIC IMAGING
Chest X-ray
• Cardiomegaly; pulmonary vascular
congestion; enlargement of right atrium,
ventricle, and pulmonary arteries
Doppler echocardiography
• Altered mitral flow velocity, j LVEDP, LV
hypertrophy with concentric remodeling,
LA enlargement, l pulmonary artery
systolic pressure (PASP)
LAB RESULTS
• l BNP/NT-proBNP

59
 afratafreeh.com exclusive
OTHER INTERVENTIONS
( T_R_EA_:T_M_EN_T
) • Manage contributing factors and associated
conditions
MEDICATIONS • Lifestyle modifications
Alleviation of symptoms , Smoking cessation, ! sodium intake,
• Diuretics; antihypertensives weight management,! alcohol intake
O Beta blockers, ACE inhibitors, ARBs,
aldosterone antagonists

LEFT HEART FAILURE

osmsJl:/lef-1:-heo.T-l:-fo.iluTe
RISI( FACTORS
( PATHOLOGY & CAUSES ) • Coronary artery disease, infiltrative disease
(e.g. amyloidosis, hemochromatosis) -
• A clinical syndrome due to an alteration cardiomyopathy
of structure and/or function of the left
ventricle (LV) resulting in! cardiac output,
• Hypertension, aortic stenosis - r afterload
pulmonary congestion, and ! peripheral • Mitral or aortic regurgitation - r preload
perfusion • Exposure to toxins - myocardial damage
• Categorized according to left ventricular • Arrhythmias - ! filling, ! ineffective
ejection fraction (LVEF) contractions
O Systolic HF: ! LVEF ~40 percent (HFrEF) •age> 60
O Diastolic HF: preserved LVEF (HFpEF) • Obesity
• ! cardiac output - backup of blood into • Diabetes mellitus/metabolic syndrome
left atrium - pulmonary circulation -
j pressure in pulmonary capillaries - COMPLIC ATIONS
pulmonary edema - ! gas exchange,
• Pulmonary edema, pulmonary hemorrhage
dyspnea
(congested capillaries burst), pleural
• Neurohormonal compensatory mechanisms effusion, renal insufficiency
O RAAS and adrenergic activation
- renal salt and water retention +
vasoconstriction - l contractility, j (__ SI_G_NS_&_S_Y_M_PT_O_M_
circulating volume - j CO, j organ
perfusion • Exertional dyspnea, orthopnea; (PND),
O Adverse effects of compensation: l pulmonary edema (frothy, pink-tinged
afterload, t LV workload, LV remodeling sputum), bibasilar rales, cough, nocturia,
O Natriuretic peptide secretion occurs in restlessness, confusion. S3/S4
response to compensatory mechanisms
and atrial stretch - diuresis, natriuresis,
partial RAAS inhibition

60
 Echocardiography
• LV hypertrophy with eccentric remodeling,
l LVEDP, LA enlargement, t PASP

OTHER DIAGNOSTICS
• ECG
O Identifies contributing rhythm
disturbances

(.____ T_R_E~_~_M_EN_T )
Figure 9.1 The gross pathological appearance MEDICATIONS
of pulmonary edema. Exerting pressure on • Diuretics, beta blockers, ACE inhibitors,
the lung parenchyma causes frothy white ARBs, ARNI, hydralazine/nitrate
fluid to exude from it. combination, aldosterone antagonists
• Acute decompensation
= See mnemonic

( D_IA_GN_o_s,_s__ )
LAB RESULTS MNEMONIC: PONO
Acute decompensation
• i BNP/NT-proBNP
Position (upright) +/- positive
pressure ventilation (e.g.
DIAGNOSTIC IMAGING Bi PAP)
Chest X-ray Oxygen
• Cardiomegaly, pulmonary vascular Nitrates
congestion, enlargement of right atrium, Diuretics
ventricle, and pulmonary arteries

MEDICATIONS
• Diuretics, beta blockers, ACE inhibitors,
ARBs, ARNI, hydralazine/nitrate
combination, aldosterone antagonists
• Acute decompensation
O See mnemonic

SURGERY
• Heart transplant

OTHER INTERVENTIONS
• Manage contributing factors and associated
conditions
• Lifestyle modifications: smoking cessation,
! sodium intake, weight management, !
Figure 9.2 A plain chest X-ray image alcohol intake
demonstrating pulmonary edema. The • Cardiac rehabilitation
vessels at the hila are prominent and there
• Implantable cardioverter-defibrillator (ICD)
are numerous Kerley B lines.
• Ventricular assist device

61
Figure 9.3 The histological appearance of
pulmonary edema. There is flocculent fluid
within the alveolar spaces.
RIGHT HEART FAILURE
osms.i"l/Tighl-heo.T-l-fo.iluTe
( PATHOLOGY & CAUSES
• A clinical syndrome due to an alteration
of structure and/or function of the right
ventricle (RV) leading to suboptimal
delivery of blood flow to the pulmonary
circulation and/or elevated venous
pressures
• l venous pressure - systolic volume
overload
• l RV workload (most often due to
pulmonary congestion secondary to LV
failure) - RV hypertrophy - ! pumping
ability
CAUSES
• Left-sided heart failure, associated
pulmonary edema (most common cause),
right ventricular infarction, bacterial
endocarditis, pulmonic valve stenosis.
cardiomyopathy
Figure 9.4 Pitting edema in an individual with
left-sided heart failure.
COMPLICATIONS
) • Eventual failure of left side of heart
• Tricuspid regurgitation
• Congestive hepatopathy
• Cardiac cachexia
, Nausea. vomiting, anorexia, and diffuse
abdominal pain due to abdominal
venous congestion - weight loss
(__ SI_G_NS_&_S_Y_M_PT_O_M_
)
• JVD, hepatojugular reflux, fatigue (related
to poor gas exchange). exercise intolerance,
peripheral edema. hepatosplenomegaly,
ascites. S3/S4
( 0_1A_GN_o_s1_s)
DIAGNOSTIC IMAGING
Chest X-ray
• Cardiomegaly, pulmonaryvascular
congestion; enlargement of right atrium.
ventricle, pulmonary arteries
62
Echocardiography
• Evaluates RV size and function; detects
hemodynamic alterations

MRI
• Myocardial tissue, ventricular volume,
muscle damage

Right heart catheterization

• j pressure in heart chambers and lungs

LAB RESULTS
• i BNP/NT-proBNP
• j serum total bilirubin and aminotransferase
indicates congestive hepatopathy

OTHER DIAGNOSTICS Figure 9.5 A distended external jugular vein

• Clinical presentation: right heart (EJV) in an individual with right heart failure.
dysfunction, rule out left heart dysfunction

ECG
• Identifies contributing rhythm disturbances

( T_R_E~_~_M_EN_T__ )
MEDICATIONS
• Loop diuretics
Pulmonary edema Peripheral edema
° Fluid management
• Vasopressors
Bilateral basilar rales Hepatosplenomegaly
° Circulatory support

Orthopnea Hepatojugular reflux

OTHER INTERVENTIONS
• Treat underlying condition PND JVD

MNEMONIC: LMNOP
' Treatment for Right heart
failure
Las ix
Morphine
Nitrites
Oxygen
VassoPressors

63
NOTES

NOTES
• HEART VALVE DISEASE
GENERALLY.WHAT IS IT?
c PATHOLOGY & CAUSES ) c DIAGNOSIS )
• Wear and tear, external factors, varies by • Auscultation - echocardiogram,
type transesophageal echocardiogram,
• Older age, smoking, hypertension, catheterization
hyperlipidemia, diabetes mellitus,
connective tissue disorders, endocarditis,
heart attack c TREATMENT )
c • Lifestyle changes, pharmacotherapeutics,
SIGNS & SYMPTOMS ) surgical intervention

• Normally, heart valves keep blood moving

by opening for forward flow and closing to : 1st :2nd 3rd Atrial I
I
prevent backflow; symptoms evidence of A :t;. r, Normal ~
flow alterations I I
I I
• Murmurs, altered heart sounds I
I
I
I
• Sometimes asymptomatic B
I I Aortic stenosis I
I I I
• Advanced disease - heart failure

~·
I I I

• Left ventricular failure symptoms c I

I
~
I Mitra! regurgitation
:......
I
• Forward effects I I I

O Decreased perfusion to body tissues

(e.g. decreased perfusion to brain
D ,,, .:. I

I
I
I

:w,,,w.~,,_,,
: Aortic regurgitation
I

:,
I
= syncope; decreased perfusion to I I I

coronary arteries= chest pain, angina) E-..+

I

I
I
:.
I
I
. .,.. .... ~
Mitra! stenosi~ww :
I

• Backward effects I I I
I I
O Blood backs up to left atrium, into
pulmonary circulation (e.g. pulmonary
Patent ductus
edema. dyspnea, fatigue, paroxysmal arteriosus
nocturnal dyspnea)
Diastole: Systole Diastole :systole
• Right ventricular failure symptoms
• Backup of blood to venous circulation (e.g.
peripheral edema. hepatosplenomegaly) Figure 10.1 Illustration of phonocardiograms
from normal and abnormal heart sounds .

64
 AORTIC INSUFFICIENCY
osms.i"l/ o.or-lie-insuffieiene14
( PATHOLOGY & CAUSES )
• Widening/insufficiency of aortic valve
• Doesn't close fully, blood flows backwards
during diastole
• AKA aortic regurgitation
CAUSES
Aortic root dilation
• Root dilates, pulls apart leaflets
O Most root dilations idiopathic; some
caused by aortic dissection, aneurysm,
Marfan syndrome, Ehlers-Danlos
syndrome, syphilis, ankylosing
spondylitis, rheumatoid arthritis,
systemic lupus erythematosus
O Valvular damage: infective endocarditis,
rheumatic fever, bicuspid aortic valve
O Inflammation - fibrosis - valve can't
seal
Acute aortic regurgitation (medical emer-
gency)
• Infective endocarditis, trauma, aortic
dissection
O Acute aortic regurgitation presents
with sudden cardiovascular collapse,
pulmonary edema
° Chronic aortic regurgitation presents
less urgently, signs of heart failure
RISI( FACTORS
• Hypertension, syphilis, genetic disorders
(Marfan's syndrome, Ehlers-Danlos
syndrome)
COMPLICATIONS
Heart failure
• High blood volume left ventricle - left
ventricle compensates, adding sarcomeres
in series - eccentric left ventricular
hypertrophy - left ventricular dysfunction
- heart failure
( SIGNS & SYMPTOMS )
Abnormal heart sounds
• Early decrescendo diastolic murmur, usually
heard at left lower sternal border/apex
• Systolic flow murmur may develop in
chronic aortic regurgitation; increased
blood flow through valve during systole,
regardless of stenosis
Wide pulse pressure
• Increased systolic blood pressure (SBP) and
decreased diastolic blood pressure (DBP) =
hyperdynamic circulation
• Calculation for pulse pressure (PP)
• SBP - DBP = PP
• Hill's sign
• Exaggerated difference in SBP when
comparing upper, lower limbs
• Bounding pulses
• Evidence of wide PP
• Corrigan pulse (water-hammer pulses):
bounding pulse, blood hammers against
arterial walls
Other signs
• de Musset's sign
• Head bobs in time with heartbeat
• Quincke's sign
O Light compression of capillary bed leads
to visible pulsations in fingers
65
• Traub's sign OTHER DIAGNOSTICS
O Pistol shot sound head over femoral
Electrocardiogram
arteries
• Shows non-specific features of left
• Duroziez's sign
ventricular hypertrophy
O Systolic, diastolic bruit over femoral
artery when partially compressed
• Landolfi's sign ( T_R_E~_rT_M_EN_T__ )
O Diastolic pupil dilation
• Goal: improve cardiac output, decrease
Acute aortic regurgitation regurgitant flow volume
• Severe dyspnea, chest pain, hypotension =
left ventricular failure, cardiogenic shock
MEDICATIONS
• Vasodilators to reduce afterload
( D_IA_GN_o_s,_s__ )
SURGERY
DIAGNOSTIC IMAGING • Surgical valve replacement
Echocardiograph
y • Surgical replacement once ejection fraction
• Using Doppler flow, observe regurgitation < 55%
jet through aortic valve during diastole

Chest X-ray
• Nonspecific, may observe cardiomegaly

AORTIC STENOSIS
osms.i"l/ o.or-lie-s-lenosis

( PATHOLOGY & CAUSES ) COMPLICATIONS

• Heart failure, microangiopathic hemolytic
anemia (red blood cells damaged as they
• Stiffening, thickening/calcification of aortic
squeeze through small valve opening),
valve (no longer opens fully during systole)
Heyde's syndrome
• Valve opening narrows - pressure
gradient increases across valve

CAUSES
( SIGNS & SYMPTOMS )
Asymptomatic
Mechanical stress
• Due to slow progression; abnormal heart
• Damaged endothelial cells over time - sounds heard on auscultation
fibrosis and calcification - stiff valve does O Ejection click
not open fully
O Harsh, systolic, crescendo-decrescendo
Rheumatic heart disease systolic murmur at upper sternal border,
• Repeated inflammation, repair - fibrosis radiating to carotids
- commissural fusion
Advanced state aortic stenosis
• Classic triad: angina, syncope, exertional
dyspnea

66
• Additional heart sounds: soft, single
S2/paradoxical S2 split; crescendo- ( T_R_E~_iT_M_EN_T )
decrescendo systolic murmur peaks later
(the later the peak, the more severe the MEDICATIONS
stenosis); S4 • Venodilators, calcium channel blockers,
• Pufsus parvus et tardus (pulse weak, administer beta blockers with caution
delayed)
• Narrowed pulse pressure SURGERY
• Surgical valve replacement if necessary

MNEMONIC: SAD
' Characteristi
cs of Aortic
OTHER INTERVENTIONS
• If mild, no exercise restrictions; if severe,
stenosis
reduced physical activity
Syncope
Angina
Dyspnea

( D_IA_GN_O_s,_s
__ )
DIAGNOSTIC IMAGING
Transthoracic echocardiogram (TTE)
• Observe small aortic orifice during systole,
increased pressure gradient across valve,
left ventricular hypertrophy, calcification of
aortic valve Figure 10.2 Gross pathology of severe aortic
stenosis as a consequence of previous
Cardiac catheterization
rheumatic heart disease. The valve leaflets
• Useful for surgical planning are stiffened and fused resulting in a
narrowed lumen.
OTHER DIAGNOSTICS
Electrocardiogram
• Shows non-specific features of left
ventricular hypertrophy

Figure 10.3 Gross pathology of a nodular

bicuspid aortic valve.

67

Mechanical stress over time.
rheumatic heart disease
Small opening - increased pressure
Congestive heart failure.
microangiopathic hemolytic anemia.
Heyde's syndrome
Age. bicuspid aortic valve.
rheumatic fever
Asymptomatic for extended period,
late triad of angina. syncope,
exertional dyspnea
Ejection click. harsh systolic,
crescendo-decrescendo systolic
murmur, radiation to carotids
Nonspecific changes.
Left ventricular hypertrophy
Observe small orifice (during systole)
Left ventricular hypertrophy
Mild:watch
Severe: valve replacement
MITRAL INSUFFICIENCY
osmsJl/ mi-lTe1l-insuffieien14
( P_AT_H_Ol_O_G_Y _&_C_AU_S_Es ) disorders (e.g. Marfan syndrome,
• Mitral valve prolapses (falls back into
atrium)
• Most common valvular condition
• AKA mitral regurgitation
CAUSES
Myxomatous degeneration
• Leaflets, connective tissue, surrounding
tissue weakened °""' mitral valve prolapse
CREAM
Congenit.il Rheumatic damaqe.
Endocarditis, Aortic dissection,
Aortic root dilation, Marfa n's
Valve improperly closes
- backward flow
Congestive heart failure
Hypertension. syphilis,
Marfan's. Ehlers-Danlos
Severe dyspnea. chest pain,
hypotension
Blowing decrescendo diastolic
murmur best at left lower sternal
border or apex
Nonspecific changes.
Left ventricular hypertrophy
Doppler shows regurgitation
during diastole
Mild:watch
Severe: valve replacement
, Associated with connective tissue
Ehlers-Danlos Syndrome)
, Causes larger valve leaflet area,
elongation of chordae tendineae °""'
mitral valve more prone to rupture
(rupture usually happens to chordae
tendineae on posterior leaflet, leaflet
folds up into left atrium)
, Doesn't always cause mitral
regurgitation but often does since blood
will leak backwards into left atrium if
leaflets don't form perfect seal
68
Damage to papillary muscles • Atrial fibrillation
• Caused by heart attacks O Left atrium dilates c- muscle walls
O Papillary muscle dles-« can't anchor stretch, pacemaker cells irritated
chordae tendineae ---'> mitral valve flops • Thrombus formation, embolism
back-» blood leaks back into left atrium O Atrial fibrillation ---'> blood stagnates,
pools---'> increased risk of thrombus
Left-sided heart failure
formation, blood clots ---'> goes to
• Left sided heart fallure=-s left ventricle systemic circulation
dllates e- stretches mitral valve annulus
• Dysphagia
(ring) ---'> blood leaks back into left atrium ---'>
O E.g. difficulty swallowing solid foods;
ventricular dilation
dilated atrium compresses neighboring
Rheumatic fever esophagus
• Inflammatory disease affecting heart tissue,
leading to chronic rheumatic heart disease
• Chronic inflammation ---'> leaflet fibrosis-«
(__ s,_G_NS_&_S_Y_M_PT_O_M_s )
leaflets cannot form complete seal ---'> blood
• Clinical manifestations of heart failure (e.g.
leaks through
fatigue, swelling, rapid heartbeat)
Mitral regurgitation • Holosystolic murmur
• Can also cause left-sided heart failure • Lasts for duration of systole
• Regurgitant flow back into left atrium---'>
increased preload ---'> increased workload
on left atrium, ventricle ---'> left eccentric ( D_IA_GN_O_s,_s
__ )
hypertrophy (new sarcomeres added in
series to existing ones) ---'> left sided heart DIAGNOSTIC IMAGING
failure
Transthoracic echocardiograph
y (TTE) or
transesophageal echocardiogram (TEE)
RISI( FACTORS • Enlarged left atria/ventricle
• Intravenous (IV) drug use (increases • Rupture/tear/elongation of mitral valve
likelihood of infective endocarditis) chordae
• Congenital bicuspid aortic valve (baby born • Regurgitation (seen as retrograde blood
with aortic valve that has only two instead flow on Doppler imaging)
of three leaflets)
• Systolic bowing of mitral leaflet (>2mm
• Diabetes, high blood pressure, smoking beyond annular plane)
• May reveal leaflet thickening, flail leaflet,
COMPLICATIONS annular dilation
• Pulmonary congestion, edema
Chest X-ray
° Constant elevation in blood volume,
• May demonstrate cardiomegaly secondary
pressure in left atrium causes dilation
to left atrial/ventricular dilation
---'> blood backs up into pulmonary
circulation
• Pulmonary hypertension OTHER DIAGNOSTICS
O Extra blood volume, pressure in left
ECG
atrium backs up into lung causing higher
• Abnormal findings often observed in MVP
pressure in pulmonary circulation
• Right-sided heart failure
O Early repolarization in inferior leads
OST depression, QTc prolongation
O Backup of blood in left atrium, lungs
---'> pulmonary hypertension---'> right OPremature ventricular contractions
ventricular hvpertrophv -e right-sided • Not conclusive; result can be normal in
heart failure people who have mild mitral valve disease

69
 , Reshape valve tissue to create tighter
( T_R_E_AT_M_EN_T__ ) seal
, Repair tears to increase support at base
MEDICATIONS of valve
• Lower high blood pressure (e.g. diuretics)
, Replace with prosthetic valve
• Lower cholesterol (e.g. statins)
• Prevent arrhythmias (e.g. amiodarone)
• Prevent clots with blood thinners/
MITl!Al VALVf;.
anticoagulants (e.g. heparin, warfarin)
• Treat heart failure (e.g. digoxin to increase
contractility)

SURGERY 010(~\t IV OPttJ

Replacing/repairing valve
• Severe mitral regurgitation or stenosis =
valve repair or surgical replacement of valve
Jot~~-t
T OpCA'l
a.II ~t WA'}
O Separate fused valve flaps i
4iT£MOSI~

Figure 10.4 Illustration depicting differences

in mitral valve shape between mitral valve
insuffiency (regurgitation) and mitral stenosis.

MITRAL STENOSIS
osms.tl/ mi-l,-o.1-s-lenosis
• Diabetes, high blood pressure, smoking
( PATHOLOGY & CAUSES )
• Narrowing of mitral valve COMPLICATIONS
• Rheumatic fever: inflammation ----. leaflets • Pulmonary congestion, edema
fuse together (commissural fusion) ----. , Constant elevation in blood volume,
prevents seal formation pressure in left atrium ----. left atrium
O Normal mitral valve opening duates=- blood backs up into
(4-6cm2/1.6-2.4in2) narrows to pulmonary circulation
2cm2/0.8in2 • Pulmonary hypertension
O Smaller opening ----. harder for blood OExtra blood volume, pressure in left
to flow from left atrium to ventricle atrium backs up into lung ----. higher
----. blood backs up in atriurn-e- higher pressure in pulmonary circulation
pressure in left atrium • Right-sided heart failure
O Backup of blood in left atrium, lungs
RISI( FACTORS ----. pulmonary hypertension ----. right
ventricular hvpertrophv-« right-sided
• IV drug use
heart failure
O Increases likelihood of infective
• Atrial fibrillation
endocarditis
, Left atrium duates=- muscle walls
• Congenital bicuspid aortic valve

70
 stretch, pacemaker cells irritated OTHER DIAGNOSTICS
• Thrombus formation, embolism
ECG
O Atrial fibrillation - blood stagnates,
• Reveals abnormal electrical activity
pools - increased risk of thrombus
depending on severity
formation, blood clots entering systemic
circulation , Not conclusive; result can be normal
in people who have mild mitral valve
• Dysphagia
disease
O Dilated atrium compresses neighboring
esophagus
( TR_E_~_irM_E_N_T__ )
(__ SI_G_NS_&_S_Y_M_PT_O_M_s_) MEDICATIONS
• Lower high blood pressure (e.g. metoprolol,
• Clinical manifestations of heart failure
lisinopril, diuretics)
• "Snap" sound after S2 (closure of aortic,
• Lower cholesterol (e.g. statins)
pulmonic valves)
• Prevent arrhythmias (e.g. amiodarone)
O Higher pressure flowing through fibrotic
valve makes "snap" sound when valve • Prevent clots with blood thinners/
anticoagulants (e.g. heparin, warfarin)
opens
O Diastolic rumble following "snap" as • Treat heart failure (e.g. digoxin to increase
blood forced through smaller opening contractility)

• Dyspnea/difficulty breathing
O Pulmonary congestion, pulmonary SURGERY
edema • Replacing/repairing valve: severe mitral
regurgitation or stenosis = valve repair or
surgical replacement of valve
( D_IA_GN_O_s,_s
__ ) = Separate fused valve flaps
= Reshape valve tissue to create tighter
DIAGNOSTIC IMAGING seal
Echocardiograph
y = Repair tears to increase support at base
of valve
• Shows abnormal blood flow, narrowed/
insufficient valve = Replace with prosthetic valve

Transesophageal echocardiogram (TEE)

• Enlarged left ventricle
• Enlarged left/right atria
• Possible rupture/tear of mitral valve
chordae
• Possible regurgitation

Stress test (echocardiography)

• Measure blood pressure pre-, post-test
• Record how long individual able to carry
out test

Chest X-ray
• Shows heart size, lung condition Figure 10.5 Gross pathology of a stenotic
mitral valve, viewed from the left atrium.

71
 MITRAL VALVE PROLAPSE
osms.i"l/mi"lTo.1-vo.lve-pTolo.pse
against closed airway), click comes
( PATHOLOGY & CAUSES ) sooner, longer murmur
, Standing reduces venous return ----.
• Floppy mitral valve
less blood in ventricle ----. ventricle is
• Cusps of valve flop into atrium during slightly srnaller=-s less room for leaflets
systole. ----. leaflet forced out earlier during
• Myxomatous degeneration from connective contraction
tissue disease (e.g. Ehler-Danlos, Marfan • Individual may report palpitations
syndromes)
• Familial mitral valve prolapse
• Autosomal dominant: variable (..____ D_IA_GN_O_s,_s
)
penetrance and expression
DIAGNOSTIC IMAGING
RISI( FACTORS Chest X-ray
• Age • May demonstrate cardiomegaly secondary
• Hypertension to left atrial/ventricular dilation
• History of rheumatic fever
Transthoracic echocardiography (TIE) or
• Connective tissue disorders
transesophageal echocardiogram (TEE)
• Enlarged left atria/ventricle
COMPLICATIONS • Rupture/tear/elongation of mitral valve
• Heart failure, arrhythmias, systemic emboli, chordae
cardioembolic stroke, chordal rupture, • Regurgitation (seen as retrograde blood
sudden death flow on Doppler imaging)
• Systolic bowing of mitral leaflet (> 2mm
beyond annular plane)
(__ s,_G_NS_&
__SY_M_PT_O_M_s ) • May reveal leaflet thickening, flail leaflet,
annular dilation
• Usually asymptomatic
• Classic heart murmur: midsystolic click
followed by systolic murmur OTHER DIAGNOSTICS
• Murmur: blood leaks backward from left Physical examination
ventricle into left atrium
• Crescendo murmur in late systole heard
• Click: leaflet folding into atrium, suddenly over apex
stopped by chordae tendineae
• Mid-systolic click {due to rapid tensing of
o When an individual squats, click comes chordae tendineae)
later, shorter murmur
o Squatting increases venous return ----. ECG)
fills left ventricle with more btooc -» left • Abnormal findings often observed in MVP
ventricle gets slightly larger----. leaflets n Early repolarization in inferior leads
have more space ----. ventricle contracts,
, ST depression, QTc prolongation
gets srnaller e- takes slightly longer for
n Premature ventricular contractions
leaflet to be forced into atrium
• Not conclusive; result can be normal in
o When individual stands/performs
people who have mild mitral valve disease
Valsalva maneuver (forceful exhalation

72
(..__ __ T_R_E~_iT_M_EN_T )
MEDICATIONS
• If palpitations present
O Beta blockers; avoid smoking, caffeine

SURGERY
• Severe prolapse
O Valve repair/replacement (esp. when left
ventricular systolic function impaired)
Figure 10.6 Gross pathology of a mitral valve
prolapse (anterior superior leaflet) viewed
from the left atrium.

PULMONARY INSUFFICIENCY
osms.i"l/ pulmonie-insuffieie ne14

( PATHOLOGY & CAUSES ) (__ SI_G_NS_&_SY_M_PT_O_M_s )

• Pulmonary valve doesn't close fully----. • Abnormal heart sounds
blood leaks back into right ventricle ° Crescendo-decrescendo murmur: blood
• AKA pulmonic regurgitation flows through narrow pulmonary valve,
• Blood backflow increases right ventricular causes turbulence that gets louder as
blood volume ----. right ventricle needs to more blood flows/quieter as blood flow
work harder during systole ----. eccentric slows, blood leaks back from pulmonary
ventricular hvpertrophv -« heart failure artery into right ventricle, causes
murmur that starts loud, quietens
• Signs of right-sided heart failure may
CAUSES be present (e.g. fatigue, swelling, rapid
• Congenital malformation of the leaflets heartbeat)
common
O Tetralogy of Fallot (TOF), Noonan's
syndrome, congenital rubella ( D_IA_GN_O_SI_S __ )
• Infective endocarditis, rheumatic heart
disease, systemic disease (e.g. carcinoid DIAGNOSTIC IMAGING
disease)
Echocardiogram
• Regurgitation seen on Doppler
COM PLICATtoNS
• Right-sided heart failure Chest X-ray
O Ventricles cannot compensate for • May show enlarged right ventricle
increased workload
• Microangiopathic hemolytic anemia
O Shearing damage to red blood cells
forced through smaller valve, leading to
hemoglobinuria

73
 (..____ T_R_E~_iT_M_EN_T
)
SURGERY
• Valve replacement if symptomatic

Figure 10.7 Illustration depicting decrescendo

murmur as blood flows back into the right
ventricle.

PULMONARY STENOSIS
osms.i"l/ pulmonie-s-lenosis

( PATHOLOGY & CAUSES ) (__ SI_G_NS_&_SY_M_PT_O_M_s )

• Pulmonary valve doesn't open fully; harder • Initially asymptomatic
for right ventricle to pump blood to lungs • Diastolic crescendo-decrescendo murmur:
• Mechanical stress over time abnormal heart sound caused by turbulent
, Damages endothelial cells around blood flow through pulmonary valve
that does not close properly; starts loud,
valves - fibrosis, calcification -
hardens valve, makes it more difficult to quietens
open fully • Ejection click: valve resists, then finally
• Eccentric right ventricular hypertrophy: snaps open
right ventricle must compensate for larger • Appears often as right-sided heart failure
amount of blood volume due to backflow of
blood
(..____ D_IA_GN_O_SI_S
)
CAUSES DIAGNOSTIC IMAGING
• Congenital malformation of leaflets
, Associated with tetralogy of Fallot, Echocardiogram
Noonan's syndrome, congenital rubella • Thickened leaflets, hard to see location of
• Systemic disease (e.g. carcinoid disease) stenosis

RISI( FACTORS ( T_R_E~_iT_M_EN_T )

• History of rheumatic heart disease, heart
surgery, or infective endocarditis • Balloon valvuloplasty
• Valve replacement if symptomatic right-
COMPLICATIONS sided heart failure
• Right-sided heart failure
, Right ventricle cannot compensate for
increased force required to push blood
through valve

74
 «M115T C.UJE.IZ~T£
Hl4H PP.€SSVflLS
J
C.ONCE.>JTJIC fll<:tHT
V£NTfZ.IC.U~Alt H'tP£fZ.lllOPH'(

Figure 10.8 Illustration depicting hypertrophy

of right ventricle due to increased blood
pressure in the right ventricle.

TRICUSPID INSUFFICIENCY
osms.i"l/-l,-ieus id-insu ffieie ne14
• Infective endocarditis
( PATHOLOGY & CAUSES ) • Trauma
° Catheter insertion
• Cusps of valve prolapse during systole -
blood backs up into right atrium. O Endocardial pacemaker insertion
• AKA tricuspid regurgitation
O Blunt chest trauma

CAUSES RISI( FACTORS

• Rheumatic heart disease • Disease processes may cause pulmonary
O Most
hypertension
common cause
• IV drug abuse
Autoimmune reaction involving valve
O

leaflets - chronic inflammation -

leaflet fibrosis - valve unable to form COMPLICATIONS
seal • Heart failure
• Myocardial infarction O Increased ventricular preload -
O Papillary muscles malfunction - eccentric ventricular hypertrophy -
destroyed papillary muscles can't anchor right ventricular failure
chordae tendineae - blood flows from • Ventricular hypertrophy
right ventricle to right atrium O Structural change in heart - annulus
• Pulmonary hypertension stretches - more blood leakage -
O Increase in right ventricular pressure - worsens regurgitation
dilates tricuspid valve - blood flows
backward
• Congenital causes
, Leaflets are displaced - difficult to form
seal (e.g. Ebstein anomaly)
• Carcinoid syndrome
, Fibrous tissue deposited on valves
• Myxomatous valve degeneration

75
(__ SI_G_NS_&_SY_M_PT_O_M_s ) (..____ D_IA_GN_O_SI_S )
• Holosystolic murmur DIAGNOSTIC IMAGING
O Movement of blood heard throughout
Echocardiogram with Doppler
systole
• Shows backflow
• Carvallo's sign
O Murmur gets louder with inspiration X-ray
due to negative pressure in chest, more • Shows right ventricular enlargement
blood backs up into heart
• S3, S4
• Signs of right-sided heart failure (..____ T_R_E~_rl"_M_EN_T
)
SURGERY
• Surgical repair/replacement if symptomatic

TRICUSPID STENOSIS
osms.tl/-l:Tieuspid-s-1:enests

( PATHOLOGY & CAUSES ) (__ SI_G_NS_&_SY_M_PT_O_M_

• Valve unable to open completely during • Ejection click
diastole. ° Fibrotic valve makes distinctive snap
• Valve leaflets fuse (commissural fusion) - • Diastolic rumble
narrowing oftricuspid valve - impaired O As blood is forced through small valve
blood flow from right atrium to right opening
ventricle
• Increased ventricular preload - right
ventricular failure - signs of congestion in
CAUSES venous system
• Rheumatic heart disease O Jugular venous distention (JVD)
O Most common cause may cause some individuals to feel
° Can occur with mitral regurgitation, uncomfortable fluttering in neck
aortic valve disease
• Congenital atresia, stenosis
(..____ D_IA_GN_O_SI_S )
• Pacemaker-induced fibrosis
• Cardiac tumors DIAGNOSTIC IMAGING
• Infective endocarditis
Echocardiography
• Assess degree of leaflet damage, flow
COMPLICATIONS across valve
• Increased right atrial volume. pressure -
atrial dilation - blood backs up into venous
circulation OTHER DIAGNOSTICS
• Dilation of right atrium - muscle walls Cardiac catheterization
stretch - pacemaker cells become irritable
• Measure pressure in right side of heart
- increases risk of atrial flutter. fibrillation

76
(..____ T_R_E~_iT_M_EN_T )
SURGERY
• Balloon valvuloplasty, valve repair/
replacement

TJIC.USPIP VAL'IE.... H0Lorvs101,.1e, ~V/lMv,-

,-; l2.i~ul261TAT IOtJ ,v ~

SI S2.
PuL~O>Jf\'2'1' s-: T~eATfO w,tLv

a
l'-Hfll~ATIC.
J Rlu"4T-SID~D
HE.A'2T FAI LV~E.
HcAIZT 01sfAS~ IV ST£Nos,s,..,

~OllTIC. VALVl
..... 1Tf2AL VAi.Ve

St
Figure 10.9 Illustration depicting differences between tricuspid valve regurgitation and tricuspid
valve stenosis.

77
 Upper sternal border ..... Paradoxical 52 split, 54,
crescendo-decresendo
radiates to carotids narrow pulse pressure
with ejection click

Early decrescendo Left lower de Musset's Sign,

diastolic sternal border Quincke's sign

Apex: radiates to the Diminished Sl,

Holosystolic murmur
back or clavicular area wide 52. 53 gallop

52 with opening snap

Left lateral decubitus High left
then low-pitched
position with bell atrium pressure
diastolic rumble

Standing increases
Midsystolic click
Apex murmur, squatting
..... systolic murmur
decreases

Left upper
Crescendo-decrescendo NIA
sternal border

Diastolic Right upper

Ejection click
crescendo-decrescendo sternal border

Blowing holosystolic Left lower

Carvallo's sign, 53, 54
murmur sternal border

Left lower
Diastolic rumble Ejection click
sternal border

78
 NOTES

- NOTES
•
-
HYPERTENSION s HYPOTENSION

GENERALLY,WHAT ARE THEY?

( PATHOLOGY & CAUSES ) ( D_IA_GN_O_s,_s
__ )
• Changes of blood pressure above (hyper-) OTHER DIAGNOSTICS
or below (hypo-) normal (120/80mmHg) • Blood pressure cuff (sphygmomanometer)
or arterial catheter
CAUSES
• Mostly idiopathic, but can include various
causes: impaired regulatory pathways
( T_R_E~_~_M_EN_T
)
(hormonal/neurologic disorders), heart
• Hypotension: generally requires no
disease, kidney disease, medications etc.
treatment
• Hypertension: treated according to degree,
treat underlying cause if present
(__ SI_G_NS_&_S_Y_M_PT_O_M_s_)
• Can be asymptomatidinclude symptoms MEDICATIONS
according to underlying cause, degree of • Hypertension: beta blockers, diuretics, ACE
change in blood pressure inhibitors, calcium channel blockers, etc.
O Hypertension: range from headache,
dyspnea, to blurred vision, oliguria,
seizures
O Hypotension: range from fatigue, pallor
to syncope

r' r r r r
MtCaH PllE.SSU~t
.. 1 1 1 l
€tJDOTH€ LI Al..
Cf;LLS DAM~6E.D
L_. t-1\'t'OC.A~DIF'l.INF'A~c..l ION I

At-J~U~'w'SI"\ I ~ STP.O-'E.

Figure 11.1 Illustration depicting endothelial damage caused by hypertension .

79
 afratafreeh.com exclusive

HYPERTENSION
osms.i-l/h14pe,--l:ension
glomerular filtration rate - ! secretion of
( PATHOLOGY & CAUSES ) sodium + water - j volume - j blood
pressure
• Condition in which blood pressure is
• Preeclampsia/eclampsia in pregnancy:
regulated maladaptively, elevating blood
mechanism unknown
pressure over 140/90mmHg
• Coarctation of the aorta: low pressure
• Isolated systolic hypertension: systolic
past coarctation - low renal perfusion -
blood pressure is elevated, diastolic is not
activation of renin angiotensin-aldosterone
• Isolated diastolic hypertension: diastolic system (RAAS) - secondary hypertension
blood pressure is elevated, systolic is not
• Cushing's syndrome: combination of
several pathophysiological mechanisms
CAUSES (e.g. elevated cortisol) that regulate plasma
volume, cardiac output, peripheral vascular
Primary/essential hypertension resistance
• Most cases (90%) • Chronic kidney disease: fluid overload,
sodium retention
Secondary hypertension
• Known etiology, often reversible
• Renovascular hypertension: anything
MNEMONIC: RHNECCk
partially obstructing blood flow to
Causes of Secondary
kidneys (e.g. atherosclerosis, vasculitis,
hypertension
fibromuscular dysplasia).
Renovascular hypertension
O Decreased blood flow to kidneys -
kidneys secrete renin - renin converts 1 ° Hyperaldosteronism
angiotensinogen to angiotensin I - NSAIDs
angiotensin converting enzyme converts Pre-Eclampsia I Eclampsia
angiotensin I to angiotensin II (active) - Coarctation of the aorta
angiotensin II effects:
Cushing's syndrome
O Vasoconstriction: directly increases
Kidney Disease (chronic)
blood pressure
O Stimulation of sodium reabsorption:
increases water reabsorption RISk FACTORS
O Stimulation of adrenal cortex to release
aldosterone from adrenal cortex - Primary hypertension
aldosterone increases reabsorption of • Risk increases with age, biological male,
sodium + water - increased volume - obesity, stress. sedentary lifestyle, family
high blood pressure history of hypertension
• Primary hyperaldosteronism: increased • Race (in decreasing order of risk): African
aldosterone - increased reabsorption of descent> white European descent > Asian
sodium + water - increased volume - descent
increased blood pressure • Diet: excessive sodium, alcohol intake
• Nonsteroidal anti-inflammatory drugs • Abnormal lipid panel (high low-density
(NSAIDs) - inhibit cyclooxygenase in lipoproteins. low high-density lipoproteins,
kidneys - ! production of PGE-2 (renal high triglycerides)
vasodilator) - vasoconstriction of afferent
arterioles in kidneys - ! renal blood flow,

80
Secondary hypertension
• Atherosclerosis: Elderly biological males
(__ SI_G_NS_&_S_Y_M_PT_O_M_s_)
O Atherosclerosis ----. renal stenosis ----. less
• Vast majority of cases asymptomatic
blood flow to renal artertes=- activation
• May experience headache, dyspnea
of RAAS ----. renovascular hypertension
• Renal bruit in secondary hypertension due
to renal artery stenosis
COMPLICATIONS • Hypertensive retinopathy
• Increased risk of atherosclerosis,
arteriosclerosis
• Arteriolar rarefaction: loss of arterioles ( D_IA_GN_O_SI_S
__ )
• Coronary artery disease, left ventricular
hypertrophy, atrial fibrillation, stroke, OTHER DIAGNOSTICS
hypertensive nephropathy, retinopathy, • Non-invasive/invasive blood pressure
aortic dissection, aneurysms monitoring
• High blood pressure: at least 2 separate
measurements with blood pressure
> 140/90mmHg

StSTOLIC. 01ASTOLIC.
GlOOl) p~E.SSIJ~l BLOOD PIZ£.SSVl2E.

STACIE. 1

Figure 11.2 Illustration depicting stages of hypertension.

81

Figure 11.3 Retinal photograph
demonstrating changes of hypertensive
retinopathy (AV nipping and tortuous
vessels).
Figure 11.4 Histological appearance of
renal artery hyalinosis; a manifestation of
hypertensive renal disease.
( T_R_EA_~_ME_N_T )
• Reduce BP <140/90mmHg with lifestyle
modification first, then medical treatment
MEDICATIONS
Monotherapy/together
• Thiazide-type diuretics: reduce blood
volume by increasing excretion of sodium,
water
• Angiotensin-converting enzyme (ACE)
inhibitors: block ACE from converting
angiotensin I to angiotensin II - blocks
RAAS - dilates arteries, decreases blood
volume
, Lower levels of angiotensin II -
vasodilation
• Dihydropyridine calcium channel blockers:
disrupt movement of calcium through
calcium channels in blood vessel walls -
vasodilation
Other agents
• Angiotensin II receptor blockers (ARBsJ:
prevents the vasoconstrictive effects of
angiotensin II by blocking its receptors -
lowering blood pressure
, Used when individuals get a cough from
ACE inhibitors
• Beta receptor blockers: decrease
contractility, heart rate
• Alpha-2 agonist: stimulates alpha-2
receptors - decreases sympathetic activity
- decreased blood pressure, heart rate
• Renin inhibitor: aliskiren
, Lower levels of angiotensin I
• Hydralazine: elicits direct vasodilation
of vascular smooth muscle, useful in
pregnancy
OTHER INTERVENTIONS
• Low sodium diet, exercise, quit smoking,
limit alcohol, maintain healthy weight
82
 HYPERTENSIVE EMERGENCY
osmsJl:/h14 eT-lensive-emeTgene14

( PATHOLOGY & CAUSES ) ( D_IA_GN_o_s,_s __ )

• Elevated blood pressure> 180/120mmHg OTHER DIAGNOSTICS
with signs of acute end organ damage • Sphygmomanometer
(e.g. encephalopathy, stroke, papilledema, = Blood pressure> 180/120mmHg
myocardial infarction, heart failure,
• Evaluation to identify at-risk target organ
microangiopathic hemolytic anemia, etc.)
• Electrocardiography {heart)
• Complication of poorly managed
= Chest X-ray (heart, lungs)
hypertension
• Urinalysis (kidneys)
= Serum electrolytes, serum creatinine
RISK FACTORS {kidneys)
• Kidney failure, renovascular hypertension,
• Cardiac enzymes {heart)
stimulant abuse, medication non-adherence
= CT scan of brain (if brain suspected --->
• More common in young adults, particularly
neurologic symptoms, retinopathy)
those of African descent
• Contrast-enhanced CT scan of chest (if
aortic dissection suspected)
COMPLI CATIONS
• Neurological complications (stroke,
seizures), myocardial infarction, kidney ( T_R_EA_iT_M_EN_T__ )
failure, permanent blindness, pulmonary
edema • Treatment varies case-by-case, dependent
on affected organ

(__ SI_G_NS_&_S_Y_M_PT_O_M_s_)
MEDICATIONS
• Unwise to lower blood pressure too quickly/
• Blood pressure> 180/120mmHg, signs of
too much, as this can lower cerebral
end-organ damage
perfusion excessively
O Blurred vision, altered mental state,
• Most cases: mean arterial pressure
chest pain, headache, nausea,
(MAP) should be reduced using
vomiting, numbness in extremities,
intravenous medication 10-20 % in first
oliguria, seizure, dyspnea, weakness,
hour, then 5-15% over the following
papilledema
23 hrs. Specific medications used
dependent on case

OTHER INTERVENTIONS
• Exceptions to most cases: acute phase
ischemic stroke (not lowered unless specific
conditions met)
• Acute aortic dissection (rapid lowering),
intracerebral hypertension (variable)

83
 HYPOTENSION
osms.i"l/h14 o-lension

( PATHOLOGY & CAUSES ) (__ SI_G_NS_&_S_Y_M_PT_O_M_

)
• Condition in which arterial blood pressure • Lightheadedness, fatigue, pallor, confusion
drops below 90/60mmHg • Significant hypotension - syncope
• Physiological in some cases (professional
athletes); considered pathologic if
symptomatic (._____ D_IA_GN_o_s,_s)
• Not a distinctive disease, but a
manifestation of various conditions OTHER DIAGNOSTICS
• Orthostatic hypotension: hypotension • Evaluation of blood pressure with
caused by standing up from a sitting/lying sphygmomanometer/arterial catheter
position O Systolic blood pressure< 90mmHg
O Diastolic blood pressure< 60mmHg
CAUSES O Mean arterial pressure< 65mmHg
• Orthostatic hypotension
Hypovolemia O Drop in 20mmHg of systolic
• Fluid loss (hemorrhage, diarrhea, vomiting), pressure/lOmmHg of diastolic pressure
low fluid intake (starvation, oligodipsia), when standing up from a sitting/lying
endocrine disorders, anemia position
Heart disease
• Cardiomyopathies, heart valve disease (e.g.
mitral stenosis), congestive heart failure,
(._____ T_R_EA_~_M_EN_T
)
myocardial infarction, arrhythmias
• Asymptomatic hypotension does not
Medications require treatment
• Most commonly cause orthostatic • Treat underlying cause
hypotension
• Excessive use of diuretics, alpha/beta
blockers, nitrate preparations. calcium
channel blockers. angiotensin II (ATl)
receptor blockers. antidepressants

Neurological disorders
• Spinal cord injury resulting in ! sympathetic
output or I parasympathetic output
• Dysautonomia (intrinsic autonomic system
dysfunction), Parkinson's disease

COMPLICATIONS
• lschemia
• If severe, can lead to shock

84
 NOTES

NOTES
• INFECTIONS & INFLAMMATION
OF THE HEART

GENERALLY,WHAT ARE THEY?

( PATHOLOGY & CAUSES ) (__ SI_G_NS_&_SY_M_PT_O_M_s_)
• Heart infections, inflammation (may affect • See individual disorders
epicardium, myocardium, endocardium)
• May include: infective endocarditis,
Libman-Sacks endocarditis, myocarditis, ( D_IA_GN_O_s,_s
__ )
rheumatic fever
• May cause/be caused by/coexist with other • See individual disorders
infections

COMPLICATIONS ( T_R_E~_~_M_EN_T
__ )
• Heart failure, arrhythmias, fibrosis • See individual disorders
• Infective, Libman-Sacks endocarditis can
cause
O Damage to heart valves: dysrhythmias,
valve dysfunction
O Invasion of myocardium: heart failure,
heart block, sepsis
O Vegetation can embolize to extremities:
infarction/ischemia causing stroke,
pulmonary edema, glomerulonephritis

85
 INFECTIVE ENDOCARDITIS
osms.i"l/ endoee1rdi-l.is
( PATHOLOGY & CAUSES
• Infection of endocardium, usually with
bacteria, may include heart valve
• Valves have small blood vessels - damage
to valve, vessels - microbes in blood
escape into valvular tissue/microbes enter
small vessels - infection
• Valve endothelial lining damaged
• Microbes enter body via: dental/surgical
procedures, injection with infected needle/
infected substance, wound/abscess
• Vegetation: fibrin, leukocytes, microbes
attach to thrombosis - abnormal growth
- potential embolism
• Often affects left side heart valves
O Predisposing conditions: mitral valve
prolapse, bicuspid aortic valves
TYPES
• Classified by microbial cause
O Acute bacterial endocarditis: infection of
normal valves, rapid progression
O Subacute bacterial endocarditis:
indolent infection of abnormal valves
(e.g. S. viridans)
O Endocarditis in IV drug users:
Methicillin-resistant Staphylococcus
aureus (MRSA), Pseudomonas, Candida
O Prosthetic valve endocarditis:
Staphylococcus epidermidis within 60
days of replacement; after 60 days,
resembles native valve endocarditis
CAUSES
Viridans streptococci (most common)
• Low virulence
• Found in mouth
• Attacks previously damaged valves
• Small vegetations: don't destroy valve
Staphylococcus aureus
• High virulence
• Found on skin
) • Infects damaged, healthy valves
• Large vegetations: can destroy valve
• Most commonly contracted from IV drug
use
Staphylococcus epidermidis
• Infects prosthetic material (e.g. prosthetic
heart valves)
• Enters body during valve surgery/infected
IV catheter: sticks around valve/catheter
• Nosocomial infection (infection in hospital)
• Gut flora
O Enterococcus faecalis
O Streptococcus bovis
• Severe colorectal disease (e.g. colorectal
cancer/ulcerative colitis): bacteria migrate
into bloodstream
Coxiella burnetii
• Exposure to infected animals (e.g. cows,
sheep, goats)
• Q fever - months/years later, endocarditis
• Affects those at high risk:
immunocompromised, pregnant individuals,
pre-existing heart valve defect
• Diagnosis difficult
Candida albicans
• Fungal endocarditis
• Connected with IV drug use
Culture-negative endocarditis
• Cannot be linked to bacteria using blood
cultures
• Aortic vascular infection, persistent low
fever, rash
• Often caused by Coxiella burnetii
HACEK organisms
• Haemophilus, Aggregatibacter,
Cardiobacterium, Eikenella, Kingella
• Gram-negative bacteria
• Normal flora of mouth, throat
86
Nonbacterial thrombotic endocarditi
s
• Damage in valve exposes collagen, tissue
( D_IA_GN_O_SI_S
)
tactor-« platelets, fibrin adhere ---'> form tiny
thrombosls=- mitral valve regurgitation
DIAGNOSTIC IMAGING
O Bacteremia ---'> bacterial attach to Chest X-ray
thrombi ---'> bacterial endocarditis • Enlarged heart, possible pulmonary
congestion
RISI( FACTORS Echocardiogram
• Valvular problems
• Inflamed heart muscle walls, dilation
O Mitra I valve prolapse
O Bicuspid aortic valves
o Prosthetic valves
LAB RESULTS
• Elevated troponin, creatine kinase levels
O Valves affected: mitral > aortic, tricuspid
(due to heart muscle damage)
• Congenital cardiac defects
• Damage to valves due to rheumatic heart Cardiac muscle biopsy
disease • Definitive diagnosis
• IV drug use (esp. tricuspid valve) • Risky procedure, performed only if test
• Chronic hemodialysis results would change treatment plan
• Poor dentition
OTHER DIAGNOSTICS
(__ SI_G_NS_&_S_Y_M_PT_O_M_S_)
ECG
• Sinus tachycardia (increased heart rate)
• Anorexia, weight loss, fatigue • T-wave inversions
• See mnemonic below • "Saddle-shaped" ST segment elevations

\ I MNEMONIC: FROM J"ANE (.____ T_R_EA_~_M_EN_T

__ )

i
Signs & Symptoms
Fever • Viral: improves slowly over time
Roth spots: antigen-antibody • Arrhythmias resolve as inflammation
complex deposits in eyes improves
Osler nodes: painful antigen-
antibody complex deposits in MEDICATIONS
pads of digits
• Antibiotics
Murmur: turbulent blood flow
• Signs of heart failure: managed with
past damaged heart valve
medication, fluid balance
Janeway lesions: erythematous
lesions due to emboli; small,
painless, flat SURGERY
Anemia • Heart transplant in severe cases (e.g.
Nail-bed hemorrhage (splinter Chagas, giant cell myocarditis)
hemorrhages): deposition of
emboli
Emboli: vegetations detach
from valve, deposit
elsewhere (nail beds,
kidneys, spleen, central
nervous system)

87
 Figure 12.2 Bacterial vegetations on the
mitral valve in endocarditis.

Figure 12.1 Janeway lesions are hemorrhagic

macules or nodules that may appear on the
palms of the hands or soles of the feet in
cases of infective endocarditis.

Figure 12.3 Roth spots seen in the retina.

88
 LIBMAN-SACl(S ENDOCARDITIS
osmsJl/ endoeo.Tdi-1:is

( PATHOLOGY & CAUSES ) ( D_IA_GN_O_SI_S

__ )
• Autoimmune endocarditis associated • Must exclude infective endocarditis (may
with systemic lupus erythematosus (SLE). coexist)
advanced malignancy, rheumatoid arthritis
• AKA nonbacterial thrombotic endocarditis/ DIAGNOSTIC IMAGING
verrucous endocarditis
Transesophageal echocardiogram (TEE)
CAUSES • Small, warty, vegetations on both atrial and
ventricular sides of valves
• Antigen-antibody complexes settle in
endocardium • Regurgitation, valve insufficiency
O Arises on valves /chordae tendineae,
most often mitral valve LAB RESULTS
O Arises even on atrial/ventricular • CRP. WBC levels, and antiphospholipid/
endocardium anticardiolipin antibody level may aid in
O Sterile vegetations: aortic valves differentiation

COMPLI CATIONS ( T_R_EA_iT_M_EN_T__ )

• Damage to heart valves
• Invasion of myocardium • Treat underlying SLE
• Vegetations may embolize
• In rare cases, may cause secondary MEDICATIONS
infective endocarditis
Anticoagu lants
• E.g. heparin, direct thrombin, Xa inhibitors
(__ SI_G_NS_&_S_Y_M_PT_O_M_s_)
• Address embolic risk

• Regurgitant murmurs
= Bilateral vegetations on valve leaflets
• Clinical manifestations indicate systemic
emboli
° Kidney: flank pain, hematuria
= Skin: rash, digital ischemia
= Cardiadcentral nervous system (CNS):
chest pain. stroke

89
 MYOCARDITIS
osmsJl/ m14oeo.Tdi"lis
RISI( FACTORS
( PATHOLOGY & CAUSES ) • Viruses that cause flu-like illnesses, HIV/
AIDS, Lyme disease, strep, staph infections,
• Inflammation of/damage to myocardium parasites
• Swelling impairs myocardial contraction ---->
less blood pumped out of heart with each
heartbeat COMPLICATIONS
• Heart failure, fibrosis, arrhythmias

CAUSES
Coxsackieviruses A & B infections (__ SI_G_NS_&_SY_M_P_TO_M_
)
• Viral infections----> lymphocytic myocarditis:
• Clinical manifestations of heart failure (e.g.
B, T cells, water invade interstitial space
fatigue, shortness of breath, hepatomegaly,
• Common in North America edema)
Trypanosoma cruzii • Acute heart failure-« cardiogenic shock
• Single-cell protozoan-» Chagas disease • Arrhythmias (e.g. ventricular fibrillation,
ventricular tachycardia) ----> sudden cardiac
• Amastigotes within heart muscle cells
(intracellular stage of trypanosomes) ----> death
necrosis of heart muscle cells • Fever
• Common in South America • Positional chest pain, related to
pericarditis: better/worse depending on
Trichinella body's position
• Intestinal roundworm may move into heart
----> myocarditis
(..____ D_IA_GN_o_s_,s )
Borrelia burgdorferi
• Lyme disease bacterium DIAGNOSTIC IMAGING
Toxoplasma gondii Chest X-ray
• Single cell parasite harbored by cats • Enlarged heart, possible pulmonary
congestion
Systemic lupus erythematosus (SLE)
• Non-infectious myocarditis Echocardiogram
• Immune system attacks myocardium • Inflamed heart muscle walls, dilation

Drug-associated/hypersensitivity
LAB RESULTS
• Adverse drug reaction inflames heart
• Elevated troponin, creatine kinase levels
• Eosinophils enters blood vessels in
(due to heart muscle damage)
myocarditis
Cardiac muscle biopsy
Giant cell
• Definitive diagnosis
• Inflammation of heart from unknown cause
• Risky procedure, performed only if test
• Macrophages fuse to form single giant cell
results would change treatment plan

90
OTHER DIAGNOSTICS
( T_R_E~_~_M_EN_T
)
ECG
• Sinus tachycardia (increased heart rate) • Viral: improves slowly over time
• T-wave inversions • Arrhythmias resolve as inflammation
• "Saddle-shaped" ST segment elevations improves

MEDICATIONS
\ ' MNEMONIC: BCD ST~G • Antibiotics

i
Common Causes of • Signs of heart failure: managed with
Myocarditis medication, fluid balance
Borrelia bu rgdorferi
Coxsackieviruses A and B SURGERY
Drug-associated • Heart transplant in severe cases (e.g.
Chagas. giant cell myocarditis)
Systemic lupus
erythematosus
Trypanosoma cruzi
Trichinella
Toxoplasma gondii
Giant cell

Figure 12.4 Histological appearance of

myocardium in viral myocarditis.

91
 RHEUMATIC FEVER
osms.i"l/rheumo.-lie-heo.r-l-diseo.se
( PATHOLOGY & CAUSES
• Autoimmune inflammatory disease caused
by complication of streptococcal infection
• Develops after streptococcal pharyngitis
(strep throat) from Group A beta hemolytic
streptococcus
CAUSES
Molecular mimicry
• Antibodies against streptococcal M-protein
cross-reacts with proteins on myocardium,
heart valves, joints, skin, brain - cytokine-
mediated inflammatory response
• Inflammation results in widespread
pathology
Pancarditis
• Inflammation of endometrium, myometrium,
pericardium (three layers of heart tissue)
• Myometrium: Aschoff bodies
(microscopically viewed nodules caused by
inflammation) - leads to fibroid necrosis
° Characteristic feature of pancarditis
O Anitschkow cells (enlarged
macrophages inside Aschoff bodies),
caterpillar-like nuclei
• Pericardium: pericarditis causes pain,
friction rub due to visceral pericardium
rubbing against parietal pericardium
Chronic rheumatic heart disease
• Repeated exposure to group A beta-
hemolytic streptococcus - immune attacks
on tissues (esp. heart tissue)
• Valves (typically mitral valve, sometimes
aortic) develop scar tissue - leaflets
thicken, fuse - commissural fusion
O Stenosis AKA "fish-mouth"/"buttonhole"
stenos is
O Regurgitation (blood flows backward)
• Chordae tendineae attached to valves
thicken
TYPES
) • When only a subset of symptoms present,
classified as the following
Pediatric Autoimmune Neuropsychiatric
Disorders Associated with Streptococcal
Infections (PANDAS)
• Neuropsychiatric symptoms
Poststreptococcal reactive arthritis
• Joint symptoms
RISI( FACTORS
• Small number of individuals with strep
throat develop rheumatic fever, more
likely in children/those in areas of poverty,
crowding
• Rheumatic fever primarily affects children
5-7 years old, 20 days after infection
• One third of cases asymptomatic
(__ s,_G_NS_&_SY_M_PT_O_M_
)
Acute rheumatic fever
• Following symptoms develop 2-4 weeks
after streptococcal pharyngitis
• Fever
• Migratory polyarthritis of joints: temporary
inflammation, swelling, joint pain
• Erythema marginatum: non-itchy, reddish
rash, rings on arms/trunk
• Subcutaneous nodules: firm collagen lumps
under skin
O Reaction to hypersensitivity
, Painless
, Back of wrist, outside elbow, front of
knee
• Pancarditis (inflammation of three layers of
heart)
• Dyspnea, sharp chest pain
• Friction rub heard on auscultation due to
pericarditis
• Impaired ability of heart to contract
92
 (myocarditis) ----> heart failure, death • Minor criteria
• Sydenham's chorea: rapid, jerky O Signs/symptoms: fever
movements of face, arms from damage to (>38.5°C/101.3°F), arthralgia
basal ganglia O Laboratory evidence: increased
O Autoimmune reaction on basal ganglia acute phase reactants (i erythrocyte
of brain sedimentation rate, l C-reactive protein,
O Appears late (three months after t leukocytosis)
infection) O Electrocardiograph: prolonged PR
interval
Chronic rheumatic heart disease
• Evidence of recent infection
• Symptoms dependent on type of O Positive throat culture
damage to heart: aortic stenosis, aortic
O Positive rapid antigen detection test
regurgitation, mitral stenosis, mitral
regurgitation, pulmonic regurgitation O Elevated antistreptolysin O titre (ASO)
• Exception: Sydenham's chorea/pancarditis
PANDAS independently may indicate rheumatic fever
• Pediatric, abrupt onset, episodic course of • Electrocardiogram changes
symptoms
• Neurologic abnormalities: motoric Chronic rheumatic heart disease
hyperactivity (fidgeting), choreiform • Previous repeated cases of rheumatic fever
movements in stressed postures (sudden, • Diagnosis depends on damage done to
jerky movements), frank chorea (rapid, heart: aortic stenosis, aortic regurgitation,
irregular, jerks, movements continuous mitral stenosis, mitral regurgitation,
while awake but improve with sleep) pulmonic regurgitation
• Obsessive-compulsive disorder/tic disorder

Poststreptococcal reactive arthritis

• Arthritis occurring after a streptococcal
infection

( D_IA_GN_o_s,_s__ )
OTHER DIAGNOSTICS
Jones criteria for acute rheumatic fever
• Evidence of previous group A
streptococcus infection plus two major Figure 12.5 Anitschkow cells (enlarged
criteria/one major plus two minor criteria macrophageswith linear nucleoli) in an
Aschoff body (a granuloma) in a case of
rheumatic myocarditis.
MNEMONIC: J"ONES
Major criteria
Joints: polyarthritis
myOcarditis: 0 = vaguely
heart-shaped
Nodules: subcutaneous
Erythema marginatum
Sydenham's chorea

93
 Rheumatic heart disease
(..____ T_R_EA_:l'_M_EN_T
) • Prevent repeated attacks/acute rheumatic
fever, streptococcal infections
MEDICATIONS
• History of acute rheumatic fever:
Rheumatic fever prophylactic treatment for extended period
• Goals of treatment: control, eradicate (benzathine penicillin G/oral penicillin V, 10
streptococcus, prevent complications, years to life)
relieve joint pain, relieve fever
O Antibiotics: penicillin G
O Anti-inflammatory agents: aspirin,
non-steroidal anti-inflammatory drugs
(NSAIDs), steroids
O Antipyretics: NSAIDs
O Rest

OTHER INTERVENTIONS
Rheumatic fever
• Maintain dental health
• Strict long-term, prophylaxis: history of
bacterial endocarditis, heart transplant,
artificial heart valve, other congenital defect

Figure 12.6 Massive cardiomegaly secondary

to aortic and mitral valve disease in a severe
case of rheumatic fever.

Figure 12.7 Gross pathology of acute rheumatic endocarditis; there is a line of acute inflammation
(valvulitis) along the closure line of the mitral valve.

94
 NOTES

- NOTES
•
-
LYMPHATIC DYSFUNCTION

LYMPHEDEMA
osms.i"l/114mphedemC1

( PATHOLOGY & CAUSES )

• Lymphatic system becomes obstructed,
causing protein-rich fluid buildup in tissues
• When flow is blocked, lymph gets backed
up----. drainage stops v- fluid accumulates
• Inflammatory reaction: macrophages
release inflammatory molecules ----.
damages nearby cells ----. scarring, fibrosis
(connective tissues thicken/scar tissue
forms) ----. hardening

CAUSES
• Filariasis: most common cause in low-
income countries
O Infection with nematode parasites (e.g.
Wuchereria bancrofti)
O Nematode enters lymphatic system.
causes fibrosis, creates a blockage
• Cancer, associated treatment: most Figure 13.1 Gross lymphedema of the left leg.
common cause in high-income countries
O Removal of lymph nodes most common
cancer treatment-related cause (e.g. STAGING
axillary lymph nodes removed during
• Stage 0: latent stage. Damage to
mastectomy)
lymphatics but enough lymph still removed.
• Lymphedema praecox/primary Lymphedema not present
lymphedema: congenital, results from
• Stage 1: spontaneously reversible. Tissue in
lymphatic system not developing correctly
pitting stage. Affected area normal/almost
< 35 years old
normal size in morning, progressively
• Lymphedema tarda/primary lymphedema: worsens throughout day
> 35 years old, associated with genetic
• Stage 2: spontaneously irreversible. Tissue
disorders (e.g. Turner syndrome)
spongy, non-pitting (bounces back when
pressed). Fibrosis starts to develop----. limbs
RISI( FACTORS harden. increase in size
• Older age, obesity, rheumatoid/psoriatic • Stage 3: lymphostatic elephantiasis.
arthritis, Turner syndrome. smoking, cancer/ Swelling irreversible, limbs large, hard from
associated treatment (esp. breast cancer) fibrosis

95
COMPLICATIONS
• Recurrent cellulitis, limb swelling (esp. (..____ T_R_E~_~_M_EN_T
)
lower limbs), erythema, pain
• No cure, no medication
• Depends on severity, limb fibrosis
( SIGNS & SYMPTOMS )
SURGERY
• Chronic swelling, one limb larger than • Goal: improve drainage/reduce fluid load
other
• Usually lower limbs; impairs movement
OTHER INTERVENTIONS
• Fatigue, fever, chills, weakness
• Therapeutic exercises, self care
• More likely to occur with superimposed
• Kinesio tape: applied to skin to channel
bacterial/fungal skin infection
lymph, reduce swelling
• Regional edema: begins as soft, pitting
• Aquatic therapy
edema - progresses into chronic fibrosis
without treatment Manual lymphatic drainage (MLD)
• Pneumatic pumps: substitute for MLD

(..____ D_IA_GN_o_s,_s __ ) Compression

• Multilayer compression bandage: stop fluid
DIAGNOSTIC IMAGING accumulation
Lymphoscintigraphy • Compression massages help lymph flow
• Nuclear imaging to assess lymphatic flow • Compression garments
O Radiotracer injected into affected limb
- able to visualize dermal backflow,
absent/delayed radiotracer movement,
absent/delayed lymph node visualization

MRI
• Shows severity, distribution of edema,
lymphatic channels can be depicted after
intracutaneous contrast injection

MR venogram
• Helps differentiate lymphatic channels from
superficial veins

CT scan
• Assists in localization (subfascial,
epifascial), characteristics (skin thickening,
honeycomb pattern of edema)

Ultrasound
• May be used to reveal blockages

Figure 13.2 A plain X-ray of the forearm

showing edema of the subcutaneous tissues.
The subcutaneous fat shows characteristic
streaky densities.

96
 NOTES

- NOTES
•
-
PERICARDIAL DISEASE
GENERALLY.WHAT IS IT?
( PATHOLOGY & CAUSES ) ( D_IA_GN_o_s,_s)
• Disorders affecting pericardium DIAGNOSTIC IMAGING
• Pericardial cavity: space between visceral, • Echocardiogram
parietal layer, normally filled with 15-50ml • X-ray
of plasma filtrate

OTHER DIAGNOSTICS
CAUSES • ECG
• Infections: mostly viral; bacterial, fungal rare
• Malignancy, mediastinal radiation
• Dressler's syndrome ( T_R_E~_~_M_EN_T
__ )
• Trauma
• Pericardiocentesis, pericardiectomy
• Drugs, toxins
• Metabolic disease (e.g. uremic syndrome,
myxedema, ovarian hyperstimulation MNEMONIC: CARDIAC
syndrome), connective tissue disease
RIND
• Immune-mediated disorders
Causes of Pericarditis
Collagen vascular disease
COMPLICATIONS Aortic aneurysm
• Heart failure, circulatory problems. Radiation
problems breathing
Drugs
Infections
(__ SI_G_NS_&_S_Y_M_PT_O_M_s_)
Acute renal failure
Cardiac infarction
• See individual diseases Rheumatic fever
Injury
Neoplasms
Dressler's syndrome

97
 Absent

Normal

Audible Absent Absent

Present Absent Present

Normal

Present Absent Absent

ACUTE PERICARDITIS
osmsJl/o.eul:e-peTieo.Tdi-1:is
( PATHOLOGY & CAUSES
• Pericardia! inflammation, myopericarditis
• Most common pericardia I disorder
CAUSES
• Idiopathic, viral (e.g. Coxsackie B),
uremic syndrome (toxic to pericardium),
Dressler's syndrome, autoimmune (e.g.
rheumatoid arthritis. scleroderma, systemic
lupus erythematosus). cancer, radiation,
medications (e.g. penicillin, anticonvulsants)
COMPLICATIONS
) • Constrictive pericarditis, pericardia!
effusion. cardiac tamponade
( SIGNS & SYMPTOMS )
• Fever, sharp chest pain worsened with
deep breathing, symptoms improve with
sitting up and leaning forward
• Pericardia! friction rub heard on
auscultation, like two pieces of leather
rubbing together, loudest on left sternal
border

RISI( FACTORS
• Surgery, cancer. autoimmune
disease, connective tissue disorders,
immunosuppression

98
( D_IA_GN_o_s,_s __ ) ( T_R_E~_iT_M_EN_T__ )
DIAGNOSTIC IMAGING • Targeted at etiology

X-ray
• "Water bottle sign," liquid collects at the
MEDICATIONS
bottom if effusion present • Treat pain, inflammation
O Non-steroidal anti-inflammatory drug
Echocardiography (NSAID) + colchicine
• Performed to exclude pericardia! effusion O Glucocorticoids if NSAID
contraindicated
OTHER INTERVENTIONS ° Colchicine important if rheumatoid
• Clinical presentation suggestive of arthritis, Dressler syndrome involved
pericardia! effusion
SURGERY
ECG
• Pericardiotomy if high recurrence
• ST elevation, PR depression, voltage
changes, flattened, inverted T wave
OTHER INTERVENTIONS
• Rest
MNEMONIC: PSPPS
Acute pericarditis ECG
PericarditiS
PR depression in Precordial
leads t-\oVE. ,~rro
ST elevation
P€.~\CA~D\AL 1i~':><.)£S

'

LAYt.fZ BcCoMl S
'"'CK€.~
Figure 14.2 Illustration depicting sclerosing
of pericardia I tissues in cross-section of heart
wall.

Figure 14.1 A chest radiograph demonstrating

the globular cardiac silhouette seen in a case
of pericardia! effusions secondary to acute
pericarditis. This is also known as the water
bottle sign.
99
 afratafreeh.com exclusive

Figure 14.4 Histology photomicrograph

demonstrating acute pericarditis. The
mesothelial cells of the pericardium are
surrounded by neutrophils and there is no
fibrosis, indicating an acute inflammatory
reponse.

Figure 14.3 Gross pathology of acute

fibrinous pericarditis. The yellow fibrinous
exudate is clearly visible on the external
surface of the heart.

CARDIAC TAMPONADE
osmsJl/ eo.Tdio.e--lo.mpono.de
RISI( FACTORS
( PATHOLOGY & CAUSES ) • Individuals with malignancy, tuberculous,
purulent pericarditis ::::: those with idiopathic
• Buildup of fluid in pericardium, constricts
pericarditis
heart
• Individuals with fibrinolytic therapy,
• Tamponade = pressure obstructing flow myocardial infarction
• Heart unable to pump normally - blood
flow through chambers obstructed -
cardiac output decreases - hypotension (__ SI_G_NS_&_SY_M_PT_O_M_
- lower tissue perfusion - heart rate
increases • Pulsus paradoxus due to ventricular
interdependence
CAUSES • Beck's triad (see mnemonic)
• Acute onset: trauma, myocardial infarction, • Tachycardia, coughing, dyspnea, weakness,
aortic dissection, pericardia! effusion myocardial ischemia
• Slow onset: cancer, chronic inflammation,
uremic pericarditis, hypothyroidism,
connective tissue disease

100
 \ MNEMONIC: 30s
Beck's triad (Signs &
Symptoms)
Distant heart sounds
Distended jugular veins
Decreased arterial pressure

( D_IA_GN_O_SI_S
)
DIAGNOSTIC IMAGING
Figure 14.5 A CT scan in the axial plane
Echocardiography demonstrating a large pericardia! effusion,
• Excess pericardia! fluid, heart "swinging" separating the pericardium from the heart
inside pericardia! cavity itself and increasing the intrapericardial
pressure, leading to cardiac tamponade.
SURGERY
• Cardiac catheterization
four chambers equal
----> pressure in all
C T_R_E~_~_M_EN_T
)
MEDICATIONS
OTHER INTERVENTIONS • IV fluids
• Clinical presentation

ECG OTHER INTERVENTIONS

• Tachycardia, low QRS complex voltage, Pericardiocentesis
electrical alternans (QRS complexes have
• Needle inserted into pericardium to drain
different heights)
excess fluid

\.\1;,(:\it Co(!> l\loT $TR&T(.t\ 0<>'f h>LW

i!,~a.1-l (01JTRl\<.TlolJ~

l Cl-!At'\6€.RS t:,o,.1'-r f,1.,1. PitoP£RW

l Les~ c~~II-\C. OuTPI),

~ l·NPOTC:~100

L HO\~ &Ai~ fA~TtR

Figure 14.6 Illustration depicting fluid build up around pericardium, putting pressure on
the heart walls and decreasing stroke volume.

101
 CONSTRICTIVE PERICARDITIS
osms.i"l/constrictive- erico.rditis

( PATHOLOGY & CAUSES ) • Edema: part of fluid overload; ascites,

hepatosplenomegaly (HSM), cachexia
• Formation of thick, fibrotic pericardium - (signs of hepatopathy); dyspnea
compresses heart (consequence of low cardiac output)
• Fibroblasts accumulate between pericardia! • Clinical manifestations of pleural effusion
layers - collagen deposits - creates scars
- layers become adherent, lose elasticity
• Heart filling difficult due to stiffness of ( D_IA_GN_o_s,_s
__ )
pericardium
O Ventricular interdependence: lowered DIAGNOSTIC IMAGING
heart wall compliance, decreased
X-ray
transpulmonary venous pressure - left
• Pericardia! calcifications
ventricular filling decreases - lower
volume in left heart - right bends Echocardiogram
septum towards left to increase volume
• Stiff serous pericardium restricts heart's
O Maximal volume diminished, continues movement
to decrease with disease progression
• Volume overload, hepatopathy, decreased CT scan
cardiac output • Anatomical variations, thickness,
distribution of scarring
CAUSES
• Idiopathic, viral, radiation, myocardial LAB RESULTS
infarction, collagen disorders, tuberculosis
Plasma brain natriuretic peptide (BNP)
• Differentiate between tamponade. cirrhosis,
RISI( FACTORS restrictive cardiomyopathy
• Acute pericarditis
• Cardiac surgery, radiation, connective tissue
disorders, bacterial (purulent) infections
OTHER INTERVENTIONS
Invasive hemodynamic monitoring
COMPLICATIONS • Increased pressure in right atrium,
• Heart failure, arrhythmias, cardiac Kussmaul's sign
tamponade

(__ s,_G_NS_&_SY_M_PT_O_M_s_) (.____ T_R_EA_~_M_EN_T__ )

• Elevated jugular venous pressure (JVP) MEDICATIONS
• Kussmaul's sign: paradoxical inspiratory • Diuretics, NSAIDs. corticosteroids
JVP
• Pericardia! knock: heard before S3 on
SURGERY
auscultation
• Pericardiectomy (for progressive disease)

102
Figure 14.7 A chest radiograph
demonstrating pericardia! calcification
secondary to a chronic pericarditis.

DRESSLER'S SYNDROME
osms.i"l/ dTessleTs-s14ncJ.Tome

( PATHOLOGY & CAUSES ) ( D_IA_GN_O_s,_s)

• Secondary pericarditis, rare DIAGNOSTIC IMAGING
• AKA postmyocardial infarction syndrome
Echocardiogram
• May or may not involve pericardia! effusion
• Evaluate ventricular contractility; effusion,
• ~ two weeks after myocardial infarction
signs of tamponade
(Ml), immune-mediated response to injury
- antimyocardial antibodies respond Chest X-ray
to cardiac antigens - immune complex • Cardiac effusion
deposits in pericardium. pleura

LAB RESULTS
( SIGNS & SYMPTOMS ) • Complete blood count (CBC). CRP,
erythrocyte sedimentation rate (ESR);
• Unusual fatigue after cardiac surgery/Ml troponin studies show leukocytosis, j CRP,
• Persistent fever. tachycardia, pulsus l ESR; anti-heart antibody titer
paradoxus
• Manifestations of pericarditis: friction rub, OTHER INTERVENTIONS
symptoms improve in sitting position
ECG
• Pleural effusion signs: pleuritic pain
• Changes same as acute pericarditis
• ST segment elevation. PR depression
103
( T_R_EA_~_M_EN_T
__ )
MEDICATIONS
• Colchicine recommended after cardiac
surgery as preventative measure
• High dose aspirin, NSAIDs, corticosteroids

PERICARDIALEFFUSION
osmsJl/ peTieo.Tdio.1-effusion

( PATHOLOGY & CAUSES ) ( D_IA_GN_O_SI_S __ )

• Abnormal accumulation of inflammatory DIAGNOSTIC IMAGING
fluid, immune cells v- diffuse into
interstitium ----> fluid pools in pericardia! X-ray
space ----> pericardia! dilation ----> pressure • Silhouette pools to bottom of heart, gives
on heart, vena cava ----> decreased cardiac classic "water bottle" sign
filling ----> cardiac tamponade----> decreased
Echocardiogram
cardiac output
• Pericardia! effusion makes heart looks like
• Types of effusion: serous, serosanguinous,
it's dancing within pericardium, "swinging
chylous
heart"

CAUSES
LAB RESULTS
• Aortic dissection, heart failure,
• Elevated markers of inflammation:
hypoalbuminemia, lymphatic obstruction,
(-reactive protein (CRP)
malignancy, radiation, renal failure, trauma,
autoimmune disease, acute pericarditis
(viral, bacterial, tuberculous, idiopathic in OTHER INTERVENTIONS
origin), myxedema, some drugs, iatrogenic,
idiopathic ECG
• Low QRS complex voltage, electrical
alternans, sinus tachycardia
COMPLICATIONS
• Cardiac tamponade
• Constrictive pericarditis ( T_R_EA_~_M_EN_T
__ )
MEDICATIONS
(_~S_IG_NS_&~SY_M_P_TO_M_s_)
• Relieve pain, treat underlying cause of
inflammation
• Clinical presentation nonspecific, related
to underlying cause, reflecting impaired
cardiac function SURGERY
• Diminished heart sounds • Pericardiocentesis
• Jugular vein distention
• Tachycardia, dyspnea, decreased blood
pressure, lightheadedness

104
 NOTES

NOTES
• PERIPHERALARTERY
DISEASE

GENERALLY.WHAT IS IT?
certain drugs)
( PATHOLOGY & CAUSES ) • Intermittent arterial constriction ----. !
diameter ----. ! blood flow
• Narrowing of the arteries in peripheral,
non-coronary arterial circulation
• Vessels of the lower extremities are most RISI( FACTORS
commonly affected • Smoking
• ! blood flow----. arterial insufficiency----. • High blood pressure
tissue ischemia • Diabetes
0 ! gas and nutrient exchange ----. tissue • Hyperlipidemia
loss, ulcer formation ----. poor healing • Metabolic syndrome
O Embolus formation ----. acute limb •Age> 60
ischemia ----. tissue loss
• Obesity
O lschemic cells release adenoslne c-
• I risk in black people of African descent
adenosine signals nerves----. sensation
of pain
O Claudication: pain caused by poor COMPLICATIONS
circulation; occurs when oxygen • l risk of developing coronary artery
demand is greater than oxygen supply cerebrovascular disease
• Location of pain is dependent upon artery • Tissue necrosis
implicated • Amputation
O Lower aorta or iliac artery= pain in hips • Pain
and buttocks
O Iliac or common femoral artery= pain in
thigh (__ SI_G_NS_&_S_Y_M_PT_O_M_s_)
O Superficial femoral artery= pain in
upper% of calf • Often asymptomatic until significant
O Popliteal artery= pain in lower 'h of calf occlusion develops
O Tibial or peroneal artery= pain in foot • Intermittent claudication
O Muscle pain due to l oxygen demand
and! supply
TYPES • Rest pain
Occlusive (most common) • Pain or burning sensation in forefoot
• Usually caused by blockage due to and toes when legs elevated, pain
atherosclerosis relieved when legs are lowered (gravity
assisting blood flow)
• Buildup of plaque ----. narrowed artery----. !
blood flow • ! lower peripheral pulses (e.g. pedal. tibial)
• Leg/foot ulcers that do not heal normally
Functional O Have classic punched out appearance
• Caused by a defect in the normal O Often form on toe joints. malleoli. shin.
mechanisms that dilate and constrict base of heel, pressure points
arteries (e.g. inherited defects. injuries.
105
 O Painful
O Slow healing ---'> j risk of infection
(..____ D_IA_GN_O_SI_S
)
• Cutaneous color changes DIAGNOSTIC IMAGING
O Elevation pallor: foot turns pale when
raised due to circulation having to work Doppler ultrasound
against gravity as well as narrowed • ! blood flow
artery
O Dependent rubor: foot turns red when
OTHER DIAGNOSTICS
lowered as gravity works increases
perfusion Auscultation
• Skin: cool, dry, shiny, hairless • Bruit (whooshing sound) heard on
• Nails: brittle, hypertrophic, ridged auscultation of suspected artery
• Signs of acute limb ischemia , Usually pulse of leg's iliac artery
O See mnemonic , Whooshing sound due arterial
narrowing

MNEMONIC: 5Ps Ankle-brachia

) index (ABI)
Signs of acute limb ischemia • ABI < 0.9: peripheral artery disease
Pain • ABI of 0.4-0.9: claudication
Pallor • ABI of 0.2-0.4: rest pain
Pulselessness • ABI of 0-0.4: tissue loss, ulcers, gangrene
Paresthesia
Paralysis (a surgical
emergency)
C..____ T_R_EA_~_M_EN_T
)
MEDICATIONS
• Antiplatelet therapy

SURGERY
• Angioplasty, stent insertion
• Endarterectomy
• Bypass surgery to restore blood flow by
diverting it around blockage
• Amputation

OTHER INTERVENTIONS
• Modify risk factors; e.g. smoking cessation,
Figure 15.1 An arterial ulcer on the dorsum healthy eating habits, exercising regularly,
of the foot; a consequence of peripheral managing diabetes
vascular disease. Note the punched out
• Wound care
appearance.

106
 DIAutJOCE,\S

o.o o:t o.ti

0.9
Figure 15.2 Illustration depicting the Ankle-branchial index.

ARTERIOLOSCLEROSIS
osmsJl/o.T-leTioloseleTosis
membrane becomes "leaky" ----> serum
( PATHOLOGY & CAUSES ) proteins move into endothelial cells and
build up into tunica media
• Arteriosclerosis: a general term for
diseases where the artery wall becomes Hyperplastic arteriolosclerosis
thicker, harder, and less elastic • Smooth muscle cell hyperplasia ----> very
O Arteriolosclerosis: a disease of the small small lumen ----> ! blood flow ----> tissue
arteries and arterioles characterized by hypoxia
stiffening and thickening of the vessel • Malignant hypertension ----> smooth
wall due to high blood pressure or muscle cells lining arteriole exposed to
diabetes. manifested primarily in the plasma proteins ----> concentric layers of
kidneys smooth muscle cell proliferation ("onion-
skinning")
TYPES
RISI( FACTORS
Hyaline arteriolosclerosis
• Diabetes mellitus
• Accumulation of proteins and pink hyaline
• Chronic hypertension
material ----> j thickness and stiffening of
• Malignant hypertension
vessel wall ----> ! compliance ----> ! blood flow
----> tissue hypoxia
O Sustained high-pressure in vessels=- COMPLICATIONS
serum proteins pushed into blood vessel • Arteriolonephrosclerosis
walls ----> protein build-up in tunica media • Formation of intraluminal thrombi
° Chronic high blood glucose----> • Chronic renal failure
endothelial cells become glycosylated
----> endothelial dysfunction ----> basement

107
 (..____
(__ SI_G_NS_&_SY_M_PT_O_M_s
) D_IA_GN_O_SI_S
)
• Clinical manifestations of chronic kidney LAB RESULTS
disease • Signs of arteriolonephrosclerosis
O Anemia (fatigue, activity intolerance, • f blood urea nitrogen
pallor) • l creatinine
Fluid and electrolyte imbalance (edema,
°
• ! hemoglobin
muscle weakness, palpitations)
• ! hematocrit
O Uremia (anorexia, mental status
• Proteinuria
changes)
• Oliguria
O Renal osteodystrophy

(..____ T_R_EA_:T'_M_EN_T
)
OTHER INTERVENTIONS
• Management of diabetes and hypertension;
support renal function

ATHEROSCLEROSIS
osms.i"l/ o.-1:heToseleTosls
RISI( FACTORS
( PATHOLOGY & CAUSES ) • Family history of coronary heart disease
• Smoking
• Arteriosclerosis: a general term for
diseases where the artery wall becomes • Hypertension
thicker, harder, and less elastic • Dyslipidemia; especially low HDL
O Atherosclerosis: atheromatous plaques • Metabolic syndrome
on the tunica intima of large and • Males ;;;: 45; females 55 ;;;: or premature
medium vessels menopause without hormone replacement
• Damage to endothelium - low-density therapy
lipoproteins enter endothelial wall - LDL
oxidation - uptake of LDL by macrophages COMPLICATONS
- foam cell formation - cytokine and
• Cardiovascular and coronary heart disease
growth factor release from foam cells -
formation of thrombogenic fatty streak - , Myocardial infarction, heart failure,
platelets release platelet-derived growth death
factor - migration of smooth muscle cells • Cerebrovascular disease
from vascular media to intima - fibrous O Transient ischemic attack, stroke
cap - atherosclerotic plaque - chronic • Peripheral artery disease
inflammation O Leg ulcers, amputation
• Calcium deposits into plaque - stiffening • Aortic aneurysm
of arteries

108
(__ SI_G_NS_&_S_Y_M_PT_O_M_s
)
• Symptoms vary according to extent and
location of blockage
• Carotid artery
O Weakness, difficulty speaking, dizziness,
difficulty walking, blurred vision,
numbness of face/arms/legs, severe
headaches
• Peripheral arteries
° Claudication, presence of ulcers
• Coronary arteries
o Angina
• Cerebral arteries
O Auscultation of bruit, neurological
complaints (e.g. visual changes, facial
paresis)

(..____ D_IA_GN_O_s,_s)
• History and presence of clinical
manifestations indicating occlusive disease

DIAGNOSTIC IMAGING Figure 15.3 The abdominal aorta at post

mortem showing moderate atherosclerosis.
Angiography
• Vascular calcifications, stenosis, occlusion,
collateral circulation
C..____ T_R_E~_~_M_EN_T__ )
Ultrasound
• Luminal stenosis, atheromatous • Goal: reduce risk of complications with
calcification (hyperechoic foci producing an management risk factors; e.g. lipids, blood
acoustic shadow) glucose, hypertension

Magnetic resonance angiography

• Thickened arterial wall, heterogeneous MEDICATIONS
signal within vessel wall (lipid rich necrotic • Antiplatelets
core, plaque, fibrous cap) • Antilipemic agents
• Antihypertensives
LAB RESULTS
• hs-CRP (high-sensitivity C-reactive protein) SURGERY
test • Complications: stents, bypass grafts,
O l CRP indicates "silent atherosclerosis" angioplasty, carotid endarterectomy (CEA)
before cardiovascular event
• Fasting lipid profile

109
Figure 15.4 An atherosclerotic artery. Note
how the plaque protrudes into the lumen. It
is composed primarily of cholesterol with an
outer rim of foamy macrophages.

Diminished or absent Normal

Ulcers: "punched out" and concentric;

mostly on toes, heels. other bony Ulcers: irregular borders; "stocking"
prominences; minimal exudate; distribution. most common near medial
poor healing matleolus, significant exudate

Skin: shiny, loss of hair. thick. Skin: hemosiderin deposits cause

brittle toenails brawny discoloration; stasis dermatitis
and pruritis
Elevation pallor, dependent rubor

Cool Warm

> 3 sec < 3 sec

Soft, thin skin Hardened, leathery skin

Not present present

Intermittent claudication Dull, achy pain relieved by elevation

Painful ulcers Painful ulcers

Mnemonic: PATCHES

110
 NOTES

- NOTES
•
-
PRE-EXCITATION DISORDERS

GENERALLY,WHAT ARE THEY?

( PATHOLOGY & CAUSES ) ( T_R_E~_~_M_EN_T
__ )
• Heart rhythm disturbances due to • Acute termination of preexicitation-
accessory pathway in conduction system associated arrhythmias
that allows depolarization to bypass • Chronic prevention of preexicitation-
atrioventricular node and spread from atria associated arrhythmias
to ventricles
• Accessory pathways can promote
arrhythmias by two mechanisms
MEDICATIONS
• Acute termination
O Acting as one limb of a reentrant circuit,
= Adenosine: short acting; causes
with atrioventricular node acting as the
other transient heart block (! rate of diastolic
depolarization, ! HR)
O Bypassing physiologic atrioventricular
• Diltiazem (Class IV): calcium channel
nodal delay - impulses reaching
ventricle not regulated - very rapid blocker(! AV node conduction - ! HR)
ventricular responses in atrial arrhythmia • Chronic prevention
setting such as atrial fibrillation, atrial • Amiodarone (Class 111): slows
flutter conduction rate (i AP duration, l QT
• If ventricular rate becomes too high - interval)
ventricles don't have time to fill - low = Procainamide (Class lA): slows
cardiac output - shock conduction velocity (l AP duration,
j ventricular refractory period, j QT
interval)
(__ SI_G_NS_&_S_Y_M_PT_O_M_s_)
OTHER INTERVENTIONS
• Tachyarrhythmias - palpitations,
• Acute termination
chest discomfort, breath shortness,
• Vagal maneuver (carotid sinus massage/
lightheadedness, syncope
Valsalva maneuver) - activates vagus
nerve
( D_IA_GN_O_s,_s
__ ) O Electrical cardioversion (if
pharmacological treatment ineffective/
• See individual disorders fast heart rate is poorly tolerated)
• Chronic prevention
= Radiofrequency catheter ablation
(definitive treatment)

111
 AV REENTRANT TACHYCARDIA
(AVRT)
osmsJI:/ e1v-Teen-l:Te1n-l:--le1eh14ee1Tdie1

( PATHOLOGY & CAUSES ) ( T_R_E~_~_M_EN_T__ )

• Arrhythmia due to accessory pathway MEDICATIONS
between atria and ventricles that allows • Acute termination
electrical signal to move backwards , Adenosine, Diltiazem (Class Ill)

Orthodromic atrioventricular reentrant • Chronic prevention

tachycardia (AVRT) , Amiodarone (Class Ill), Procainamide
• Signal moves downward through (Class lA)
atrioventricular node - ventricles contract
- upward through accessory pathway OTHER INTERVENTIONS
- atria contract - moves back down • Acute termination
atrioventricular node - etc.
, Vagal maneuver
Antidromic atrioventricular reentrant , Electrical cardioversion (if
tachycardia (AVRT) pharmacological treatment ineffective/
• Signal moves downward through fast heart rate is poorly tolerated)
accessory pathway - ventricles contract • Chronic prevention
- upwards through atrioventricular node , Radiofrequency catheter ablation
- atria contract - moves back down the
accessory pathway - etc.

(__ SI_G_NS_&_SY_M_PT_O_M_s_)
• Tachyarrhythmias - palpitations,
chest discomfort, breath shortness,
lightheadedness, syncope

( D_IA_GN_O_s,_s
__ )
OTHER DIAGNOSTICS
ECG
• Orthodromic AVRT
O Regular, narrow-complex tachycardia,
P waves are typically retrograde in
morphology and come after QRS
complex
• Delta wave is not seen
• Antidromic AVRT
O Regular, wide-complex tachycardia, P
waves often not visible
112
L -= I I' .J,;. r. :1 :i I
-· . .,. f·
i "1};'"+-rJ'lii-+--t-h
i
. -· ""'
f:P r :1 1:~

11,
•A J,- 1 .\,; IF"
.,.

A '-

t I,

I
I·
,.

Figure 16.1 An electrocardiogram demonstrating othodromic AVRT. Note the narrow QRS
complexes and absence of a discernible P wave.

Figure 16.2 An ECG demonstrating orthodromic AVRT with regular, narrow-complex

tachycardia with retrograde P waves visible just after the QRS complexes, most visible in leads II
and V4-V6.

113
 n~J\AMMMN.
r1))J)J)JJJJ~ ~
~ MMMrwv
n
Figure 16.3 An ECG demonstrating antidromic AVRT. There is a regular, wide complex
tachycardia that is usually indistinguishable from VT.

WOLFF-PARl(INSON-WHITE
SYNDROME
osms.tl/ wolff- o.Tkinson-whi"le-s14ndTome

( PATHOLOGY & CAUSES ) • Most common type of ventricular

preexcitation syndrome
• Congenital accessory pathway conducts , 0.1 % of individuals have Wolff-
electrical signals between atria and Parkinson-White pattern, a small
ventricles ----> preexcitation, predisposes proportion of them develops syndrome
individuals to clinically significant
arrhythmias up to sudden cardiac death
O Though "bundle of Kent" is a common ( SIGNS & SYMPTOMS )
eponym for congenital accessory
pathway, several different pathways • Tachyarrhythmias ----> palpitations,
can occur, most commonly direct chest discomfort, breath shortness,
atrioventricular connections, but also lightheadedness. syncope
atriofascicular, nodofascicular, atrio-
Hisian, etc.
O Wolff-Parkinson-White pattern: benign
asymptomatic form, solely described
by compatible electrocardiographic
changes

114
( D_IA_GN_O_s,_s) ( T_R_E~_~_M_EN_T
)
OTHER DIAGNOSTICS MEDICATIONS
• Acute termination
ECG
, Adenosine, Diltiazem (Class Ill)
• Short PR interval (< 120ms)
• Chronic prevention
• Delta wave
, Amiodarone (Class Ill), Procainamide
Slurred upstroke of QRS
O
(Class lA)
• Widening of the QRS complex (> 1 lOms)
• Secondary ST segment, T wave changes
OTHER INTERVENTIONS
• Acute termination
• Vagal maneuver
• Electrical cardioversion (if
pharmacological treatment ineffective/
fast heart rate is poorly tolerated)
• Chronic prevention
• Radiofrequency catheter ablation

Figure 16.4 ECG pattern in Wolff-Parkinson-White syndrome.

115
 I

>----
v v

I I
-~ [JI

I lu, v

\. V1 ... ,. ..... -
-"
" '
,...'\.

'
... v
I

'\.- ./ - IA
I
,/
"'"
/I
-- I

,.,_ -- ..... ,~ ~
" IJI~ ~
A
I\ IA ,/ .I'\ '-

Figure 16.5 An ECG of an individual with Wolff-Parkinson-White syndrome (sinus rhythm).

Delta waves are most visible in the V leads.

n~~
. •"'-

n ,~.~

Figure 16.6 An ECG demonstrating "pre-excited a-fib" or atrial fibrillation in a person with
Wolff-Parkinson-White. It's an irregularly irregular wide-complex rhythm with no discernible P
waves.

116
 NOTES

- NOTES
•
~
PREMATURE CONTRACTION

GENERALLY.WHAT IS IT?
• Alcohol use
( PATHOLOGY & CAUSES ) • Heart dilation: cardiomyopathies, cor
pulmonale
• Depolarizing potential from anywhere in
• Heart scarring: after myocardial infarction,
heart other than sinoatrial (SA) node -
myocarditis
contraction earlier than normal in cardiac
cycle
• Triggered activity COMPLIC ATIONS
° Cells triggered by preceding action • Rarely atrial/ventricular fibrillation
potential after repolarization
° Cause: reperfusion therapy after
myocardial infarction/digoxin toxicity (_~SI_G_NS_&~SY_M_P_TO_M_s_)
• Ectopic focus
• Usually asymptomatic
° Cells irritated by electrolyte imbalances,
drugs, ischemic damage - increased • In case of frequent premature contractions:
sympathetic activity - enhanced lightheadedness, palpitations
automaticity - early depolarization
• Reentrant loop
O Tissue unable to depolarize (e.g. scar
( D_IA_GN_O_s,_s
__ )
tissue, amyloid) - no signal conduction
- depolarizing wave obstructed -
OTHER DIAGNOSTICS
depolarizing wave circles tissue - • ECG
abnormal electrical circuit • Holter monitor
• ZIO patch
CAUSES
• Often idiopathic ( T_R_E~_~_M_EN_T__ )
• Electrolyte imbalances (hypokalemia,
hypercalcemia, hypomagnesemia) • See individual disorders
• Recreational/prescription drugs
(methamphetamines, cocaine, digoxin
intoxication)

117
 PREMATURE ATRIAL
CONTRACTION (PAC)
osms.i"l/ Y-emo.tu Y-e-o.-lY-io.l-eon-lY-o.e-lion
• Noncompensatory pause
( PATHOLOGY & CAUSES ) , Premature impulse enters sinoatrial (SA)
node ----> shortens cycle
• Contraction of atria earlier than normal in
, Distinct from compensatory pause:
cardiac cycle
premature ventricular contractiorr-e
• Atrial bigeminy: premature atrial premature impulse does not reach
contraction consistently occurs after each SA node-« if AV node still refractory,
normal cardiac cycle pauses ----> lengthens cycle
• Atrial trigeminy: premature atrial
• Normal QRS
contraction consistently occurs after every
O Premature impulse reaches AV node in
two normal cardiac cycles
refractory----> blocked premature atrial
contraction ----> QRS nonexistent
CAUSES • Ashman phenomenon
• Heart structural disorders, intoxication, OR-R interval prolonqs e- increases
electrolyte imbalances refractory period of right bundle branch
----> abnormal conduction of subsequent
COMPLICATIONS impulse----> right bundle branch block on
ECG
• Atrial fibrillation
• Holter monitor
0 24h, detect premature contractions
(__ SI_G_NS_&_SY_M_PT_O_M_s_)
• Usually asymptomatic
( T_R_EA_~_M_EN_T__ )
• In case of frequent premature contractions:
lightheadedness, palpitations • Typically requires no treatment

( D_IA_GN_O_s,_s
__ ) MEDICATIONS
• If symptomatic: beta blockers/calcium
OTHER DIAGNOSTICS channel blockers
• Electrolyte replacement
ECG
• Early, abnormal P wave
O Ectopic focus in bottom of atria ---->
SURGERY
negative P wave • If triggering atrial fibrillation:
radiofrequency catheter ablation
O Ectopic focus closer to atrioventricular
(AV) node e- PR interval shorter
OP wave, T wave overlap

118
Figure 17.1 Illustration depecting abnormal P wave in atrial bigeminy and trigeminy.

PflE.IY\ATUftE. ATlllAl.. COtJTAACrTION

AT'11A Cot-JT\2~'-'T £Afl.L'(

L f<olYI l£.1'0P\L foe.us
Ji. t\JOllMAL
lOJJbUC..TIO>-J

~ A&tJOltN'~l.
C.OtJOUC.T\Oµ

*- ~o
U>\JOUC..TIOt.J

Figure 17.2 Illustration comparing normal ECG tracing vs ECG tracing with premature atrial
contraction.

119
 afratafreeh.com exclusive

PREMATURE VENTRICULAR
CONTRACTION (PVC)
osmsJl/pTemaluTe-ven-lTieulaT-eon-lTae-lion

RISI( FACTORS
( PATHOLOGY & CAUSES ) • Hypertension, smoking, exercise, stress,
people of African descent (+30% risk),
• Contraction of ventricles earlier than normal biological male
in cardiac cycle
• Ectopic focus
, Latent pacemakers: AV node, bundle of
COMPLICATIONS
His/Purkinje fibers take over SA node's • Ventricular tachycardia, ventricular
function of pacemaker fibrillation, increased risk for sudden cardiac
death
, Irritated cardiac muscle cells - early
depolarization
• Triggered activity
(_~S_IG_N_S_&_SY_M_P_TO_M_
, Ventricular repolarization - ventricle
cells triggered by preceding action • Can be asymptomatic
potential
• Lightheadedness, palpitations
, Cause: reperfusion therapy after
myocardial infarction/digoxin toxicity
• Reentrant loop ( D_IA_GN_O_S_IS
__ )
, Tissue unable to depolarize (e.g. scar
tissue, amyloid) - no signal conduction OTHER DIAGNOSTICS
- depolarizing wave obstructed -
depolarizing wave circles tissue - ECG
abnormal electrical circuit • Wide, bizarre QRS: signal goes through
• Ventricular bigeminy: premature ventricular ventricular muscle, not normal conduction
contraction consistently comes after each pathway - conduction is slower than
normal cardiac cycle normal
• Ventricular trigeminy: premature ventricular • Ectopic impulse in right ventricle
contraction consistently comes after every 'Left bundle branch block pattern of QRS
two normal cardiac cycles complex
=Vt: large negative complex, dominating
Swave
CAUSES
• Ectopic impulse in left ventricle
• Heart structural disorders, intoxication,
electrolyte imbalances , Right bundle branch block pattern of
QRS complex
' Vl: large positive complex, dominating
Rwave

120
• Abnormal ST segments: deviation from
isoelectric baseline in opposite direction
from QRS complex
• Inverted T waves in leads, QRS complex
predominantly positive
• Nonexistent P wave: covered by wide QRS
complex
O QRS followed by compensatory
• Ventricular fusion beat: premature QRS
complex occurs during PR segment,
combines with normal depolarization wave
• R-on-T phenomenon: premature QRS
complex occurs at/near T wave apex
• Holter monitor
( T_R_E~_iT_M_EN_T
)
• Typically requires no treatment
MEDICATIONS
• If symptomatic: venodilators, calcium
pause channel blockers, administer beta blockers
with caution
SURGERY
• If triggering ventricular arrhythmias:
radiofrequency catheter ablation to destroy
ectopic focus/replacement if necessary

OTHER INTERVENTIONS
• If mild, no exercise restrictions; if severe,
reduced physical activity

Du~ltJ~ A P-WAVE.

,::....
'16tJT~1,vc..l\ll- Fvs1otJ)

Du111M~ T-wAVE.
~ R-o~-T P11woMHIOtJ
~ StCatJ oJ I~P6WOl~Gt
~ll~liVTHMl~(O

Figure 17.3 Illustration comparing premature ventricular contractions that occur during a P wave,
during a PR segment, and during a T wave.

~ PVC. llfl<ll £Alll CAltOoM.. ~ Pvc. l\f1<1t £wt.rzv 1

,YCLL ll'IIOIH CYCLlS

Figure 17.4 Illustration comparing ventricular bigeminy and trigeminy.

121
NOTES

SHOCI(
osms.i"l/shock
creatinine concentration (non-specific,
(__ P_AT_H_Ol_O_G_¥_&_C_AU_S_E_S
_) i.e. seen in all forms of shock)
• Abnormal potassium levels
• Global inadequate tissue perfusion
= Metabolic acidosis/alkalosis
O Extremely low blood pressure (BP) -
• Hematocrit, serum albumin
end-organ failure
concentration - reduction in plasma
volume increases concentration
TYPES Cardiogenic Shock
• Hypovolemic shock, cardiogenic shock,
obstructive shock, distributive shock • General clinical manifestations
= Hypotension,manifestations of
Hypovolemic Shock pulmonary edema
• General clinical manifestations • Subtypes of cardiogenic shock
O Reduced preload with suspected cause • Myopathic: find specific cause via ECG/
• Variable presentation based on etiology of lab values/chest radiograph
fluid loss • Arrhythmogenic: caused by arrythmia
• Hemorrhage, evidence of trauma
Obstructive Shock
OInternal bleeding into thoracidperitoneal/
retroperitoneal space • General clinical manifestations
• Nonhemorrhagic fluid loss • Low preload; obstruction of blood flow
outside the heart
O Decreased tissue perfusion
, Cardiac tamponade, pulmonary
O Elevated blood urea nitrogen, serum
embolism, tension pneumothorax

l-wPollOL£~1c. S11°'"
J:\ffU:T~ th< 'W~ SOI>~
°" lle;t\ol!Rll"G.E.
l:>£.IIYl>Q.I\T\ou
p.,,.w<. °'1'-Ao.l~ •IRi'>K \or
~...,"~ &ooc, ~I-IE.
1"1~" f'A1Wll£

U\Rt>I0'-0,IIC.. ~~oc:
MI\PP-oAIS. w•"'IN3'JIN .. ~Tt:1.,CTIO'>
l)RLO(o.lT~ ~Atl' ._. l'u,<p,.,4 UFi<oetJI\.Y

bMR181Jl'IIIE. S..ouc
AoJ Au.UC.IL At"'-Tloo.> .a ~~ .,. ....._ l.lu.loo,~ 5'1STO'\
c~ .. s,...oc, "=~.,. ~.-, "' k<.-.L LCA""

Figure 18.1 Illustration summarizing the causes and effects of hypovolemic, cardiogenic, and
distributive shock.
122
Distributive Shock RISI< FACTORS
• General clinical manifestations • Dependent upon type
O Hypotension without reduced preload, • Septic shock most common in United
fluid overload States, followed by cardiogenic,
• Subtypes of distributive shock hypovolemic, other forms of distributive/
obstructive shock
O Septic: caused by infection
• Hypovolemic shock from gastrointestinal
O Anaphylactic: allergic reaction -
(GI) losses/dehydration most common in
respiratory distress, vomiting, abdominal
low-income countries
pain, chest pain, dysrhythmia, collapse
O Neurogenic: pain at site of spinal
fracture, evidence of spinal injury (loss STAGING
of sensation, paralysis, loss of reflexes)
Initial
O Endocrine: adrenal crisis (nonspecific
symptoms, eg. anorexia, nausea, • Cellular. not clinically apparent
vomiting, abdominal pain, fatigue,
Compensatory
lethargy, weakness, fever, confusion,
• Neural, hormonal, biochemical
coma); confirmation of adrenal
compensation to maintain homeostasis;
insufficiency
inadequate perfusion - autonomic nervous
system attempts to compensate
= Sympathetic nervous system

Trauma

Vomiting, pancreatitis, diarrhea

Non-hemorrhagic Low preload bowel obstruction, severe burns,
fistula drainage, diabetes insipidus

Myocardial infarction, acute

dccornpcnsation of any etiology
Myopathic Low contractility
of chronic hcort foilurc.
blunt cardiac injury, myocarditis

Arrhythmogenic Low HR. Arrhythmia -···-···--···-···

Low preload

Cardiac tamponade, pulmonary

Obstructive Low preload
embolism, tension pneumothorax

Septic LowSVR Infection

LowSVR.
Neurogenic Trauma
Low HR

Allergic reactions: insect bites or

Anaphytactic LowSVR
stings. drugs. allergies. IV contrast

LowSVR,
Endocrine/hypoadrenal Adrenal insufficiency
Low preload

123
 vasoconstriction, t contractility • Preserved/hyperdynamic left ventricle=
O Release of catecholamines, vasopressin, distributive shock
angiotensin II ----> j vasoconstriction, • Point-of-care ultrasond
t retention water, sodium ----> t SVR, t , Examination of heart -e cause of
blood volume ----> I BP ----> i perfusion cardiogenic shock, obstructive shock

Progressive Focused assessment and sonographyfor

• Compensation fails, requires aggressive trauma (FAST)
interventions to prevent multiple organ • Fast ultrasound examination for
dysfunction syndrome hemopericardium, intra-abdominal
bleeding; rule out/in hypovolemic shock
Irreversible
• Decreased perfusion (vasoconstriction,
decreased cardiac output) ----> anaerobic Hemodynamic monitoring
metabolism; profound hypotension, • Via central venous catheters
hypoxemia, organ failure; recovery unlikely • Elevated central venous pressure, low
mixed venous oxygen saturation=
cardiogenic shock
(~~SI_G_NS_&~SY_M_P_TO_M_s_)
• Altered mental state, decreased peripheral LAB RESULTS
pulse, tachycardia, hypotension
Elevated serum lactate
• Varies by type and subtype of shock (see
• Early indicator, reflective of poor tissue
table below)
perfusion

Renal, liver function tests

(.__ __ D_IA_GN_O_s,_s __ ) • Elevated blood urea nitrogen (BUN),
creatinine, transaminases indicate end-
DIAGNOSTIC IMAGING organ damage
Chest radiography n May help point to cause (acute hepatitis,
chronic cirrhosis)
• Clear in hypovolemic/obstructive shock
from pulmonary embolism Coagulation studies, 0-dimer level
• Pneumonia • Elevated fibrin split products, elevated
, Septic shock 0-dimer level, low fibrinogen level = severe
• Pneumothorax shock
, Obstructive shock
Cardiac enzymes, natriuretic peptides
• Pulmonary edema
• Elevated troponin, creatine phosphokinase,
, Cardiogenic shock/ARDS
N-terminal pro-brain natriuretic peptide,
Pulmonary artery catheterization brain natriuretic peptide = cardiogenic
shock due to ischemia/pulmonary embolism
• Hemodynamic measurements can be
helpful Complete blood count, differential
• Measure cardiac output, systemic vascular • High hematocrit
resistance, pulmonary artery occlusion O Hemoconcentration from non-
pressure, right atrial pressure, mixed
hemorrhagic hypovolemic shock
venous oxyhemoglobin saturation
• Anemia, bleeding
• Rarely necessary to identify etiology of
O Hemorrhagic shock
shock
• Elevated eosinophil
Ultrasound/echocardiography O Allergy, anaphylactic shock
• Allows visualization of altered cardiac • Leukocytosis
function O Septic shock, not specific; more common

124
 in septic shock, may also occur in other OTHER DIAGNOSTICS
types of shock as sign of poor prognosis
History & physical
Coagulation studies, D-dimer level • Low blood pressure, tachycardia,
• Elevated prothrombin time, international tachypnea, signs of poor end-organ
normalized ratio, activated partial perfusion (low urine output, confusion, loss
thromboplastin time of consciousness), weak pulse, cool skin,
O Septic shock, other issues (e.g. sepsis, metabolic acidosis, hyperlactatemia
systemic inflammatory response
Shock index
syndrome); elevated D-dimer levels
common in septic shock • Heart rate divided by systolic pressure
• Normal range 0.5-0.8
Peripheral 02 sat via pulse oximetry • If index higher, increased suspicion of
• Hypoxemia underlying state of shock
O Obstructive, cardiogenic shock • Most useful for isolated hypotension/
tachycardia
Urinalysis
• Infection, septic shock ECG
• Arrhythmia, ST segment changes
Material gram stain from infection sites consistent with ischemia
• Septic shock • Low-voltage ECG
Blood culture = Pericardia! effusion
• identifies causative microbe in case of • Arrhythmia
septic shock; directs targeted antibiotic = Arrhythmogenic cardiogenic shock
therapy • lschemia
• Myopathic cardiogenic shock

BLOOD PRESSURE CHANGES IN SHOCIC

TISSUE
PRELOAO PUMP FUNC, TION SVR
(PWP) (C.O) PERFUSION
(SvOa)

HVPOVOLEMIC.

CAROIOGENIC

DISTRIBUTIVE t (early), ! (lotc)

OBSTRUC. TIVE
(PULMONARY EMIOLISM,
MYPERTENSION, TENSION
PNEUMOTMORAX)

OBSTRUCTIVE
(PERICARDIAL
TAMPONADE)

125
MNEMONIC: ABCOE
Treatment for shock (~~~TR_E_AT_M_E_N_T~
)
Airway: ensure clear airway,
• See chart for a detailed summary of
possibly intubate
treatments for different forms of shock
Breathing: assist individual
in breathing, mechanical
ventilation/sedation OTHER INTERVENTIONS
Circulation: administer fluids
Surviving sepsis campaign guidelines
(e.g. isotonic crystalloid)
• End resuscitation when urine output 0.5ml/
Delivery of oxygen: monitor
kg/hr, central venous pressure (CVP) 8-12
lactate levels
mmHg, mean arterial pressure (MAP)
Endpoint resuscitation (specific 65-90mmHg, central venous oxygen
to septic shock) concentration > 70%, normalize lactate
levels
, CVP 8-12mmHg (recent literature
shows CVP poorly predicts fluid
responsiveness, poor marker of
adequate resuscitation)

- Fluid resuscitation
Non-hemorrhagic
- Prevent hypothermia caused by fluid resuscitation

- Same treatment as non-hemorrhagic shock

Hemorrhagic
- Packed red blood cell transfusion

- Diuretics
- lnotropes
Cardiogenic - Intra-aortic balloon pump
- ACE inhibitors
- Hydralazine

Obstructive - Treat underlying cause

- Antibiotics
Septic - Fluid resusitation
- Vasopressors

- Epinephrine
Anaphylactic - Antihistamines
- Fluid resusitation

- Fluid resusitation
Neurogenic - Vasopressors
- Corticosteroids

Endocrine/hypoadrenal - Corticosteroids

126
 NOTES

- NOTES
•
-
SUDDEN CARDIAC DEATH
GENERALLY.WHAT IS IT?
( PATHOLOGY & CAUSES ) ( T_R_E~_~_M_EN_T
)
• Abrupt cessation of cardiac activity (cardiac MEDICATIONS
arrest) in someone asymptomatic up until • According to Advanced Cardiac Life
moment of arrest Support protocols

OTHER INTERVENTIONS
(__ SI_G_NS_&_S_Y_M_PT_O_M_s_)
Cardiopulmonary resuscitation (CPR)
• Asymptomatic
• Maintains blood flow by mimicking
pumping motion heart makes during a
medical emergency
( D_IA_GN_O_s,_s
__ )
• Made based on lack of pulse

BRUGADA SYNDROME
osms.i-l/\>Tuge1de1-s14ndTome
• 20% associated with SCN5A gene
( PATHOLOGY & CAUSES ) mutation which encodes for sodium ion
channel in cell membranes of heart muscle
• Condition with characteristic abnormal cells
electrocardiogram findings - increases
risk of sudden cardiac death in healthy
individuals RISI( FACTORS
• Mixture of normal, abnormal sodium • Biological males. more common in Asia
channels within adjacent myocardial tissue than North America. Europe
can set up heterogenous refractory periods
necessary for development of reentrant COMPLIC ATIONS
rhythms - ventricular tachycardia/
• Ventricular fibrillation. high risk of sudden
fibrillation
cardiac death

CAUSES
• Inherited
O Autosomal dominant. variable
expression

127
( SIGNS & SYMPTOMS ) • Type I
, Right bundle branch block pattern
, Gradually descending ST elevations, at
• Brugada pattern
least 2mm (0.2mV) in leads V1-V3
o ECG findings, no symptoms
, Negative T- wave in leads V1-V3
• Brugada syndrome
• Type II
o ECG findings, symptoms of sustained
, Class IV antiarrhythmic can convert to a
ventricular tachycardia (palpitations,
Type I Brugada pattern-often needed
syncope, dyspnea, lightheadedness)
for diagnosis
, Saddle-back pattern with at least 2mm
J point elevation, lmm ST elevation
(~ __ D_IA_G_N_os_,s ) (positive/biphasic T wave)

LAB RESULTS
Genetic testing (....___ T_R_E_AT_M_E_N_T )
• Confirms diagnosis
• Brugada pattern: none

OTHER DIAGNOSTICS SURGERY

ECG
Implanted cardiac defibrillator (ICD)
• Type 1/11 Brugada electrocardiogram pattern
• Brugada syndrome
o May present simultaneously, may be
induced by certain drugs (e.g. calcium
channel blockers), or may resurface due
to unknown triggers

t
I 1-+-+-+-+-t-
!!::r+++:r:+=++1::J.=++++++++-t++--H-t-t-t-1=:tJ±:1jjtt1tt1ttn~'
I,

~~++++++-+-t-ttr I I II
I ! I I I I I I
I 1 I I I I J I

Figure 19.1 ECG (lead V1) demonstrating Brugada waveforms type I (left) and type II (right).

128
 \J~NT~llULAI?
TAU-\VC.AROIA V~NTRIC.ULAR F1&RILLAT,ON
\ r
R~E.NTi:l.ArJT LOOP NO~MAL BRUGAOA $VNOfOME-.
~MVTHM ~ ~HVTMM

1111111111 _,) E.C.6

=r:
* ST- ~LE:VATIONS.
~ Vu1tr'-'ulu,~roVI
* ~IGHT ~!\JO~ ~AIIJtH
~ \/t.n\:f\.(.les. o,im't
(!.LOC"

d~olnn51.nj no,vv,all~

Figure 19.2 Calcium channel blockers can increase the chance of developing Brugada syndrome.
The condition is typically associated with right bundle branch block, which makes the heart
susceptible to developing a reentrant rhythm, which in turn causes ventricular tachycardia and
sometimes ventricular fibrillation.

PULSELESS ELECTRICAL
ACTIVITY
osms.tl/ pulseless-elec-lTice1l-e1c-livi-l14
MNEMONIC: GTs & GHs
( PATHOLOGY & CAUSES ) Obstruction to blood flow
Tablets/toxins (drug overdose)
• Pulseless, despite electrical activity (evident
on ECG) typically resulting in pulse Cardiac Tamponade

• Heart does not contract in spite of electrical Tension pneumothorax

activity/does not generate enough cardiac Thrombosis (myocardial
output to cause pulse infarction)
• Survival -20% Thrombosis (pulmonary
embolism)
Trauma (hypovolemia - blood
CAUSES loss)
• Abrupt drop in preload
Hypovolemia
• Abrupt pump failure
Hypoxia
Hydrogen ions (acidosis)
Hyperkalemia/hypokalemia
Hypoglycemia
Hypothermia

129
 C..____
(__ SI_G_NS_&_SY_M_PT_O_M_s
) T_R_EA_~_M_EN_T
)
• Loss of consciousness MEDICATIONS
• Breathing stops • If cause unclear medicine used similar to
a systole
, I ntravenous/intraosseous line,
(..____ D_IA_GN_o_s,_s) administer epinephrine lmg/3-5
minutes
OTHER DIAGNOSTICS
• Absence of pulse

ECG
• Organized/semi-organized electrical activity

VENTRICULAR FIBRILLATION
osmsJI:/ ve n-l:Tieulo.T-fi\>Tillo.-1:ion
• Electrolyte imbalances: hypokalemia,
( PATHOLOGY & CAUSES ) hyperkalemia
• lschemia to ventricular muscle
• Ventricular electrical activity disorganized
• Scar tissue from previous myocardial
to point that coordinated contraction is
infarction
impossible
• Anatomical reentry
• Rapid, irregular electrical activity prevents
• Electrocution/external electrical stimulation,
ventricles from contracting in sync -
such as in unsynchronized cardioversion
cardiac output falls to zero
• If heart tissue stimulated during T wave
• Often due to tissue heterogeneity: heart
upslope (in an electrocardiogram), can
cells stressed/damaged, tissues of different
induce fibrillation
areas structurally, electrically different
• Mechanism: tissue heterogeneity in
cardiac electrical system - asynchronous (__ SI_G_NS_&_S_Y_M_PT_O_M_s )
depolarization & contraction - inadequate
blood pumped - oxygen deprivation -
• Chest pains, dizziness, nausea, rapid pulse,
death
dyspnea
• Functional reentry: arrhythmia causes
different areas of heart to depolarize &
contract out of sync - heart non-functional
(..____ D_IA_GN_o_s,_s)
CAUSES OTHER DIAGNOSTICS
• Medications causing long QT syndrome • Pulse check: no pulse
• Illicit drugs (e.g. methamphetamine,
cocaine) ECG
• Congenital arrhythmogenic syndromes (e.g. • Absence of PQRST waves; instead, fine,
Brugada, hypertrophic cardiomyopathy, coarse fibrillatory waves
arrhythmogenic right ventricular dysplasia, • Electrocardiogram appears chaotic
Wolff-Parkinson-White syndrome, • Undulating baseline
congenital long QT syndrome)

130
 OTHER INTERVENTIONS
( T_R_E~_~_M_EN_T
)
Cardiopulmonary Resuscitation (CPR)
SURGERY
Defibri llation
ICD • High energy shock depolarizes large
• Used when cause is unpreventable enough portion of tissue (critical mass) that
• Surgically implanted sinus node can take control
• Constantly monitors electrocardiogram
Electrophysiology study
• ICD recognizes ventricular fibrillation,
• If individual has had previous Ml/has
delivers responsive defibrillating shock
survived cardiac arrest in whom signs are
• Doesn't fix underlying condition; treats not apparent after routine, non-invasive
symptom, improves survival testing
• Primary prevention = Evaluate for possible ventricular
O Individuals with heart failure at risk of tachycardia ablation
ventricular tachycardia/fibrillation
• Secondary prevention Revascularization

° Cardiac arrest survivors for whom • If ventricular fibrillation occurs in setting of

triggers cannot be treated/prevented myocardial infarction
= Cardiac catheterization
= CABG

Figure 19.3 Histological appearance of fatal ventricular fibrillation demonstrating broken

myocardial fibers and squared-off nuclei.

131
Court"')' Q( Ja,on I! Rocd,gc<, CCT, CRAT

Figure 19.4 ECG demonstrating ventricular fibrillation.

132
 NOTES

NOTES
• SUPRAVENTRICULAR
TACHYCARDIA

GENERALLY.WHAT IS IT?
( PATHOLOGY & CAUSES ) ( D_IA_GN_O_s,_s __ )
• Abnormally fast heart rhythms due to LAB RESULTS
inappropriate electrical activity in upper • Electrolytes, thyroid stimulating hormone
portion of heart, atria/atrioventricular (AV) levels
node
• Ventricles contract> 100 beats per minute,
OTHER DIAGNOSTICS
pathology originates above ventricles
• ECG
O Ventricles protected by gating at AV
node
( T_R_E~_~_M_EN_T__ )
CAUSES
• Reentry conductive loops • See individual disorders
• Increased automaticity
• Triggered activity MEDICATIONS
• Calcium channel blockers, beta blockers,
RISI( FACTORS anticoagulants
• Cardiac: coronary artery disease, heart
failure SURGERY
• Non-cardiac: chronic obstructive pulmonary • Catheter ablation
disease (COPD), pulmonary embolism,
alcohol abuse, hyperthyroidism

(__ s,_G_NS_&_S_Y_M_PT_O_M_s_)
• Palpitations, chest pain, anxiety, dyspnea,
syncope, lightheadedness

133
 ATRIAL FIBRILLATION
osms.i-1:/C1-lTiC1l-fi\>rillo..l:ion

( PATHOLOGY & CAUSES ) ( s,_G_NS_&_S_Y_M_PT_O_M_

• Rapid, irregular (no discernible rhythm) • May be asymptomatic
heart rate • Dyspnea, fatigue, palpitations,
• Progression lightheadedness, weakness, chest pain,
O Paroxysmal: intermittent rhythm, hemodynamic shock
may revert back to sinus rhythm
spontaneously
O Persistent:> seven days, requires ( D_IA_GN_O_s,_s
__ )
intervention to convert back to sinus
rhythm LAB RESULTS
O Permanent: long-standing atrial • Thyroid stimulating hormone (TSH) levels:
fibrillation, cardioversion unsuccessful exclude hyperthyroidism

CAUSES DIAGNOSTIC IMAGING

• Disorganized waves of atrial depolarization,
exact mechanisms not well understood
O Regular impulses of sinus node
overwhelmed by rapid electrical
discharges from various sources
(automatic foci, multiple reentry
phenomena)
O Arise from left more than right atrium
Transthoracic echocardiogram
• Evaluate atrial, ventricular size; valvular
disease; left ventricular function; pericardia!
disease
Transesophageal echocardiogram
• Evaluate for atrial thrombi

OTHER DIAGNOSTICS
RISI( FACTORS
• Old age: affects 4% 60-70, 14% > 80 ECG
• Obesity, diabetes mellitus, excessive • Absent P waves
alcohol consumption, genetic predisposition • Irregularly timed QRS complexes (irregular
• Cardiovascular disease: heart failure, R-R intervals)
hypertension, coronary artery disease, • No sawtooth wave in atrial fibrillation
non-rheumatic mitral regurgitation, mitral
valve prolapse, rheumatic heart disease,
damaged atrial myocytes
• Increased catecholamine levels
• Lung disease
• Hyperthyroidism

COMPLICATIONS
• Thromboembolic events, heart failure,
hypotensive shock

134
 OTHER INTERVENTIONS
( T_R_E~_;,-_M_EN_T
)
Rhythm control
MEDICATIONS • Restore sinus rhythm via cardioversion

Anticoagulation Electrical cardioversion

• E.g. warfarin, dabigatran, apixaban, • Defibrillator for synchronization
rivaroxaban
• CHA2DS2-VASdCHADS2 score Catheter ablation
O Estimate risk of stroke in non-rheumatic • Destruction of heart regions responsible for
atrial fibrillation; higher score = greater abnormal impulses
risk of stroke
O Score O (biological male)/1 (biological
female): low risk, no anticoagulation
recommended
O Score 1 (biological male): moderate risk,
consider anticoagulation
O Score ;;: 2: high risk, anticoagulation
recommended
O See table of scores +l

Rate control
Hypertension: consistently
• < 100 beats per minute > 140/90 mmHg
+1

• Beta blockers (preferably 131 selective)

• Non-dihydropyridine calcium channel Age: ~ 75 years +2
blockers (e.g. diltiazem, verapamil)
Diabetes mellitus +1
• Digoxin
Prior stroke, TIA,
Chemical cardioversion +2
thromboembolism
• Administer antiarrhythmic medication
Vascular disease: peripheral artery
• Class le antiarrhythmics disease, myocardial infarction, +1
• Class Ill antiarrhythmics aortic plaque

• Maintenance of sinus rhythm after

Age: 65--74 years +1
cardioversion
° Class le antiarrhythmics Sex: biological female +l
° Class Ill antiarrhythmics

135
Figure 20.1 An ECG demonstrating atrial fibrillation.

ATRIAL FLUTTER
osms.i"l/o.-lTio.1-flu-HeT
• Isthmus-independent
( PATHOLOGY & CAUSES )
• Reentrant circuit develops in either atrium
• Atria depolarize regularly at very high rates • Associated with variety of reentry loops
(200-350bpm). appear to flutter (common after incomplete atrial ablation
procedures, right atrial surgical scars)

TYPES
CAUSES
Typical atrial flutter (AKA Type 1 flutter) • Reentrant electrical signal from either
• More common atrium
• Single reentrant circuit, right atrium • Reentrant signal loops back on itself
• Isthmus-dependent: reentry circuit crosses ----> overrides normal sinus rhythm ---->
cavotricuspid isthmus establishes endless loop of stimulation
• Circles tricuspid annulus (ring), usually • Underlying disease (e.g. heart failure,
counterclockwise (viewed from below) valvular disease, hypertension, pulmonary
disease) ----> heart cells less electrically
• Cavotricuspid isthmus tissue propagates
signal slower than surrounding tissue stable ----> alters refractory periods ---->
increased risk of reentrant circuits
----> circuit loops ----> slows propagation ---->
surrounding tissue exits refractory period • Reentrant circuits initiated by premature
atrial contraction (PAC)----> partial premature
Atypical atrial flutter (AKA Type 2 flutter) contraction, normal tissue relaxes=- wave
• Less common of stimulation propagates ----> normal tissue

136
 contracts, premature tissue recovers -
chance of reentrant circuit, stimulation ( D_IA_GN_O_SI_S
)
wave doubles back on itself
DIAGNOSTIC IMAGING
RISI( FACTORS Echocardiogram
• Diseases that change atrial heart cell • Evaluate size of right, left atria, ventricles
properties - differing electrophysiological • Detect pericardial/valvular heart disease
properties in adjacent areas - reentry • Decreased ejection fraction (% of blood
circuit pumped by heart per contraction)
• lschemia, fibrosis, previous myocardial
infarction, heart failure, high blood pressure,
diabetes, valvular heart disease, obstructive LAB RESULTS
sleep apnea • Serum electrolytes
• Renal function

COMPLICATIONS • Thyroid stimulating hormone (TSH) levels:

exclude hyperthyroidism
• Heart failure, thromboembolic events, atrial
fibrillation
OTHER INTERVENTIONS
(__ s,_G_NS_&_S_Y_M_PT_O_M_s_) ECG
• Typical P waves absent
• Palpitations, tachycardia, fatigue • Typical atria/ flutter: P waves, saw tooth
• Pain/tightness/discomfort in chest shape (F waves) localised to leads 11, 111, aVF
• Heart failure • Atypical atrial flutter: atrial activity
O Exercise intolerance (sawtooth waves/otherwise) may occur
anywhere, dependent on reentrant circuit
O Difficulty breathing at night/while lying
location
flat
• Ventricular rate usually 1/2 atrial flutter rate
O Edema of legs, abdomen
(even ratios 2:1, 4:1 more common than
odd, 3:1, 5:1)
• 1:1 atrial: catecholamine excess, presence
of accessory bypass tract/class lA, lC
antiarrhythmic drug therapy

Cl'VOT~\tuSP\D \S1l,UAUS
* ~ro~aio.tes ~)s~al
l't'IOr€. S\oo.J\~

' ['fe~c.+o'<~ ~ri otJ.

Figure 20.2 An electrocardiogram demonstrating atrial flutter with a 3:1 AV nodal block. The atrial
trace demonstrates a characteristic sawtooth pattern.
137
Figure 20.3 An electrocardiogram demonstrating atrial flutter with a 3: 1 AV nodal block. The
atrial trace demonstrates a characteristic sawtooth pattern.

SURGERY
C..____ T_R_EA_:T_M_EN_T
)
Radiofrequency catheter ablation
MEDICATIONS • Cavotricuspid isthmus no longer able to
• Anticoagulants (reduce chance of clot carry electrical signal, prevents reentry
formation), beta blockers/calcium channel
blockers (control rates of ventricles)
OTHER INTERVENTIONS
Electrical cardioversion
• Depolarize atrial tissue, resynchronize
contraction

138
 ATRIOVENTRICULAR NODAL
REENTRANT TACHYCARDIA
(AVNRT)
osms.tl/ av-nodal-reen-b·an-l--laeh14eardia

RISI( FACTORS
( PATHOLOGY & CAUSES ) • Biologically-female tndtviduats-« 75%
of cases, emotional stress ---'> alcohol use
• Heart rate disturbance due to accessory
disorder, hyperthyroidism, electrolyte
pathway in/near AV node
disturbances
• Electric conduction splits into two
pathways ---'> forms loop
O Alpha pathway: slow conduction, short (__ s,_G_NS_&_S_Y_M_PT_O_M_s_)
refractory period
O Beta pathway: fast conduction, long • Palpitations, transient chest pain, bouts
refractory period of transient tachycardia, transient
hypotension, (pre)syncope
TYPES
Slow-fast/"typical" AVNRT ( D_IA_G_N_os_,s )
• Anterograde conduction to ventricles via
slow pathway (alpha) LAB RESULTS
• Retrograde to atria conduction via fast • Thyroid function
pathway (beta) • Serum electrolytes
• Depolarization down both pathways---'>
reaches end of beta pathway ftrst-« signal
splits
OTHER DIAGNOSTICS
O Travels to ventricles ---'> contraction ECG
O Travels up alpha pathway---'> meets slow • Tachycardia 140-280bpm
signal ---'> signals cancel each other out • Absent P waves
• Depolarization wave from premature beat • P waves immediately before/after QRS
reaches AV node c- refractory fast (beta) complex
pathway---'> signal initially down alpha
• P waves inverted/retrograde
pathway only---'> splits
• R' waves (small secondary R waves)
O Travels to ventricles ---'> contraction
O Travels up beta pathway---'> signal
travels down alpha pathway, beta ( T_R_E~_~_M_EN_T
__ )
pathway comes out of refractory period
---'> signal reaches end of alpha pathway MEDICATIONS
-'>Splits back up beta pathway
• Adenosine. beta blockers. calcium channel
Fast-slow/"atypical" AVNRT blockers to slow AV node conduction
• Anterograde conduction via fast pathway,
retrograde conduction via slow pathway

139
OTHER INTERVENTIONS Slow AV node conduction
• Vagal maneuver (carotid sinus massage/
Radiofrequency catheter ablation
Valsalva maneuver) - activates vagus
• Definitive treatment nerve
• Ablation of slow alpha pathway

(AVtJ 12T)
Al9-1D\J~NTP.IC.ULAIZ. NODAL '2-~E.tJTl2AtJT l1"'-'t'l'C.~i?-Dl~
(A1~1Al L ~IU.tJfitlWLPI~
~TA ?Ail-lwAY
• SL.ow C.ot.i1>Vq101-J
• t~ST (O~Dumoµ
• S\lo~:1~eFP.Ac.To!'#
(__ Ptf1ro J • U, ... 4 ~t-Fl?A(ll)~'(
PEf'IOl)

-6~, ~ lot
tU>lt ~
,OV1'1uct ial"-

Figure 20.4 Illustration depicting path of electrical conduction in AV node during slow-fast
AVNRT.

, .. l,T ,.
:1
::, ,., ..... 1.::,-, ·~ h - 111 I- ·:!1: I su;
11

:-~1= :t-' · ,:., · T '1 i; ' : :!

. '"'' ~ - . .; :· " r:: ;: :,. ), = ;, ,, I· · =. ... :_ 1;,·, =t = ,, ·::·
r-:1~!·:·

Ii: .. ;. . i:., L: .. ..'.;; J. .: J .. • .;.

, i " '-·,;;"-
~A '1-r'-".-
.: ;"· ":: .... : .' .......:; . :: "'· ,:: ,+ ·+-_i.:i: :: :: : ;,; 1!=,.;,:r• r.

..,..: ;'' i ,. I i t ,.- ·, J ... ~ : : I . I . I

., ·,.,,. +,,, ·c:n ··rte
rl" ... ~. • --t.
,I'
.A..
;''I .: f
···u
II': 1 +. . 1: ::' ,·!t: " ·.: r ::::nl'

· .:1. ,: •.• _;l

• :_·, .r :"""' · i ,· ·r· · r: --+·.-_- :·.---i i'·:' 1: · T .,.. ; ' . 'l . ·. : : : r.'

I
Mi\C3$00SB,i- -:l-·t~~~-=-
Figure 20.5 An ECG demonstrating typical (slow-fast) AVNRT. R waves are best seen in lead
Vl.
140
 NOTES

NOTES
• VASCULAR COMPRESSION
SYNDROMES

GENERALLY,WHAT ARE THEY?

( PATHOLOGY & CAUSES ) ( D_IA_GN_o_s,_s __ )
• Range of congenital/acquired anatomical DIAGNOSTIC IMAGING
compressions of vasculature/by vascular • X-ray, CT scan, ultrasound
structure
• Acquired
OTHER DIAGNOSTICS
O Rapid changes in weight - changes to
• Physical exam
fat pad cushioning between vasculature,
other structures - compression
• Vasculature squeezed between two ( T_R_E~_~_M_EN_T__ )
structures - ischemia (artery)/vascular
engorgement (vein) SURGERY
• Vasculature compresses, obstructs another • See individual disorders
structure - obstruction

OTHER INTERVENTIONS
(__ s,_G_NS_&_S_Y_M_PT_O_M_s_) • Weight gain/loss

• Vascular obstruction - ischemia

O Pain. nausea. vomiting, weakness, cold,
pulseless extremity
• Organ obstruction
O Pain, nausea. vomiting, weight loss

141
 NUTCRAC~ERSYNDROME
osms.i"l/ nu-l:eTe1elceT-s14ndTome

( PATHOLOGY & CAUSES ) (__ s,_G_NS_&_SY_M_PT_O_M_s_)

• Left renal vein squeezed between superior • Left flank pain
mesenteric artery, abdominal aorta • Hematuria
• Three unpaired arteries • Nausea, vomiting (compression of
° Celiac, superior mesenteric, inferior splanchnic veins)
mesenteric • Individuals who are biologically male
• Aortomesenteric angle , Scrotal mass - varicocele
O Between aorta. superior mesenteric (engorgement of testicular veins)
artery
• Aortomesenteric angle reduced - arteries
pinch left renal vein - prevents blood ( D_IA_GN_O_s,_s __ )
return to heart - blood backs up in left
kidney - renal hypertension - small DIAGNOSTIC IMAGING
breaks in renal blood vessels - blood in
urine Ultrasound, Doppler, CT scan, MRI, conven-
tional angiography
• Aortomesenteric angle may decrease to 6°
• Left renal vein stenosis, reduced
• Blood may back up in left testicle
aortomesenteric angle
• Reduced blood flow through left renal vein
CAUSES on Doppler
• Young people: reduction of aortomesenteric • Collateral circulation
angle due to normal growth • Dilated testicular veins - varicocele
• Adults: extreme weight loss due to severe
illness (e.g. HIV/AIDS, cancer, anorexia
nervosa). compressing tumors (e.g. (._____ T_R_EA_~_M_EN_T__ )
pancreatic)
SURGERY
COMPLICATIONS • For tumors
• Varicocele , Move vein, place stent
O Left testicular vein drains into left renal
vein - blood backs up into left testicle OTHER INTERVENTIONS
• Ovarian vein syndrome • Weight gain
O Dilated ovarian vein compresses ureter , Increase mesenteric fat pad - widen
- abdominal/back/pelvic pain aortomesenteric angle - relieve
• Renal vein thrombosis compression

142
 flit.JAL
M'I'P~P.1€.~SIOtJ
.-.:..;
~IZlOMt~WTEltlL
~4L.E<:~s·

FAT

Figure 21.1 An illustration demonstrating the pathophysiology and sequelae of

nutcracker syndrome.

SUPERIOR MESENTERIC ARTERY

SYNDROME
osms.i'l/ supeTioT-mesen-leTie-ciT-leT14-s14nd Tome

• Post-scoliosis surgery
( PATHOLOGY & CAUSES )
• Congenital anatomic abnormalities
• Vascular structures compressing another = Ligament of Treitz too short
structure , Superior mesenteric artery branches off
• Distal third of transverse section of aorta further down
duodenum compresses between abdominal
aorta, superior mesenteric artery COMPLICATIONS
• Three unpaired arteries • Small bowel obstruction
, Celiac, superior mesenteric, inferior • Severe malnutrition, wasting - increases
mesenteric compression, worsens condition
• Mesenteric fat pad thins out - reduces
aortomesenteric angle - aorta,
superior mesenteric artery pinch down
on transverse duodenum - intestinal
obstruction

CAUSES
• Extreme weight loss
, 111 ness/intentional

143
 (
(__ SI_G_NS_&_SY_M_PT_O_M_s
) T_R_EA_~_M_EN_T
)
• Gradual/quick onset; may be intermittent SURGERY
• Early satiety; bilious emesis; weight • E.g. ligament of Treitz - allow duodenum
loss; abdominal distention; burping; to move freely
hypersensitive abdomen; reflux, heartburn
• Relieved when in left lateral decubitus OTHER INTERVENTIONS
(knee-to-chest) position/prone position;
• Management
with Hayes maneuver (apply pressure
below umbilicus towards head, spine) , Nasogastric tube to decompress
stomach, early duodenum; fluids,
electrolytes
• Weight gain (regrow mesenteric fat pad);
( D_IA_GN_O_s,_s) may require feeding tube past obstruction

DIAGNOSTIC IMAGING
Abdominal X-ray
• Dilated fluid/gas-filled stomach, proximal
duodenum

CT scan with oral contrast/MRI

• Vascular compression of third part of
duodenum, reduced aortomesenteric
angle, collapsed small bowel distal to SMA
crossing

Abdominal ultrasound
• Dilated proximal duodenum, stomach

OTHER DIAGNOSTICS
• High-pitched bowel sounds Figure 21.2 An abdominal CT scan in
• Succussion splash the axial plane demonstrating superior
O Sloshing sound of built-up gas, fluid in mesenteric artery compression syndrome.
distended digestive tract upstream of The third part of the duodenum (outlined) is
obstruction compressed between the superior mesenteric
artery and the aorta.

144
 NOTES

NOTES
• VASCULAR MALFORMATIONS

GENERALLY,WHAT ARE THEY?

( PATHOLOGY & CAUSES ) ( D_IA_GN_O_s,_s
__ )
• Developmental malformations of the DIAGNOSTIC IMAGING
vascular system • Imaging studies usually not needed

OTHER DIAGNOSTICS
(__ SI_G_NS_&_S_Y_M_PT_O_M_s_)
• History
• Usually asymptomatic • Physical examination
• Large malformations can cause obstruction
/impair organ functions
• Diverts away high volume of blood ----. heart
( T_R_EA_:T'_
__M_EN_T
)
compensates ----. high-output heart failure
• Generally no need for treatment unless
formations bleed/cause other problems

SURGERY
• Surgical/laser therapy considered for skin
ma I formations

I-Arttri•~---i----(api11 ..
vi•~ ----4-Vt.iv,~--4
"\._, 6<>,np~M a.rtV!QI
:flOuJ

- -·

Figure 22.1 Illustration depicting changing pressure between normal arteries and veins .
145
 ARTERIOVENOUSMALFORMATION
osms.it/ o.TteTiovenous-mo.lfoTmo.tion
Brain
( PATHOLOGY & CAUSES ) • Epilepsy, neurological deficits, abnormal
sensations (numbness, tingling, pain)
• Abnormal capillary bed formation between
• Sudden onset, severe headache indicates
artery and vein
subarachnoid hemorrhage
• Capillary bed does not form, leaving
• Difficulty with movement coordination,
arteries connected directly to veins ---->
muscle weakness, paralysis, vertigo
tangling ----> nidus
• Apraxia (physical difficulties performing
• Arteriovenous fistula: single artery to single
actions upon command, though command
vein direct connection
is understood)
• No capillaries----> arteries, veins subjected
to high pressures----> arteries dilate ----> veins Lungs
thicken (fibrosis) • Asymptomatic 1/3 of time
• AVM expands over time----> compresses • Dyspnea, cough, clubbing, cyanosis,
surrounding tissues ----> decreases blood hemoptysis, chest pain
flow
• Platypnea (shortness of breath in upright
° Can cause bruit (whooshing noise heard position, relieved by lying down)
on auscultation)
• Orthodeoxia (decrease in oxygen saturation
O Noise can get loud enough that person bv z 2% when rising from lying down to
with the bruit can hear it, causing standing)
psychological stress, hearing issues and
sleep issues Spine
• Muscle weakness, paralysis
CAUSES Pediatric
• Possibly congenital/genetic
• May present with
• Osler-Weber-Rendu disease/hereditary
, Heart failure, macrocephaly, prominent
hemorrhagic telangiectasia connected to
scalp veins
AVM

RISI( FACTORS ( D_IA_GN_o_s,_s __ )

• Biological male, 10-30 years old, genetic
conditions (e.g. Osler-Weber-Rendu) DIAGNOSTIC IMAGING
Angiography
COMPLICATIONS • Functional anatomy of AVM
• lschemia at site, high output heart failure, ° Feeding arteries: stenosis
rupture, haemorrhage O Sharp twisting of veins (ectasia)
O Arterial and venous dilatation
(__ SI_G_NS_&_SY_M_PT_O_M_s_)
CT scan/MRI
• Anatomy of AVM
• Most are asymptomatic
O Irregular/bulbous masses in brain/
• Depend upon location
brainstem
• Presence of bruit on auscultation

146
OTHER DIAGNOSTICS
• Bruit

( T_R_E~_~_M_EN_T__ )
• Treat all arteriovenous malformations

SURGERY
Radiosurgery
• Radiation beams target AVM to close
vessels

Endovascular embolization
• Catheter placed to obstruct vessels
Figure 22.2 CT scan of the head in the
axial plane demonstrating an arteriovenous
malformation in the left parietal lobe.

CAVERNOUS HEMANGIOMA
osms.l"l:/eo.veTnous-hemo.nglomo.
RISI( FACTORS
( PATHOLOGY & CAUSES ) • Von Hippel-Lindau disease

• Benign tumor formed from tangle of

unencapsulated, dilated, thin-walled blood COMPLIC ATIONS
vessels • Rupture---> hemorrhage
• Spherical caverns form from dilatation of • Obstructive jaundice, clotting disorders
vessels separated by fibrous connective • lntravascular thrombosis
tissue • Dystrophic calcifications
• Characteristic "mulberry" presentation

TYPES (__ s,_G_NS_&_S_Y_M_PT_O_M_s__ )

Cerebral cavernous malformations • Usually asymptomatic

Liver cavernous hemangioma Brain

• Sometimes found in people with previous • Brain tissue cornpression-s- seizures,
pregnancies ---> high estrogen levels hemorrhages
thought to be a factor • Stroke, speaking difficulties, memory/
attention difficulties
Ocular cavernous hemangioma
• Headaches, balance issues

CAUSES Liver
• Genetic mutations • Pain in right upper quadrant
° CCM1, CCM2, CCM3 • Gastrointestinal issues: nausea, vomiting,
loss of appetite, early fullness after eating
147
Eyes
• Double/decreased vision, proptosis
(...____ T_R_EA_~_M_EN_T
__ )
• Monitoring usually sufficient
(...____ D_IA_GN_O_s,_s __ )
MEDICATIONS
DIAGNOSTIC IMAGING • Facial hemangioma growth slowed with
steroids
MRI
, Administered orally/injected at site
• Seen as popcorn/mulberry shape -
pathognomonic Sclerotherapy
• Hepatic cavernous hemangiomas best • Close tumor's blood supply - tumor
diagnosed with shrinks
o Ultrasound
IV contrast CT scan
o
SURGERY
• Can be mistaken for metastatic tumors • Prevent damage to healthy tissues

Figure 22.4 A cavernous hemangioma from

the liver, composed of large vascular spaces
Figure 22.3 Abdominal CT scan in the axial with thin walls. No normal liver is present.
plane showing a hemangioma of the liver.

148
 HEMANGIOMA
osms.tl/hemo.ngiomo.
black, crusted, sometimes painful
( PATHOLOGY & CAUSES ) ulceration
, > five hemangiomas----> ultrasound for
• Hemangioma/infantile hemangioma (IH):
hepatic hemangioma
benign vascular skin tumor
• Deeper hemangiomas: bluish lesions,
O Originates from endothelial cells lining
poorly defined
blood vessels
n Can proliferate into bigger tumors
• Most frequent tumors of infancy
O Some have superficial capillary
• Develop a few days after parturition
counterparts visible over/surrounding
O If IH appears to be present at birth=-s affected area
not IH O Slower development
• Compound hemangiomas: deep IH +
RISI( FACTORS superficial IH
• Intrauterine hypoxia
• Localised hypoxia
( D_IA_GN_o_s,_s __ )
• Biological females more at risk
• Premature underweight infants DIAGNOSTIC IMAGING
• Usually no need for imaging
COMPLICATIONS
Doppler ultrasound
• Usually none
• High vessel density and high peak arterial
• Easily infected, start bleeding
Doppler shift, may show peripheral feeding
• Possible ulceration ----> infection vessels
• Usually psychosocial: mostly appear on
highly visible areas (face, neck, etc.) MRI
• Multiple IH in newborns can indicate liver • Multiple high signal intensity lobules (due
hemangiomas to cavernous/cystic spaces containing
stagnant blood); often heterogeneous

(__ SI_G_NS_&_S_Y_M_PT_O_M_s_)
LAB RESULTS
• Usually no need for biopsy unless history
• Appear in first weeks of life
unclear; used to confirm diagnosis
• Tumors can grow for s six months after
birth
• Most regress over time, around five years OTHER DIAGNOSTICS
old
History and physical exam
• Can leave marks: fibroadipose tissue,
• History of onset, age, growth pattern,
changes in pigmentations, scars
duration, treatment
• Resemble red/white patch, blemish; can be
found all over body
• Superficial lesions: bright red, flat/raised
from the skin ----> papules AKA "strawberry
hemangiomas"
O Exposed areas: if grazed, can produce

149
C..__ __ T_R_E~_~_M_EN_T
)
• Most do not require treatment, regress in a
few years
• Exceptions
° Functional issues: feeding (laryngeal
hemangiomas), obscuring vision
O Bleeding
O Hepatidcardiac complications
O Permanent scars causing disfigurement
Figure 22.5 A capillary hemangioma on the
MEDICATIONS abdomen of an infant.
• Beta blockers, oral corticosteroids
• Antiangiogenic therapies
O Usually intravenous: interferon-alfa 2a,
2b, vincristine

SURGERY
• Rarely, surgery/laser therapy ...

Figure 22.6 Histological appearance of a

capillary hemangioma showing multiple small
vascular spaces containing red blood cells.

LYMPHANGIOMA
osms.i"l/l14mpho.ngiomo.
layer; fluid-filled cavities; indistinct margins
( PATHOLOGY & CAUSES ) • One/more cysts 2: 2cm3/0.8in3 in size
• Tends to develop in deep soft tissues;
• Benign lymphatic malformations
propensity for rapid growth, local invasion
• Composition: lymphatic channels/cysts of muscle, bone, underlying tissue
• May present at birth/develop during first
few years of life Lymphangioma circumscrip
tum
(microcystic)

TYPES • AKA simple/capillary lymphangioma

• Composition: capillary-sized endothelial-
Cystic hygroma (macrocystic) lined lymphatic networks
• AKA cavernous lymphangiomas • One/more cysts< 2cm3/0.8in3 in size
• Composition: cystic masses. dilated • Tend to develop in superficial tissues
lymphatic networks with thin endothelial

150
Mixed
• Contains variously-sized cysts
(..____ D_IA_GN_O_s,_s
__ )
DIAGNOSTIC IMAGING
CAUSES
• Failure to form communication between MRI
lymphatic, venous circulation (e.g. jugular • Multiloculated fluid-filled cystic lesions;
lymph sac, internal jugular vein) during fetal identifies lesion's extent
development
Ultrasound
• May be sporadic/associated with genetic
• Prenatal cystic mass detection; j nuchal
mutations (e.g. VEGFR3 germline mutation)
translucency (hypoechoic space)

RISI( FACTORS Histopatholog

y
• Chromosomal anomalies • Cystically dilated channels lined with flat
O Turner syndrome, Down syndrome, endothelial cells, filled with lymphatic fluid;
Noonan syndrome (cystic hygroma) deeper vessels are large, contain smooth
muscle

COMPLICATIONS
• Lymphedema, recurrent cellulitis ( T_R_E~_iT_M_EN_T
__ )
• Disfigurement
• Lesion recurrence SURGERY
• Fetal hydrops, fetal death, obstructed labor, • Resection
chylous pleural effusion, chylopericardium,
airway compression (cystic hygroma) OTHER INTERVENTIONS
• Image-guided percutaneous chemoablation
• Sclerotherapy
(__ s,_G_NS_&_S_Y_M_PT_O_M_s_)
Cystic hygroma
• Large (up to 15cm/5.9in), poorly-defined
soft, fluctuant mass
° Commonly located in deeper layers of
cervicofacial region. axilla, lateral chest
wall
° Covered with normal skin

Lymphangioma circumscriptum
• Slightly elevated lesion
° Commonly located in head (oral cavity),
trunk, proximal extremities, axillary
region
O May appear as translucent/hemorrhagic
vesicles
O Overlying skin may be reddish-purple
color secondary to small hemorrhages,
thrombus formation/may contain wart-
like lesions Figure 22.7 CT scan in the coronal plane
demonstrating a large cystic hygroma on the
left side of the neck.

151
 Capillary bed does not
compression ischemia; most Bruit common;
form; arteries directly
asymptomatic; more symptoms Ox with angiography
connected to veins
when in brain, lungs, spine

Can rupture, obstruct,

Tangle of unencapsulated, thrombose. or calcify; most "Popcorn" or "Mulberry"
thin-walled blood vessels asymptomatic: more symptoms shape on MRI
when in brain, liver, eye

Most common tumor of

Endothelial cells lining
infancy: usually no
blood vessels: red. white, Physical exam
complications: can grow for
or blue patch
6 months

Congenital lymphatic Prenatal: fetal ultrasound

Prenatal: fetal hydrops: Postnatal: gross
cyst-like malformation examination
airway compression (cystic
between jugular lymph hygroma): disfigurement
sac, internal jugular vein MRI identifies
lesion's extent

Figure 22.8 Histological appearance of

a lymphangioma composed of dilated
lymphatic spaces, lined by simple
endothelium.

152
 NOTES

NOTES
• VASCULAR STEAL SYNDROMES
GENERALLY,WHAT ARE THEY?
( PATHOLOGY & CAUSES ) ( D_IA_GN_O_SI_S
__ )
• AKA steal syndrome DIAGNOSTIC IMAGING
Occlusion - blood follows path of least
O • Incidental finding
resistance - abnormal blood flow
CT angiography
• Hemodynamics
• Blood flow/occlusion
O Length, width of vessel
• Obstruction/narrowing of vessel - Doppler ultrasound
increased resistance - blood follows • Retrograde blood flow
path of least resistance - area distal to
obstruction/narrowing receives less blood,
others receive more blood LAB RESULTS
• Atherosclerosis, elevated troponin

TYPES
• Coronary steal syndrome OTHER DIAGNOSTICS
° Coronary arteries • Nuclear stress test
• Subclavian steal syndrome • ECG alterations
O Subclavian artery • Heart catheterization

CAUSES ( T_R_E~_~_M_EN_T
__ )
• Narrowing/obstruction of vessel
• Atherosclerosis/structural abnormalities MEDICATIONS
• Pharmacological treatment; see individual
disorders
(__ SI_G_NS_&_S_Y_M_PT_O_M_s_)
• See individual disorders SURGERY
• Revascularization of ischemic area
O Endovascular methods, bypass surgery,
percutaneous transluminal angioplasty

153
 CORONARY STEAL SYNDROME

• Drugs
( PATHOLOGY & CAUSES ) , Dipyridamole, nitroprusside, isoflurane
(inhaled anesthetic), vasodilators
• Narrowed/obstructed coronary vessel
+ vasodilator alters cardiac circulation • Coronary arteriovenous fistula between
coronary artery, cardiac chamber
- blood shunted away from area distal
to narrowing/obstruction exacerbating
ischemia COMPLICATIONS
• AKA cardiac steal syndrome • Recurrent myocardial infarction (Ml),
• Artery narrowing/obstruction - dilation of ischemia
distal arteries to compensate for decreased
blood flow - addition of vasodilator -
dilation of resistance vessels - blood (__ SI_G_NS_&_S_Y_M_PT_O_M_
)
supplying ischemic zone shunted away to
areas of least resistance - more ischemia • Cerebrovascular
• Narrowing of coronary arteries+ , Presyncope/syncope, vertigo, vision loss,
vasodilator (e.g. dipyridamole, adenosine) memory loss, weak pulse
- blood flows to non-obstructed vessels • Chest pain
- exacerbating ischemia • Unequal pulses in upper extremities
O Dipyridamole: antiplatelet, vasodilator • Blood pressure differences between arms
- all coronary vessels dilate when in
individual with partial obstruction of
coronary artery
(..____ D_IA_GN_O_SI_S
)
O Vasodilator may steal blood from
deprived region distal to obstruction OTHER DIAGNOSTICS
• Dilation of resistance vessels - blood • Cardiac stress test
shunted away from coronary vessels
, Vasodilator produces ischemic ECG
changes (with/without exercise)
CAUSES • Coronary angiography
• Coronary artery bypass grafting surgery
(CABG)
O Rare C T_R_EA_:l"_M_EN_T
)
O Due to left internal mammary artery
(LIMA) graft
SURGERY
• Balloon angioplasty, stent insertion,
O Retrograde flow from LIMA to left
coronary bypass
subclavian artery

154
 SUBCLAVIAN STEAL SYNDROME
osmsJI:/ su \>elo.vio.n-s-leo.l-s14ndY-ome
RISI( FACTORS
( PATHOLOGY & CAUSES ) • Smoking, diabetes, obesity, lack of exercise,
unhealthy diet, family history
• Stenosis/occlusion in subclavian artery---->
• More common in individuals who are
reversal of blood flow in vertebral artery
biologically male
• Occlusion/narrowing in subclavian artery
----> blood drawn away from head, flows
retrogradely to supply oxygen to upper COMPLIC ATIONS
extremities (e.g. blood to brain stolen to • Upper limb ischemia, neurological problems
supply left upper limb)
O More often on left than right due to
anatomical location of left subclavian
artery
• Narrowing of subclavian artery----> short
low resistance pathway becomes high
resistance
• Blood flows up right brachiocephalic---->
right subclavian ----> right vertebral artery
----> basilar artery, left vertebral joins---->
blockage of left vertebral upstream ---->
blood from right vertebral artery enters left
vertebral ----> left subclavian ----> flows back to
left arm
• Rare condition

CAUSES
• Atherosclerosis (most common)
O Narrowing, hardening of arteries due to
plaque buildup
• Takayasu disease (least common)
° Chronic inflammation of aorta. large
vessels
• Giant cell arteritis
• Blalock Taussig shunt
O Surgical procedure to increase blood
flow to lungs; tube placed between
subclavian. pulmonary arteries
• Thoracic aortic dissection
• Thoracic outlet compression
• Interrupted aortic arch Figure 23.1 An illustration depicting the flow
• Congenital aortic coarctation of blood in subclavian steal syndrome. Blood
flows around the blockage in the proximal
subclavian artery, reversing flow in the
internal carotid and "stealing" the blood from
the brain.
155
(__ SI_G_NS_&_SY_M_PT_O_M_s
)
• Asymptomatic
• Numbness of arm, extends to fingertips
(most frequent)
• Vertebrobasilar artery insufficiency
O Presyncope/syncope, neurologic deficits
• Upper extremity claudication
• Tingling sensation/numbness in face
• Decreased blood pressure on affected side
• Transient hemiparesis (weakness) of
affected side
• Blood pressure (BP) in left arm < BP in right
arm
• Pulse in left arm< pulse in right arm

(...____ D_IA_GN_o_s,_s)
DIAGNOSTIC IMAGING
• CT angiography
• Doppler ultrasound scan

C...____ T_R_EA_:T_M_EN_T
)
SURGERY
• Balloon stenting, angioplasty
• Endarterectomy

156
 NOTES

- NOTES
•
-
VASCULAR TUMORS
GENERALLY,WHAT ARE THEY?
( PATHOLOGY & CAUSES ) (.____ D_IA_GN_O_SI_S
)
• Abnormal growths of blood/lymph vessels DIAGNOSTIC IMAGING
• Can be benign/malignant, can occur • Visual identification, imaging studies (MRI,
anywhere in body CT scan, ultrasound with Doppler, biopsy)
• Vascular tumors are rare, but most , Determine location, tumor size, extent of
commonly found in neonates (e.g. spread
hemangiomas), HIV-positive individuals
(e.g. Kaposi's sarcoma)
LAB RESULTS
• Biopsy for definitive diagnosis
COMPLICATIONS
• Metastasis
• Complications from chemo/radiation (.____ T_R_E~_~_M_EN_T
__ )
therapy
• Depends on type, location, severity,
malignancy; see individual disorders
(__ SI_G_NS_&_S_Y_M_PT_O_M_s_)
• See individual disorders

ANGIOSARCOMA
osms.i"l/ o.ngioso.Teomo.
( PATHOLOGY & CAUSES
• Rare blood vessel malignancy involving
blood vessel endothelial lining
• Aggressive, rapidly proliferating - poor
prognosis
• Can occur anywhere; usually occurs in sun-
exposed areas (head, neck, breast)
° Cutaneous angiosarcomas (occur
beneath skin's surface) most common
• Can affect liver blood vessels
CAUSES
) • Most likely due to lymphedema (fluid
buildup causing sweeling), radiation
exposure, carcinogens
RISI( FACTORS
• Biologically male (twice as likely), elderly,
sun-exposure, radiation therapy, chronic
post-mastectomy lymphedema, frequent
exposure to vinyl chloride monomer gas in
PVC manufacturing/arsenic insecticides
• High-grade: aggressive, fast-growing

157
 O Low-grade: less aggressive, slow-
growing (..____ D_IA_GN_O_s,_s
__ )
LAB RESULTS
COMPLICATIONS • Biopsy, usually diagnosed late after the
• High chance of metastasis, poor prognosis. disease has spread
Better prognosis for individuals with
smaller tumors with clearly delineated
margins. Low grade breast angiosarcoma (..__ __ T_R_EA_:t"_M_EN_T
__ )
has better prognosis than tumors with
poorly-defined borders MEDICATIONS
• Chemotherapy

( s,_G_NS_&_SY_M_PT_O_M_s_)
SURGERY
• Lesion resembling non-healing bruise/ • Difficult to resect due to delay in diagnosis
wound
• Violet color OTHER INTERVENTIONS
• Soft, visible, tactile lump/swelling • Radiation
• Can form irregular vascular channels that
disrupt tissue planes
• Fatigue
• Bone pain
• Anemia

Figure 24.1 A surgically excised

angiosarcoma.

Figure 24.2 Histological appearance of

an angiosarcoma composed of malignant
endothelial cells with vascular spaces
containing red blood cells.

158
 GLOMUS TUMOR
osmsJl/ glomus--lumoY-

( PATHOLOGY & CAUSES ) ( T_R_EA_:l'__

_M_EN_T
)
• Benign tumor arising from modified smooth SURGERY
muscle cells of skin's thermoregulatory • Resection
glomus bodies
• Derives from small vessels/arteriovenous
anastomoses in glomus bodies
• Malignancy, metastasis rare
• Etiology includes loss-of-function
mutation of protein glomulin in familial
glomangiomas

RISI( FACTORS
• Adults: 20-40 years old
• Most lesions solitary, localized
• Autosomal dominant inheritance pattern

COMPLICATIONS
• Good prognosis, low recurrence rate after
resection
• Malignant glomus tumors rare, have good
prognosis when treated with wide excision Figure 24.3 Homogenous enhancement of
• Metastasis associated with poor prognosis a glomus tumor of the nail bed at the ulnar
aspect of the left index finger.

(__ SI_G_NS_&_S_Y_M_PT_O_M_s_)
• Painful, small, red-blue growths
O Pain associated with solitary lesions
O Younger individuals: multiple tumors,
usually asymptomatic
• Usually found on distal extremities
• Paroxysmal pain depending on
temperature, pressure changes
° Cold, pressure worsens pain

Figure 24.4 Histological appearance of a

( D_IA_GN_O_s,_s
__ ) glomus tumor.

OTHER DIAGNOSTICS
• Visual inspection
• Occasional imaging

159
 afratafreeh.com exclusive

l(APOSI SARCOMA
osmsJl/ko.posi
STAGING
( PATHOLOGY & CAUSES )
AIDS-related Kaposi's sarcoma
• Malignant vascular tumor/lesions of blood • Takes three factors into account
vessel endothelial cells • Extent/severity of tumor
• Due to human herpesvirus 8 , TO: localized tumor
• Virus penetrates cells, causing , Tl: widespread. multiple lesions that
uncontrollable replication spread to other organs
• May involve visceral organs • CD4 cell count (immune status)
• Progression. severity of tumor depends on , 10: CD4 count above 150 cells/mm3
underlying factor
, 11: CD4 count less than 150 cells/mm3
o Genetic: usually seen in older Eastern
• Presence/absence of systemic illness
European males; tumor localized to skin
, SO: no systemic illness/opportunistic
o AIDs: tumor spreads (see staging
infections. and/or B symptoms. B
below)
symptoms: systemic fever symptoms,
o Organ transplant recipients: tumor
night sweats, weight loss, diarrhea
spreads
, Sl: presence of systemic illness,
opportunistic infections, and/or B
TYPES symptoms

AIDS-related
• Most common malignancy in AIDS COMPLICATIONS
• Lymphedema
lmmunocompromised& iatrogenic-related • Bleeding
Classic/sporadic • Infection
• Long term hyperpigmentation
Endemic (African) • Prognosis depends on individual's immune
• Burkitt's lymphoma due to Epstein-Barr status, viral load (amount of HIV virus in
virus blood)

RISI( FACTORS
• lmmunocompromised individuals
( SIGNS & SYMPTOMS )
oAIDS
• Most common symptoms affect skin. also
° Kaposi's sarcoma associated human affect mouth. GI tract, respiratory tract
herpesvirus-8 (HHV-8)
n Progresses from flat lesion ----.
o Organ transplant plaque----. ulcerating nodule
• Biologically male n Purple. red lesion similar to bruise that
• Eastern European does not blanch
• Higher risk: biologically-male individuals O Lesion starts off flat. may become
engaging in same-sex sexual acts ("MSM") raised. more painful

160
 O Lesions in other tissues (e.g. mouth, MEDICATIONS
nose, throat, lymph nodes, lungs, • HIV/AIDS management with antivirals
gastrointestinal tract); Commonly found = Control HIV/AIDS - lesions shrink
in mucous membranes (esp. hard palate)
• Removal of drugs (e.g. corticosteroids)
• Pulmonary symptoms: pulmonary Kaposi's allows immune system to recover
sarcoma
= Treatment more difficult in
° Coughing (possibly bloody cough) immunocompromised individuals
o Dyspnea • Chemotherapy

( D_IA_GN_O_s,_s __ ) SURGERY
• Surgically remove affected skin
DIAGNOSTIC IMAGING
• Bronchoscopy/endoscopy OTHER INTERVENTIONS
• Crvotherapv-« freeze affected skin
LAB RESULTS • Radiation
• Biopsy

(...____ T_R_E~_~_M_EN_T__ )
• Depends on
O Severity of immunosuppression
O Number, location of tumors
o Symptoms

Figure 24.5 Kaposi sarcoma of the gingiva

in a HIV positive individual. The tumor has
replaced the gingiva of the upper right side of
the jaw. There is overlying oral candidiasis.

Figure 24.6 A Kaposi sarcoma composed

of spindle cells which form slits filled with
erythrocytes.

161
NOTES

GENERALLY.WHAT IS IT?
( PATHOLOGY & CAUSES ) ( D_IA_GN_o_s,_s )
• Inflammation of blood vessels LAB RESULTS
• Vasculitides categorized by blood vessel • C-reactive protein (CRP), erythrocyte
size: small, medium. large sedimentation rate (ESR). complete blood
count (CBC). various autoantibodies
• Biopsy vessel segment
CAUSES
Damaged endothelium
• Damaged endothelium - exposed ( T_R_EA_:l"_M_EN_T )
collagen, tissue - increased blood
coagulation - weakened, damaged blood MEDICATIONS
vessel walls - aneurysms - vessel wall
Reduce inflammatory response
heals, stiffens as fibrin deposits
• Corticosteroids/immunosuppressive drugs
Autoimmune disease
• Direct method: body mistakes endothelial
layer of blood vessel for foreign pathogen
- attacks
O Molecular mimicry: immune system
white blood cells (WBCs) mistake
normal antigens of endothelial cells for
foreign invaders (e.g. bacteria)
O Medium, large-vessel vasculitides
• Indirect method: immune system attacks
healthy cells near vascular endothelium -
damages endothelial cells
O Small-vessel vasculitides (exception:
Henoch-Schonlein purpura)

(__ s,_G_NS_&_SY_M_PT_O_M_s )
• Inflammatory response symptoms: fever,
weight loss. malaise, fatigue
• lschemia
O Blood cells clump to exposed collagen
inside blood vessels - blood clots -
restricted blood flow
° Fibrin deposits in vessel wall - wall
thickens. bulges into vessel - stenosis
- restricted blood flow

162
 carotid artery jaw pains
Large
Arteries from aortic
Weak pulse. neurological symptoms
arch (branch points)

Coronary arteries 4 of 5 CRASH symptoms,

serving heart prolonged high fever

Commonly affects skin. "String of beads" appearance on

can affect almost angiogram, end organ
Medium
any orqan ischemic damage

Blood clots in small

arteries of Ulcers. dead tissues in extremities
fingers. toes

Chronic pain, saddle nose deformity,

Nasopharynx, lungs, breathing difficulties, ulcers with
kidneys bloody coughing, decreased urine
production. increased blood pressure

Vessels of kidneys. Kidney inflammation, weight loss.

Small lungs skin lesions, fever, nerve damage

Vessels of kidneys, Sinusitis, lung damage,

lungs kidney damage

Vessels of kidneys, Symptoms depend on where

lungs lgA attacks small blood vessels

Recurrent oral ulcers,

genita I ulcers, skin papules,
All vessels All vessels decreased vision, headaches,
fever, disorientation, stroke,

Swollen joints affecting knees.

wrists, ankles

163
 BEHCET'S DISEASE
osms.i"l/\>ehee-ls-disee1se

( PATHOLOGY & CAUSES ) ( T_R_E~_~_M_EN_T

)
• Autoimmune multisystem vasculitis MEDICATIONS
affecting any sized vessel, arterial/venous • Skin creams, mouth rinses, eye drops
• Corticosteroids: (e.g. prednisone) control
RISI( FACTORS inflammation
• Individuals who are 20-30 years old, of • Medications: (e.g. azathioprine,
Middle Eastern/Asian descent, biologically cyclosporine, or cyclophosphamide)
male suppress immune system
• Medications: (e.g. interferon alfa-2b) alter
immune system response
COMPLICATIONS
• Blindness from untreated uveitis
(inflammation in eyes)

(_~S_IG_NS_&~SY_M_P_TO_M_S
)
• Recurrent. painful, sterile oral/genital ulcers
(pathergy)
• Skin pa pules indistinguishable from acne
• Uveitis, optic neuritis, conjunctivitis iritis
• Neurologic involvement
(meningoencephalitis, intracranial HTN,
stroke, headache)
• Arthritis (knees, ankles)
• Fever. weight loss

( D_IA_GN_o_s,_s )
OTHER DIAGNOSTICS
Clinical presentation
• Recurrent oral ulcers {three in one year) + Figure 25.1 Mucosa I ulcer in an individual
two of following with Behest's disease.
• Recurrent genital ulcers
• Eye lesions. uveitis
• Skin lesions
• Positive pathergy test
• ~ 2mm papule 24-48 hours after oblique
insertion 5mm into skin with 20-gauge
needle. often performed on forearm

164
 BUERGER'S DISEASE
osms.i-l/\,ueTgeTs
( PATHOLOGY & CAUSES )
• Nonatherosclerotic, segmental,
inflammatory disease affecting small-,
medium-sized veins, arteries of extremities
- inflammatory occlusive thrombus -
distal extremity ischemia, digit ulcers/
gangrene - autoamputation
• AKA thromboangiitis obliterans
• Associated with use of tobacco products
RISI( FACTORS
LAB RESULTS
Biopsy
• Definitive; rarely (healing a concern)
• Histopathologically, acute-phase lesions
show highly cellular, inflammatory
thrombus with minimal inflammation of
blood vessel
( T_R_EA_:T_M_EN_T
__ )
• Immediate smoking cessation

• Individuals < 45 years old, who are

biologically male, use tobacco
• Chronic anaerobic periodontal infection (%
of people with Buerger disease)

(__ SI_G_NS_&_SY_M_PT_O_M_s
)
• Ulceration of digits
• lschemic claudication: cold, painful,
cyanotic distal extremities
• Subcutaneous nodules, superficial
thrombophlebitis
• Paresthesias of extremities
• Raynaud phenomenon

Figure 25.2 A volume rendered CT

angiogram demonstrating obliteration
( D_IA_GN_O_SI_S
__ ) of the right femoral artery secondary to
thromboangiitis obliterans. There is also
DIAGNOSTIC IMAGING stenosis of the femoral artery on the left.
Angiogram
• Lack of atherosclerosis
• Co/lateralization, segments of diseased
vessel interspersed: smoking -
atherosclerosis + Buerger disease
simultaneously

165
 CHURG-STRAUSSSYNDROME
osms.i"l/churg-s-lro.uss-s14nd Tome

( PATHOLOGY & CAUSES ) ( D_IA_GN_o_s,_s __ )

• Small, medium vessel granulomatous DIAGNOSTIC IMAGING
vasculitis involving many organ systems
(cardiac, gastrointestinal, respiratory, Chest X-ray
skin, renal, neurologic) in individuals with • Transient, patchy, symmetrical opacities,
allergy-related respiratory conditions (esp. often in hilar/peripheral distribution
asthma) • Pulmonary hemorrhage
• AKA eosinophilic granulomatosis with • Bilateral nodular disease without cavitation
polyangiitis (EGPA). allergic granulomatosis
High-resolution CT scan
• P-ANCA reacting with neutrophilic
myeloperoxidase • Peribronchial, septal thickening
• Etiology unknown • Widely scattered indistinct opacities

Pulmonary function test

RISI( FACTORS • Obstructive pattern consistent with asthma
• Age 30-50; asthma/nasal issues
Bronchoalveolar lavage
• High % of eosinophils
(__ s,_G_NS_&_S_Y_M_PT_O_M_s_)
LAB RESULTS
• Allergies
• Eosinophilia > 1500/microL. > 10% on
O Asthma, chronic rhinosinusitis, usually differential leukocyte count
precedes vasculitic phase by 8-10 years
• P-ANCA/MPO-ANCA antibodies
• Neurological
• Acute phase reactants: j ESR. CRP
O Peripheral neuropathy (usually
mononeuritis multiplex) Lung/skin biopsy
O Subarachnoid, cerebral hemorrhage, • Definitive
cerebral infarction, cranial nerve palsies
• Skin
O Palpable purpura, subcutaneous ( T_R_E~_~_M_EN_T__ )
nodules
• Prognosis poor (five year survival, 25%
• Cardiac
without treatment; 50% with treatment)
O Damage - heart failure, arrhythmias
O Accounts 112 deaths
• Other organ systems (renal,
MEDICATIONS
gastrointestinal) - symptoms similar to • Corticosteroids, immunosuppressive drugs
medium-vessel vasculitides

166
Figure 25.3 Histological appearance of
vasculitis in Churg-Strauss syndrome. The
background is composed almost entirely of
eosinophils.

GIANT CELL ARTERITIS

osms.t-1:/gio.n-1:-eell-o.T-leTi-lis

( PATHOLOGY & CAUSES ) (__ s,_G_NS_&_S_Y_M_PT_O_M_s_)

• Chronic vasculitis of large-, medium-sized • New-onset headache (most common):
vessels temporal branch of carotid artery
• AKA temporal arteritis • Jaw claudication (pain when chewing)
• Cranial branches of arteries originating • Transient unilateral vision loss (amaurosis
from aortic arch fugax): ophthalmic artery
O Temporal branch of carotid artery • Carotid bruits, decreased pulses in arms,
• Aorta, carotids also affected aortic regurgitation
• Most common systemic vasculitis • Tender, palpable nodules, absent temporal
pulse
• Increased risk of aortic dissection, aortic
CAUSES aneurysm
• Unknown: possibly genetic, environmental,
autoimmune-related
MNEMONIC: TEMPORAL
RISI( FACTORS Characteristics of Temporal
• Almost always in individuals ;;:: 50 (Giant cell) arteritis
• More common in individuals who are Temporal artery tenderness
biologically female ESR >100
• Strong association with polymyalgia Multinucleated giant cells
rheumatica (40-50% of GCA individuals) Pain
Onset >50 years old
COMPLICATIONS polymyalgia Rheumatica
• Irreversible blindness (if untreated) association
Amaurosis fugax
Lost vision

167
( D_IA_GN_O_SI_S __ )
LAB RESULTS
• Extremely elevated ESR (> lOOmm/hr), j
IL-6 associated with active disease

Temporal artery biopsy

• Tightly packed monocytes/macrophages,
as if one giant cell, in internal elastic lamina;
segmental pattern; 90% sensitivity

Figure 25.4 A histology photomicrograph

( T_R_EA_:T_M_EN_T
) demonstrating giant cell arteritis. The
external elastic lamina to the right has been
• Corticosteroids
completely destroyed by granulomatous
inflammation.

GRANULOMATOSIS WITH
POLYANGIITIS
osmsJl/ gl90.nulomo.-losis-wtlh-pol140.ngU-lis
\ MNEMONIC: 3Cs
( PATHOLOGY & CAUSES ) "C" drawn from upper
respiratory tract to lungs,
• Small-vessel vasculitis involving
kidneys (all involved)
nasopharynx, lungs, kidneys
C-anca
• AKA Wegener's granulomatosis
Corticosteroids/
• Granulomatous disease of respiratory tract
cyclophosphamide
- systemic necrotizing vasculitis (treatment)
• 8-cells release cytoplasmic antineutrophil
cytoplasmic antibodies (c-ANCA) -
binds to proteinase 3 (neutrophil granule)
in neutrophils - neutrophils release (__ SI_G_NS_&_S_Y_M_PT_O_M_
free radicals - free radicals damage
neighboring endothelial cells - vasculitis • Chronic pain: oral ulcers, bloody nasal
• Triad mucus, chronic sinusitis, saddle nose (nose
° Focal, necrotizing vasculitis caves in/curls)
O Necrotizing granulomas in upper airway, • Hemoptysis, dyspnea, cough, pleuritic
lungs chest pain (inflammation of lung vessels)
O Necrotizing glomerulonephritis (renal • Decreased urine production, hypertension,
vasculitis) hematuria, red cell casts, proteinuria
(glomerular inflammation)

RISI( FACTORS
• Middle aged individuals who are
biologically male
168
( D_IA_GN_O_s,_s) ( T_R_E~_~_M_EN_T
)
DIAGNOSTIC IMAGING • Relapse common if c-ANCA still present

Abnormal chest X-ray

• Nodules, fixed infiltrates, cavities, bronchial
MEDICATIONS
stenosis • Corticosteroids, cyclophosphamide/
rituximab

LAB RESULTS
• c-ANCA in 90%, thrombocytopenia
• Abnormal urinary sediment; microscopic
hematuria (with/without red cell casts)

Open lung biopsy

• Confirm diagnosis; granulomatous
inflammation of artery/perivascular area

OTHER DIAGNOSTICS
Nasal/oral inflammation
• Oral ulcers; painful/painless
• Purulent bloody nasal discharge
• Chronic sinusitis, saddle nose/destructive
sinonasal disease

VASC.\JL\1\ ~

•cor-Hu,'Istt,o idi,
.... c:,-dof'•'c,~p'roo.,v,\J.c.

\
~t.\Ar,t. CO'IW\MOV\

• c.-At.JCA S~I\ jXt\<.11\

Figure 25.5 Illustration demonstrating the effects of granulomatosis with polyangiitis.

169
 HENOCH-SCHONLEIN PURPURA
osmsJl/henoeh-sehonlein- UT ure

( PATHOLOGY & CAUSES ) ( T_R_E~_~_M_EN_T

)
• Small vessel vasculitis secondary to lgA • Self-resolving, may reoccur
immune complex deposition.
• Elevated lgA in blood targets self- MEDICATIONS
endothelial cells: molecular mimicry
• Steroids, only if severe
• Most common systemic vasculitis of
childhood
• Frequently follows upper respiratory
infection
• Associated with Berger disease (lgA
nephropathy)
• Unknown cause; immune-mediated
vasculitis triggered by infections/
immunizations
• Self-limited disease
• Tetrad
O Palpable purpura, without coagulopathy/
thrombocytopenia; mainly lower
extremities
O Arthritis/arthralgias
O Renal disease
O Abdominal pain

(..___s,_G_Ns_&_sv_M_PT_O_M_s
)
• Palpable purpura of buttocks. legs (skin
discolouration, as if blood collected under
skin surface); abdominal pain; arthritis/ Figure 25.6 The clinical appearance of
arthralgias; hematuria, decreased kidney Henoch-Schonlein purpura.
function (associated with lgA nephropathy)

( D_IA_GN_O_s,_s
__ )
LAB RESULTS
Biopsy
• Definitive. not necessary

170
 l(AWASAl(I DISEASE
osms.i"l/lco.wo.so.lci-diseo.se

( PATHOLOGY & CAUSES ) ( D_IA_GN_o_s,_s)

• Coronary arteries: inflammation - DIAGNOSTIC IMAGING
aneurysms
Chest X-ray
• AKA mucocutaneous lymph node
syndrome • Cardiomegaly
• Most common type of vasculitis in children Echocardiograp
hy
• Usually self-limited • Coronary artery aneurysms, pericardia!
effusions, decreased contractility
RISI( FACTORS
• Infants, children< five years old, Asian LAB RESULTS
descent, biologically male • t CRP, ESR, platelet count (reactive
throm bocytosis)
COMPLICATIONS
• Coronary artery aneurysm OTHER DIAGNOSTICS
• Decreased myocardial contractility - heart • Four of five CRASH symptoms, high fever
failure lasting five days
• Myocardial infarction (Ml)
ECG
• Arrhythmias
• Arrhythmias, abnormal Q waves, prolonged
• Peripheral artery occlusion
PR, QT intervals

( SI_G_NS_&_S_Y_M_PT_O_M_s
)
MNEMONIC: CRASH &
SVRN
Signs & Symptoms
Conjunctivitis: bilateral,
nonexudative
Polymorphous Rash:
desquamating
Cervical lymphAdenopathy
Strawberry tongue: cracked
red lips, oral mucositis
Hand-foot erythema/
desquamation: edema,
erythema
Fever: "burn"
Figure 25.7 A coronary angiogram
demonstrating a massive right coronary
artery aneurysm.

171
(..__ __ T_R_EA_~_M_EN_T
)
MEDICATIONS
• Intravenous immunoglobulin (IVIG)
• Aspirin

MICROSCOPIC POLYANGIITIS
osms.i"l/ mieroseopie-pol140.ngii"lis

( PATHOLOGY & CAUSES ) ( D_IA_GN_O_SI_S

)
• Necrotizing vasculitis: kidney, lung vessels LAS RESULTS
• No granulomas present • p-ANCA/MPO-ANCA levels; elevated ESR,
• Associated with perinuclear anti-neutrophil CRP, anemia, increased creatinine
cytoplasmic antibodies (p-ANCA)/MPO- • Protein, red blood cells (RBCs)
ANCA
• Pauci-immune glomerulonephritis (minimal
immunofluorescent staining for lgG) C T_R_EA_~_M_EN_T
)
• Older adults
• Relapse common

( SI_G_NS_&_SY_M_PT_O_M_s
) MEDICATIONS
• Corticosteroids, cyclophosphamide
• Similar to granulomatosis with polyangiitis,
without nasopharyngeal involvement
• Fever, weight loss, fatigue, myalgia,
arthralgias
• Cough, dyspnea, hemoptysis, pleuritic
chest pain
• Decreased urine output, hematuria, red cell
casts, proteinuria
• Skin lesions (especially lower extremities):
purpura - focal necrosis - ulceration

172
 POLYARTERITISNODOSA
osms.i"l/ pol14e1T-leTi-lis-nodose1

( PATHOLOGY & CAUSES ) ( D_IA_GN_O_SI_S )

• Immune system forms antibody antigen DIAGNOSTIC IMAGING
complex (sometimes associated with
hepatitis BJ ----> deposits in vessel Mesenteric angiogram
wall-» immune reaction-e- invasion • "String of beads" pattern along artery,
of polymorphonuclear leukocytes----> spasms
segmental, transmural inflammation of
muscular arteries ----> necrosis of three artery LAB RESULTS
layers (tunica intima, media, adventitia) ---->
• HBV, HCV serologies, Cr, muscle enzymes,
fibrosis as walls heal (fibrinoid necrosis)
urinalysis
----> fibrosed vessel wall weakens, prone
to aneurysms ----> fibrotic aneurysms (hard Biopsy
bulges) develop
• Different stages of inflammation in different
vessels OTHER DIAGNOSTICS
Physical exam
RISI( FACTORS • Vascular lesions, motor weakness {due to
• Individuals > 40 years old, biologically male ischemia)
• Active hepatitis B (HBV)/hepatitis C (HCV)
infection
• HIV
( T_R_E~_~_M_EN_T
)
• Prescription/illicit drug exposure,
MEDICATIONS
amphetamines
• Corticosteroids
• Cyclophosphamide: supplement
(__ SI_G_NS_&_SY_M_PT_O_M_s
) corticosteroids in moderate to severe cases

• Systemic: fever, fatigue, weight loss,

arthralgia
• End organ ischemic damage
• Renal arteries: HTN
• Mesenteric artery: mesenteric ischernia,
severe abdominal pain, gastrointestinal
bleeding
• Mononeuropathy multiplex: motor, sensory
deficits occur in > one nerve throughout
body
• Skin arteries: skin lesions (e.g. ulcers,
erythematous nodules resembling
erythema nodosum, purpura, livedo
reticularis)

173
 'n'4rertt.t\ ,, Oii\.
,J L, ¥.tc.lllt:jS lOn-\ \o\ood.
"Stn~5 of 'oco4:i" vo\u.,yQ.

Oil

I\M6104Jll'M
~~°"'~" ll\ F."''o\ttd.lWl
~o.~-tro\,t\\e.~,l~o\
~ j
&roiil M +e rit ~ • \ ,
~------ viwro\oj"" S;jMp\-On,.,
\fl,{.ATft'IE.UT: (Olt,Tlc..O~TC~ll>S

Figure 25.8 Illustration showing polyarteritis nodosa's characteristic "string of beads" pattern
running along the artery.

TAl(AYASU ARTERITIS
osms.i"l/-le1ke114e1sus-e1T-leTi-lis

( PATHOLOGY & CAUSES ) (..___s,_G_Ns_&_sv_M_PT_O_M_

)
• Segmental, patchy granulomatous • Inflammation
vasculitis of aortic arch, major branches , Aortic branches, upper extremities:
• Stenosis, thrombosis, aneurysm weak/absent pulse
, Aortic branch, head: neurological
CAUSES symptoms (e.g. headaches, syncope,
stroke)
• Unknown; possibly bacterial (e.g.
spirochetes, Mycobacterium tuberculosis, , Coronary arteries: angina
streptococcal) , Renal arteries: HTN
• Genetic • Visual disturbances: ocular vessels/retinal
hemorrhage
• Constitutional symptoms: fever, night
RISI( FACTORS sweats, arthralgias, malaise, fatigue
• Individuals of Asian descent, < 40 years
• lschemia in areas of stenosis
old, biologicallyfemale

COMPLICATIONS ( D_IA_GN_O_s,_s)
• Limb ischemia; aortic aneurysm;
aortic regurgitation; stroke; secondary DIAGNOSTIC IMAGING
hypertension (HTN) due to renal artery
CT angiography (CTA), magnetic resonance
stenosis
angiography (MRA)
• Luminal narrowing/occlusion of major aortic
branches
• Vessel wall thickening
• Aortic valve disease (e.g. regurgitation,
174
 stenosis)
• Aneurysmal dilation/pseudoaneurysm
formation

Ultrasound
• Homogeneous and circumferential
thickening of arterial wall (contrast to
atherosclerotic plaque: non-homogeneous,
calcified, irregular walls)
• Vascular occlusion due to intimal
thickening/secondary thrombus formation
• Loss of pulsatility of vessel

LAB RESULTS
• i ESR

( T_R_EA_:T_M_EN_T__ )
MEDICATIONS
• Corticosteroids
• Treat HTN
Figure 25.9 An angiogram demonstrating
multiple stenosis of the aortic arch vessels, a
SURGERY feature of Takayasu arteritis.
• Angioplasty {when no acute inflammation);
bypass grafting if severe

175
NOTES

- NOTES
•
-
VENOUS DYSFUNCTION
GENERALLY.WHAT IS IT?
• Localized hyperpigmentation/skin
( PATHOLOGY & CAUSES ) discoloration
• Hard, cord-like veins/prominent dilated
• Venous system defects affecting blood flow
tortuous veins
from lower extremities

CAUSES ( D_IA_GN_O_s,_s
__ )
• Blood clot partially/completely blocking
way/venous valves failing to pump blood DIAGNOSTIC IMAGING
against gravity
Doppler ultrasound
Virchow's triad • Assess vein diameter, thrombi, valve status,
• Hypercoagulability, increased clot formation blood flow (anterograde vs. retrograde)

° Factor V Leiden thrombophilia Venography

O Protein C and protein C deficiencies • X-ray, contrast medium injected into vein
• Venous stasis from prolonged • Assess status of vein network, detect
immobilization (e.g. bed rest) thrombi
• Damage to endothelial lining
LAB RESULTS
RISI( FACTORS • D-Dimer: High sensitivity (-100%) and
• Prolonged immobility, hereditary clotting negative predictive value (-100%) for
dysfunctions, high estrogen levels, obesity detection of venous thromboembolism
• One venous dysfunction can lead to
another
( T_R_E~_~_M_EN_T
__ )
\ I MNEMONIC: PHO MEDICATIONS
Virchow's Triad

i
• Acute manifestation: unfractionated
Prolonged immobilization heparin/low-molecular-weight heparins
(stasis) • Long-term management: oral
Hypercoagulability anticoagulants (e.g. warfarin)
Damage to endothelium • Prior DVT
, Long term anticoagulation therapy,
antiplatelet treatment, parenteral
anticoagulants
( s,_G_NS_&_SY_M_PT_O_M_s_)
• Localized pain, usually lower extremities
SURGERY
• Vein transplant/repair/removal
• Edema
• Pruritus

176
OTHER INTERVENTIONS
• Preventative: calf exercises, compression
stockings/devices, raise affected areas to
decrease swelling

CHRONIC VENOUS INSUFFICIENCY

(CVI)
osms.i"l/eh,-onie-venous-insuffieiene14
(hemosiderin deposits)
( PATHOLOGY & CAUSES ) • Pruritus, stasis dermatitis
• Painless, wet ulcers, particularly on medial
• Veins cannot push blood back to heart.
malleolus
resulting in blood pooling in leg
• Edema
• Atrophie blanche: hypopigmented atrophic
CAUSES areas with telangiectasia (clusters of red/
• Develops from varicosities, DVT. phlebitis purple capillaries). red dots
O Varicose veins affect superficial veins,
but blood sometimes rerouted to
collateral veins deep in leg, preventing ( D_IA_G_N_os_,s )
blood stagnation
• When deep veins carry more blood than DIAGNOSTIC IMAGING
normal
O Deep veins
Doppler ultrasound imaging
stretch over time, blood
pools • Most common diagnostic
O Blood flow stagnation in lower • Modified vein diameter (increased = acute
extremities causes inflammatory thrombus, decreased = chronic thrombus)
reaction in vessels, tissue, causing • Absent color flow: vein completely
fibrosis, venous stasis ulcers occluded
• Increased flow in surrounding superficial
veins
RISI( FACTORS
• Biological females, inactive standing/sitting Venography
for long periods, aging, family history, • Most effective. but invasive and cost-
ligamentous laxity, obesity, smoking, low- prohibitive
extremity trauma, prior venous thrombosis,
arteriovenous shunt, pregnancy
( T_R_EA_:l"_M_EN_T
__ )
(__ SI_G_NS_&_S_Y_M_PT_O_M_s_)
SURGERY
• Vein transplant/repair/removal
• Calf/ankle pain (most common symptom)
• Worse with prolonged standing/sitting,
improves with leg elevation. movement OTHER INTERVENTIONS
• Brown hyperpigmentation of skin • Preventative: calf exercises, compression
stockings/devices, raise affected areas to
decrease swelling

177
Figure 26.1 The clinical appearance of mild
CVI. Hemosiderin deposition is clearly visible.

900L1~~ OEE:P VE.HJ

l C.ou..A"n:11.AL}
i
'ItJFLAl't'\MJ\TlOtJ
\·h'PlilPl<.:a~UJT~l,otJ
p~url.1ns (lic.tt1t-..1E..ss) + P"'""
VLC.fi S
VClflOVS 5tO.W;,)
( utcer s

Figure 26.2 Illustration of varicose veins that have developed into a case of CVI.

178
 DEEP VEIN THROMBOSIS (DVT)
osms.i"l/ deep-vein--lhTom \>osis

( PATHOLOGY & CAUSES ) (__ SI_G_NS_&_S_Y_M_PT_O_M_s

)
• Blood clotting in deep leg veins (iliofemoral, • 50% asymptomatic due to venous collateral
popliteal, femoral veins) channels
• Arterial clots usually due to artery wall • Localized inflammation around clot
damage; venous clots don't require vein • High venous pressure engorges visible
damage superficial veins
• Valves inside veins can lower blood • If PE occurs: sudden dyspnea, chest pain
oxygen levels ----> venous stasis-associated , Fatal if enough lung tissue affected
hypoxemia can activate reactive oxygen
species, other hypoxia-inducible factors ---->
tissue factor released into blood
( D_IA_GN_o_s,_s )
O Tissue factor activation ----> prothrombin
turns into thrombin ----> fibrin fibers form
DIAGNOSTIC IMAGING
net-» traps red blood cells, white blood
cells, platelets ----> venous thrombus Doppler ultrasound imaging
• Most common diagnostic
CAUSES • Modified vein diameter
• Virchow's triad , Increased: acute thrombus
• Antiphospholipid syndrome , Decreased: chronic thrombus
• Prolonged immobilization (bed rest, • Absent colour flow: vein completely
orthopedic casts, long-distance air travel) occluded
• Genetic • Increased flow in surrounding superficial
O Antithrombin,
veins
protein C, S deficiencies
Venography
RISI( FACTORS • Most effective, but invasive/cost-prohibitive
• Pregnancy, oral contraceptives, old age,
major surgery (e.g.orthopedic surgery), LAB RESULTS
malignancy, obesity, trauma, heart failure
• 0-dimers ----> rule out DVT
, Increased level: plasmin dissolves
COMPLICATIONS thrombus
• Pulmonary embolism (PE) most common
° Can cause pulmonary infarction, death OTHER DIAGNOSTICS
• Post-thrombotic syndrome
O Develops in 50% of individuals with Wells' score
DVT • Higher score indicates increased chance of
• Extreme cases: phlegmasia cerulea dolens DVT (Scale of -2 to 9 points)
(blue, painful, swollen leg, possible venous , High score = high chance: > 2 points
gangrene) , Moderate score = moderate chance:
1-2 points
, Low score = low chance: < 1 point

179
 afratafreeh.com exclusive

C..____ T_R_EA_~_M_EN_T
)
MEDICATIONS
• Acute manifestation: unfractionated
heparin/low-molecular-weight heparins
• Long-term management: oral
anticoagulants (e.g. warfarin)
• Prior DVT: long term anticoagulation
therapy, antiplatelet treatment, parenteral
anticoagulants

OTHER INTERVENTIONS
• Preventative: calf exercises, compression
stockings/devices, raise affected areas to
decrease swelling

IVC. FILTER

Figure 26.4 Clinical appearance of a deep

Figure 26.3 An IVC filter, used to prevent vein thrombosis of the right leg. The lower
embolization of the deep vein thrombus into leg is erythematous and swollen.
the pulmonary vasculature.

180
 THROMBOPHLEBITIS
osms.i-1:/-lhTombo hlebi-lis
COMPLIC ATIONS
( PATHOLOGY & CAUSES ) • DVT, superficial thrombophlebitis,
pulmonary embolism
• Vein inflammation caused by clot in deep
leg veins
• Increased coagulability (Virchow's triad) (__ s,_G_NS_&_S_Y_M_PT_O_M_s_)
• Potential locations
O Upper limbs (usually at site of IV • Pain, inflammation/swelling, hard, cord-like
cannula) veins
O Lower limbs (coupled with varicose • Sometimes asymptomatic, can be revealed
veins) by applying pressure
O Periprostatic venous plexus in biological , Hoffman's sign (forced dorsiflexion on
males foot creates soreness behind knee); not
O Pelvic venous plexus in biological 100% accurate
females
O Large veins of cranium, dural sinuses
o Portal vein (..____ D_IA_GN_o_s,_s __ )
DIAGNOSITC IMAGING
TYPES
Venous duplex ultrasound
Migrating thrombophlebitis
• Thrombosed veins thickened, poorly
• Occurs in several different locations, usually compressible
in pancreatic carcinomas due to pro-
• Completely occluded vein= hypoechoic
clotting factors secreted by tu moral cells
{low level echoes)
Superficial thrombophlebitis • No internal flow present distal to clot
• Thrombus develops in vein near skin's
Imaging studies
surface
• Thrombus detection (e.g. CT venography
O Mondor's syndrome: thrombophlebitis
(CTV) with contrast, magnetic resonance
of subcutaneous veins of breast/arm I (MR) venography)
penis; presents as lump
• Blood coagulation tests (e.g. elevated
Suppurative (septic) thrombophlebitis 0-dimers)
• Infection from IV cannula; possible
purulence LAB RESULTS
Blood coagulation tests
CAUSES
• Elevated 0-dimers
• Most commonly: needle/catheter
• Prolonged immobilization: bed rest,
orthopedic casts, long-distance air travel OTHER DIAGNOSTICS
• High estrogen: pregnancy, estrogen • Inspection of affected area
replacement therapy, oral contraceptives , Pulse (weak/absent)
• Hereditary clotting disorders: protein D/C , Blood pressure (high)
deficiencies/factor V Leiden mutations , Temperature {high)
• Vasculitis, Behcet's disease

181
 OTHER INTERVENTIONS
(..____ T_R_EA_:T_M_EN_T
) • Preventative: calf exercises, compression
stockings/devices, raise affected areas to
MEDICATIONS decrease swelling
• Acute manifestation: unfractionated
heparin/low-molecular-weight heparins
• Long-term management: oral
anticoagulants

b b11'\(1l 6<.oob tE!)'f

l
fiifoJ 8il(AIC~wtJ
Pllobu(1'5
ere l·h~Hwke.n
6LOOC> Cu,1' ii\ -*"'ere .s e- Cc.a'T
CoMMo~ fif"\o~AI. VE•tJ

Figure 26.5 Illustration showing blood clots discovered via imaging studies.

'It LotJu -TfflM ,..tt(A1"MEtJ1' ( Pf2£\IE.N11ol'J )

4 A~1,0AC.UL£,J-r M'-b1C.A1'10tJ~
· wAer,u211J
· '-tE.PA'1\~

,}( ht-i£~ i(I H,e

hJr(l11otz V£NA
CAVA
+e Pe£\1£Al1
Puv1otJAfh'
€1"180'-' ~MS

Figure 26.6 Illustration showing a surgically-implanted filter in the inferior vena cava preventing
a pulmonary embolism.

182
 VARICOSE VEINS
osms.i"l/vertcese-vetns
( PATHOLOGY & CAUSES
• Enlarged, twisted superficial veins (most
commonly in leg)
• Downward gravitational pull causes walls
of veins to stretch over time, blood leaks
backwards ----> extra blood volume twists
veins ----> veins become varicose
• Blood sometimes rerouted to collateral
veins deep in leg
TYPES
Varicocele
• Abnormal enlargement of pampiniform
venous plexus in scrotum
• Mechanism same as varicose veins
• Most common in left testicle
O Left testicular
vein brings blood to
left renal vein at 90° angle----> difficult
----> blood backs up ----> vein becomes
varicose-e- loops back and forth on itself
0 "Bag of worms" appearance
CAUSES
• Obesity, pregnancy, standing for long
periods of time. menopause
O Pelvic vein reflux (PVR): ovarian vein
reflux. internal iliac vein reflux
• Hyperhomocysteinemia destroying
structural proteins in vessels
• Chronic alcohol use
COMPLICATIONS
• Chronic venous insufficiency
• Venous ulcers
° Can develop into carcinomas, sarcomas
over time (rare)
• Superficial thrombophlebitis
) (__ s,_G_NS_&_S_Y_M_PT_O_M_s_)
• Twisted superficial veins
• Edema, pain (usually in evening)
• Pruritus in affected area/stasis dermatitis
because of undrained waste in leg
• Prolonged bleeding, slowed healing in
injuries to adjacent areas
• Restless legs syndrome
(..____ D_IA_GN_O_s,_s)
DIAGNOSTIC IMAGING
Doppler ultrasound
• Used to discover subcutaneous varicosities,
assess saphenofemoral junction
• If blood reflux spotted during Valsalva
manoeuvre----> valve incompetence
• Reflux> ls ----> surgical intervention
OTHER DIAGNOSTICS
Trendelenburg test
• Person laid back on flat surface, leg raised
above heart, blood will flow towards heart
----> compress upper thigh with tourniquet
(not too tightly) ----> lower leg onto flat
surface ----> person stands, refilling times
assessed
O Normal: superficial saphenous vein fills
< 30-35s
° Faster filling ----> valvular incompetence
below compressed area ----> deep/
communicating veins
O Slower filling ----> tourniquet released ----> if
filling sudden----> incompetent superficial
veins
183
(..____ T_R_E~_~_M_EN_T
)
SURGERY
• Radiofrequency/laser ablation
• Sclerotherapy
• Ambulatory phlebectomy: removal of
surface vein through slits in skin

OTHER INTERVENTIONS
• Preventative: compression stockings/
devices. avoid prolonged standing

Figure 26.7 An X-ray image demonstrating

varicose veins of the left leg.

Figure 26.8 Illustration of a varicocele in the left testicle.

184
 NOTES

- NOTES
•
~
VENTRICULARTACHYCARDIA

GENERALLY.WHAT IS IT?
COMPLICATIONS
( PATHOLOGY & CAUSES ) • Sustained VT may result in sudden cardiac
death due to insufficient blood perfusion/
• Depolarization wavefronts originate in rapid ventricular fibrillation
ventricles - ventricles pump > 100 beats
per minute! stroke volume
• Premature ventricular contractions (PVCs): (__ s,_G_NS_&_S_Y_M_PT_O_M_s_)
single instance of ventricle contracting
prematurely • Chest pain, syncope, dizziness, shortness
0 2:three PVCs consecutively defined as of breath, palpitations
ventricular tachycardia (VT)

TYPES ( D_IA_GN_O_s,_s
__ )
Monomorphic VT LAB RESULTS
• Ventricular contractions have typical, • Serum electrolytes
uniform shape • Toxicology studies (therapeutidrecreational
• Typical for reentrant circuits drug use)
O Depolarizations begin from same spot, • E.g. digoxin, tricyclic antidepressants,
for focal VT because one area of cells in methamphetamine, cocaine
ventricle is responsible
O Often caused by reentry around scar OTHER DIAGNOSTICS
in ventricular wall; e.g. from previous
myocardial infarction (Ml) ECG
• Determines cardiac rhythm
Polymorphic VT
• > One QRS complex morphology type
O Includes Torsades de pointes ( T_R_EA_~_M_EN_T__ )
• Shape of contractions from each beat
changes as signal begins in different areas MEDICATIONS
of ventricle • Pharmacotherapy
• May occur when pacemaker cells stressed, O Depending on cause
increasing automaticity rates, including
from severe hypoxia
SURGERY
• Implanted devices
RISI( FACTORS
• Ventricular muscle ischemia, structural
heart disease. coronary artery disease
OTHER INTERVENTIONS
(CAD). electrolyte abnormalities • Cardioversion, pacing
• Correct underlying cause

185
 E,U.LTF-0 l A\t\) IOu i~M
"' ~t~\1.1rt e.ltt+Y1Lal ~tt,'vit} iJ

0'1\( ~-

M~tJ01MoiPtt" Vt "- -re.e.wtro."l;.. + f~\ (oYlt ,grov.l')

r --~~~---A,._~----------------~,

Figure 27.1 Illustration depicting ECG of monomorphic ventricular tachycardia.

~oc."L
VaJTl\CULAi
TAlH'<C.A~~\~
it abwiornoa.l AulOf.-ATl"T'f
I
' c::; ~(lu ce\\s
5.e.'1\4.. S\5V10.\S

Figure 27.2 Illustration depicting focal ventrical tachycardia.

186
 LONG QT SYNDROME (LQTS)
osms.i-l/long-q,-l-s14ncJ.Tome
Acquired
( PATHOLOGY & CAUSES ) • Usually caused by certain drugs (e.g. anti-
infectives, psychotropics, antiarrhythmics,
• Cardiac rhythm disorder characterized by
antineoplastics, bronchodilators, gastric
prolonged ventricular repolarization
motility agents)
• Characterized by abnormally long QT
n Common mechanism involves blockage
interval
of rapidly activating potassium channels
O QT interval: total time from ventricular (I Kr) current in potassium channel
depolarization (QRS complex) to encoded by KCNH2 gene
complete repolarization (T wave);
measured from beginning of QRS to end
ofTwave RISI( FACTORS
O QTc (corrected) accounts for changes • Electrolyte imbalances (e.g. hypokalemia,
in heart rate: QTc = QT interval 7 y'RR hypomagnesemia, hypocalcemia)
interval (in sec); AKA Bazett formula • Underlying heart disease (e.g. HF,
O Adult normal = 420 ± 20 msec hypertrophic left ventricle, history of
myocardial infarction)
• Results in j risk of polymorphic ventricular
arrhythmias (TdP), which can deteriorate • Bradyarrhythmias
into ventricular fibrillation • Biological females > biological males
• l age
TYPES • Inherited genetic mutation
• Postpartum period
Inherited O Related to physiologic stress, altered
• Caused by mutations in genes associated sleep patterns
with cardiac potassium, sodium channels • Anorexia nervosa
• Triggered by exertion, emotional events,
stress, postpartum events, noise
COMPLICATIONS
• ~ 13 types identified; associated with
• Malignant arrhythmias (TdP, VF), syncope,
mutations in genes encoding myocyte ion
seizures, sudden death
channels
• KCNQl, KCNH2, KCNEl, KCNE2 affect
potassium channels - ! outward
potassium current
(__ s,_G_NS_&_S_Y_M_PT_O_M_s_)
• SCN5A affects sodium channels - j • Palpitations, lightheadedness, hypotension
inward sodium current
O Two distinct LQT phenotypes due to
mutant alleles in same locus: (...____ D_IA_GN_o_s,_s __ )
O Romano-Ward syndrome: autosomal

dominant; LQTS without hearing loss LAB RESULTS

O Jervell and Lange-Nielsen syndrome: • Serum electrolytes
autosomal recessive; LQTS with • Hypokalemia, hypomagnesemia,
congenital sensorineural hearing loss hypocalcemia may be present

187
OTHER DIAGNOSTICS SURGERY
12-lead ECG Congenital LQTS
• Prolonged QTc (> 470msec in males, • Left cervicothoracic sympathectomy (LCTS),
> 480msec in females); presence of left cardiac sympathetic denervation;
tachyarrhythmias (TdP); altered T-wave implantable cardioverter-defibrillator (ICD);
morphology pacemaker

Bicycle/treadmill stress test Acquired LQTS

• Presence of exercise-associated • Pacemaker - if bradycardia triggers
arrhythmias arrhythmia

Catecholamine drug testing

• Differentiates etiology
O Provocative testing with catecholamine;
e.g. epinephrine
O Measure effect on QT interval
Clinical/family history, physical examination
• With compatible findings
OTHER INTERVENTIONS
Acquired LQTS
• Address underlying cause; e.g. correct
electrolyte abnormalities, discontinue
offending drug; temporary transvenous
overdrive pacing, electrical cardioversion/
defibrillation

Genetic testing Lifestyle modifications

Schwartz score • Avoidance of triggering drugs, avoidance

adrenergic stimuli; e.g. strenuous exercise,
• Diagnosis of congenital LQTS by scoring
emotional stress
QTc, clinical factors, individual history
• Scoring: probability of congenital LQTS
O s 1: low
0 1.5-3: intermediate
0 ~ 3.5: high

( T_R_E~_;i-_M_EN_T__ )
QTc (ms)
MEDICATIONS
> 480 3
Congenital LQTS
• Beta-blockers: blunt adrenergic response 460-479 2
• Mexiletine: for sodium-channel mutations
• Flecainide: if SCN5A mutation 450-459 (males) 1

Acquired LQTS 4-minute recovery from

1
exercise stress test > 480
• Magnesium sulfate: treatment, prevention
of recurrence of long QT-related ventricular TdP 2
ectopic beats
• lsoproterenol: increase sinus rate, decrease T wave alternans 1
QT interval
• Lidocaine/phenytoin: shorten duration of Notched T wave (in 3 leads) 1
the action potential
Low HR rate (age adjusted) 0.5

188
 DIAGNOSIS OF C.ONGENITAL LQTS
CLINICALHISTORY

Syncope

With stress 2

Without stress 1

Congenital deafness 0.5

FAMILYHISTORY
LQTS confirmed in family
1
member

Unexplained sudden death in

immediate family members 0.5
(< 30 years of age)

Figure 27.3 ECG trace demonstrating long-QT syndrome.

189
 QT \\J,E~\l~L QT \\JTE~\l~L
Lu.su..a.\~} c ~ conl.\o..l °a-e,\t LloV'l~t.r -b,io..n V\OY'V\'IG\I

Figure 27.4 ECG trace demonstrating long-QT syndrome.

TORSADES DES POINTES (T&P)

osms.tl:/-l0Tse1cles-cle- oin-les
MNEMONIC: T04SAOE
( PATHOLOGY & CAUSES ) Drugs that may induce QT
prolongation
• Literally means "twisting of the points"
Thiazides
• The peaks of QRS complex "twist" around
04 - Oanzapine, Opioids,
isoelectric line on electrocardiogram
Quinidine, Quinolones
• Lengthening QT interval ----> early
Sotalol/SSRls
afterdepolarizations (EADs) ----> premature
ventricular depolarizations----> polymorphic Antihistamines/antipsychotics
VT (TdP) AntiDepressants like TCAs
O May resolve spontaneously Erythromycin (Macrolide
O Transmural reentry/abnormal antibiotics
automaticity may perpetuate TdP
O May degenerate into ventricular

fibrillation (__ SI_G_NS_&_S_Y_M_PT_O_M_

RISI( FACTORS • Palpitations, lightheadedness, syncope
• LQTS, drugs associated with LQTS
• Bradycardia
( D_IA_GN_O_s,_s
__ )
• Electrolyte imbalance
• Biologically female LAB RESULTS
• Anorexia nervosa • Serum electrolytes
, Hypokalemia, hypomagnesemia,
COMPLICATIONS hypocalcemia may be present
• Ventricular fibrillation, seizures, sudden
cardiac death

190
OTHER DIAGNOSTICS SURGERY
• 12-lead ECG • Left cardio-thoracic sympathectomy
O Ventricular rate: 150-300 beats per
minute OTHER INTERVENTIONS
O RR interval: irregular • Treat underlying cause; e.g. correct
O P wave, PR interval: absent electrolyte abnormalities, discontinue
O QRS duration:> 0.12 seconds; changes offending drug
amplitude, shape, direction • Temporary pacing, permanent dual
chamber pacemaker, implantable
cardioverter-defibrillator (ICD)
( T_R_E~_~_M_EN_T__ )
MEDICATIONS
• For acquired LQTS/other causes of TdP
O Magnesium sulfate, isoproterenol,
lidocaine, phenytoin
• For congenital LQTS
O Beta-blockers, mexiletine

t [\"VII V1 Vt -

·1 r/t\ V\V\ / :-N\Afl,uv-,v....x-J,._.~N\j)A1'\JV"f~J'~VVVV'JV ij'{l/1/V{

~vJvvvwli.J1Vvwv~~~ft'1 ;r rwl/VV\~·v~1r 1 I

·:1)~ \ v\Mw1\/iw,Nwv1rM).JV!fl.rvif 0NIAf~Ml1

" v
, vt.1}'\}VMV\/!•J\)\j\r'\\NV J\.Af'Y'lr,v--.J\..0,.fi:Jv\4\j'i\J'..~v"-. -0-tV\Mrv{1
Figure 27.5 ECG demonstrating torsades de pointes.

191
 VENTRICULAR TACHYCARDIA
osms.i"l/ven-lrieule1r--le1eh14ee1rdie1
RISI( FACTORS
( PATHOLOGY & CAUSES ) • I age
• Cardiac disease
• Ventricular arrhythmia originating in
ectopic ventricular pacemaker, resulting in , Post-Ml, cardiomyopathy, valve disease,
~ three premature ventricular complexes HF
(PVCs) occurring at z 100 beats/min • Electrolyte imbalance
• Dysrhythmia may be sustained (> 30 • Cardiac ion channelopathies resulting in
seconds)/nonsustained (< 30 seconds)/ long QT syndromes
paroxysmal • Infiltrative disease; e.g. amyloidosis
• Abnormally fast ventricular contractions • Pericardia! inflammation
- !ability for ventricles to fill - ! • Blunt chest trauma
cardiac output - ! perfusion - impaired • Drugs; e.g. cocaine
hemodynamics

COMPLICATIONS
TYPES • Cardiac ischemia, infarction
Non-reentrant/focal ventricular tachycardia • May degenerate into ventricular fibrillation
• Triggered by abnormal automaticity of • Sudden cardiac death
specific area of ventricle
O Ventricular pacemaker cells fire at high
rate, preventing pacemaker cells in SA ( SIGNS & SYMPTOMS )
node from firing - heart rate driven by
ventricular pacemakers • Chest pain
O May be caused by certain medications. • Shortness of breath
illicit drugs (e.g. methamphetamine, • Dizziness
cocaine), electrolyte imbalances, • Syncope
myocardial ischemia in ventricles
• Pallor
Reentrant ventricular tachycardia • Blood pressure: norrnal/]
• More common than focal VT
• Reentry: perpetual electrical signal that ( D_IA_GN_O_s,_s
__ )
occurs due to changes in refractory period
length, rate of signal conduction
LAB RESULTS
° Cardiomyocytes can be altered when
• Serum electrolytes
stressed/irritated by external stimuli;
, Hypokalemia, hypomagnesemia,
e.g. medications/illicit drugs: change
conduction speed, refractory period hypocalcemia may be present
O Dead cells in myocardial tissue create
scar tissue - conduction signals go OTHER DIAGNOSTICS
around scar - perpetual signal, AKA
reentry ECG
• Rate: > 100 beats per minute, irregular
• P waves: may be absent
O If present, atrioventricular dissociation
common (hallmark of VT)

192
 O May be positive/upright or negative/
inverted in Lead II
• PR interval: none
• QRS complex: wide ( > 0.12 secondsj. a
3 consecutive; distorted shape: may be
monomorphidpolymorphic
• T-waves: large; polarity may be opposite
of major QRS deflection; may be difficult to
distinguish

( T_R_E~_iT_M_EN_T__ )
MEDICATIONS
• Chronic prevention
O Beta blockers, amiodarone,
nondihydropyridine calcium channel
blockers

OTHER INTERVENTIONS
• Acute treatment
° Cardioversion
• Drug cardioversion
O Procainamide, lidocaine, amiodarone
frequently used
• Electrical cardioversion
O Primary treatment for VT associated
with hemodynamic instability/when
drug cardioversion not immediately
available
• Radiofrequency catheter ablation
• ICD

193