BASIC CARDIAC STRUCTURE AND FUNCTION

Cardiac Anatomy and Histology

1. Pericardium
 Encloses the heart and its roots
 The pericardium consists of two layers: strong outer fibrous layer and
inner serosal layer.
 Inner serosal layer: adheres to the external wall of the heart  visceral
pericardium. It reflects back on itself and lines the outer fibrous layer
(parietal pericardium). The space between the visceral and parietal
layers contains thin pericardial fluid (causes less friction when the heart
beats).
 Pericardium nempel ke sternum dan bagian dari mediastinum to the
left and right pleurae  these keeps the pericardial sac firmly attached
within the thorax, helps maintaining the heart normal position.

2. Surface Anatomy of the Heart


3. Internal Structure of the Heart
a. Right Atrium & Ventricle
b. Left Atrium & Ventricle
c. Interventricular Septum
4. Histology of Ventricular Myocardial Cells
Cardiac Imaging

FETAL CIRCULATION
WAKTU MASIH FETUS RV ITU WORKHORSE DARI JANTUNG, 2/3 TOTAL
CARDIAC OUTPUT DARI RV.
1. Oxygenated blood leaves placenta through umbilical vein
2. Half of the blood is shunted through fetal ductus venosus  bypassing
hepatic vasculature  IVC
3. Remaining blood from umbilical vein  portal vein  liver  hepatic vein
 IVC
4. IVC = mixture of well-oxygenated blood from v. umbilicus + low-oxygenated
blood from the systemic veins of the fetus  bcs of this O2 tension di IVC >
blood returning from SVC to RA
5. Nanti di RA bakal di separate bloodnya  yang low oxygen ke placenta (via
descending aorta & a. umbilicalis), yg high oxygen bakal dibawa ke fetal
brain & myocardium (facilitated by crista dividens  posisinya di opening
IVC into RA)
6. IVC  RA  foramen ovale  LA
7. Di LA darah yg well-oxygenated kecampur sama poorly oxygenated blood
from lungs (in utero lungs gak terventilasi, developing pulmo tissues
removes O2 from blood) di LA via pulmonary veins
8. LA  LV  ascending aorta. Dari ascending aorta dibagi-bagi distribution
darahnya:
 9% enters coronary arteries  perfuses myocardium
 62% enters the carotid & subclavian vessels  upper body & brain
 29% enters the descending aorta  rest of the fetal body
9. REMAINING well-oxygenated blood di IVC yang masuk ke dalem RA ke
mix sama poorly-oxygenated blood dari SVC  pulmonary artery, nanti
kebagi-bagi lagi:
 12% ke lungs
 88% ke ductus arteriosus  descending aorta (ketemu sama well-
oxygenated blood dari LV)
10. Descending aorta:
  Lower body
 Umbilical artery  placenta (for gas exchange)
*The unequal RV outflow is efficient and needed for this case:
 Bypassing the lungs itu dibutuhkan bcs di fetus lungs itu belum terbentuk
sempurna & masih ada amniotic fluid di dalemnya  incapable of gas
exchange
 Low oxygen tension of the amniotic fluid  constriction of pulmonary vessels
 increases pulmonary vascular resistance  facilitates shunting of blood
through ductus arteriosus to sirkulasi sistemik.

TRANSITIONAL CIRCULATION
1. Birth  umbilical cord di clamp/constricts naturally  low-resistance placental
flow is removed from the arterial system  increase in systemic vascular
resistance
2. Pulmonary vascular resistance turun, bcs:
 Mechanical inflation of lungs after birth  stretches lung tissues 
pulmonary artery expansion & wall thinning
 Rise in blood oxygen tension + aeration of lungs  vasodilatasi
pulmonary vasculature
3. Pulmonary vasculare resistance turun  peningkatan pulmonary blood flow
(marked within the first day after birth-several weeks, sampe akhirnya adult
level of pulmo resistance ke achieve)  peningkatan blood flow to lungs thru
pulmonary artery
4. Peningkatan venous return v. pulmonalis ke LA meningkat  LA pressure
meningkat
5. Cessation of umbilical venous flow + konstriksi duktus venosus  pressure
IVC & RA turun  pressure di LA > RA  valve nya foramen ovale is
forced against septum secundum  foramen ovale ketutup (kalo gak
ketutup jadi PFO)
6. Oxygenation (oxygen tension increases  no longer hypoxia  PGE1 level
declines  smooth muscle yg ada di ductus arteriosus constricts)  ductus
arteriosus menutup (kalo gak ketutup jadi PDA)
 7. Left & right side of the heart udah kepisah secara anatomis  stroke volume
di LV > RV  equalizing the cardiac output from both ventricles
8. Pressure & volume yang sekarang lebih banyak ada di LV  induces
myocardial cells di LV buat hipertrofi
9. Pressure & volume yang skrg berkurang di RV  regresi wall thickness yang
ada di RV

CONGENITAL HEART DISEASE

ACYANOTIC LESION
1. Arterial Septal Defect (ASD)
Definition
 Persistent opening in the interatrial septum after birth  allows direct
communication between the left and right atrias
 Common  incidence 1 in 1500 live births
 Types:
o Ostium secundum: MOST COMMON. Occurs at the region of
foramen ovale. Terjadi karena inadequate formation of septum
secundum/excessive resorption of septum primum/both 
endochondral cushionnya gak nempel.
o Ostium primum: less common. Terjadi di inferior portion of
interatrial septum, adjacent sama AV valves.  failure of the
septum primum to fuse with the endochondral cushions  ada
opening dibagian bawah septum  associated with AV
abnormality (mitral regurgitation)
o Sinus venosus: absence of normal tissue between v. pulmo
dextra & RA (bukan defek dari septum atrium nya). Biasanya
besar defectnya & causes flow from the v. pulmo dextra & LA
enters RA (patofnya mirip TRUE ASD)
  ASD is related with PFO (patent foramen ovale)  20% ada di general
population (not true ASD). Biasanya PFO itu clinically silent karena
valve itu one way (jadi walaupun gak tertutup rapat, dia tetep “closed”)
 pressure LA tetep lebih tinggi daripada RA. Tapi PFO itu baru
clinically significant kalo pressure RA > LA (e.g. pulmo HTN/RHF) 
right-to-left shunt  deoxygenated blood passes into the arterial
circulation (bypassing the lungs)  hypoxemia
Pathophysiology
 Uncomplicated ASD: oxygenated blood from LA shunted into RA
(bukan kebalikannya) = LEFT TO RIGHT SHUNT at the atria level
 LEFT-TO-RIGHT SHUNT (LA  RA)  overload & enlargement of RA
+ RV  compliance (filling properties) dari RV diminishes slowly (due
to excessive load)  left-to-right shunt berkurang
 THE SHUNT MAY REVERSE KALO ADA EISSENMENGER
SYNDROME! (dari left-to-right shunt jadi right-to-left shunt) 
biasanya due to pulmo HTN/RHF  causing hypoxemia & cyanosis
Symptoms
 Infants biasanya asymptomatic
 Murmur waktu lagi childhood/teen, tapi biasanya subtle (25% ASD gak
terdiagnosis until adulthood)
  DOE
 Fatigue
 Recurrent LRTI: due to increased blood flow ke lungs  risk of getting
hematogenous infection meningkat
 Palpitation: atrial tachyarrythmias from right atrial enlargement (SVA)
PF
 Prominent systolic impulse at lower-left sternal border (due to dilated
RV  RV heave)
 Fixed split S2 (left to right shunt that increases the blood flow to the
right side of the heart, thereby causing the pulmonary valve to close
later than the aortic)
 Systolic murmur at upper left sternal border (increased blood flow to
the pulmo valve)  crescendo-decrescendo murmur
 Mid-diastolic murmur at lower-left sternal border (increased blood flow
to the tricuspid valve)
Diagnosis
 CXR: dilatasi RA & RV + pulmo arteri prominen (increased pulmonary
vascular markings)
 ECG: RVH, RAE, complete/incomplete RBBB
 Doppler/echocardiography: to assess RA & RV enlargement and to
look for ASD
Treatment

2. Ventricular Septal Defect (VSD)

Definition
 Abnormal opening in the interventricular septum
 Common  incidence 1.5-3.5 per 1000 live births
 Types: membranous (70%) and muscular (20%), AV conal
type/subpulmonary (rare)
Pathophysiology
 Depends on the size of the defect and the relative resistance of the
pulmonary and systemic vasculature
  Small VSD: more resistance to flow than the pulmonary/systemic
vasculature  preventing significant left-to-right shunting
 Large defects:
o Pulmonary vasculature resistance decreases (bcs the lungs
inflate)  pressure di LV > RV (left-to-right shunt)  the blood
goes from the high pressure to low pressure  the blood
kekumpul semua di RV  pulmonary artery to lungs (karena
ada overload blood dari RV ke lungs, akhirnya pulmonary artery
kompensasi by decreasing its diameters)  increased
pulmonary vascular resistance (due to decreased diameter of a.
pulmo)  pressure in RV > LV (RHF/pulmo HTN)  right-to-
left shunt (Eissenmenger’s Syndrome)  cyanosis
o Increased blood volume  LA & LV dilates & hypertrophied

Symptoms
 Small VSD: asymptomatic
  10% of infants punya large VSD (symptomatic): failure to thrive, poor
feeding, tachypnea, frequent LRTI (early HF symptoms)
 VSD + complicated pulmonary vascular disease: dyspnea + cyanosis
 Bacterial endocarditis
PF
 Holosystolic murmur at left sternal border (lebih kecil defeknya, lebih
gede suara murmurnya, karena turbulence nya semakin besar)
 Systolic thrill di region murmurnya
 Mid-diastolic rumbling murmur at the apex (increased flow to mitral
valve)
 Kalo ada pulmo complication: murmur berkurang, RV heave (+), loud
P2 (+), cyanosis (+)
Diagnosis
 Small defects CXR normal
 Large defects: cardiomegaly + prominent vacular markings (+)
 ECG: LAE + LVH + RVH (kalo udh ada komplikasi pulmo)
 Echochardiography: determine the location of VSD (direction &
magnitude)
 Cardiac catheterization: increased O2 saturation in RV > RA (result of
shunting highly oxygenated blood from LV into RV)
Treatment

3. Patent Ductus Arteriosus (PDA)

Definition
 Ductus arteriosus is the vessel that connects pulmonary artery to
descending aorta during fetal life. PDA = failure of closure of ductus
arteriosus after birth  persistent connection between the descending
aorta & pulmonary artery
 Incidence: 1 in 2500-5000 live term births
 Risk factors: rubella infection (first trimester of pregnancy), prematurity,
birth at high altitude
Pathophysiology
 PDA = persistent shunt between descending aorta & pulmonary artery
  Pulmonary resistance decreases after birth (due to lung inflation &
oxygenation)  blood flows from descending aorta into pulmonary
artery (left-to-right shunt)  lungs  pulmonary veins  LA  LV
 Increased blood volume in LA & LV (bcs of the shunting)  volume
overload  LV dilatation & LHF (right heart will remain normal unless
pulmo complication ensues  Eissenmenger syndrome)
 Eissenmenger syndrome in PDA: pulmonary artery constricts 
increased pulmonary vascular resistance  deoxygenated blood flows
from pulmonary artery via the PDA  descending aorta  lower
extremity (deoxygenated blood flows to the lower extremity, causing
cyanosis of the feet BUT upper extremitynya gak cyanotic
because they receive normal oxygenated blood from proximal
descending aorta into the ductus)

Symptoms
 Usually asymptomatic
 Large shunts: early CHF + tachycardia, poor feeding, slow growth,
recurrent LRTI
 Moderate shunts: fatigue, dyspnea, palpitation (teen & adult)
  Afib (LA dilatation)
 Endarteritis = endovascular infection (turbulent flow across the defect)
PF
 Continuous murmur (kyk mesin) at subclavicular region
 Eissenmenger: clubbing finger, differential cyanosis
Diagnosis
 CXR: LA & LV enlargement + prominent pulmonary vascular markings
 ECG: LAE + LVH (kalo large shunt)
 Echo to visualize the defect
 Cardiac catheterization gaperlu
Treatment

CYANOTIC LESION
1. Tetralogy of Fallot (ToF)
Definition
 Resulted from a single developmental defect: abnormal anterior and
cephalad displacement of infundibular (outflow tract) portion of
the intraventricular system
 Four anomalies: VSD (anterior malignment of the interventricular
septum), subvalvular pulmonic stenosis (obstruksi dari displaced
intraventricular septum, biasanya suka ada pulmo stenosis juga),
overriding aorta (receives blood from both ventricles), RVH (due to high
pressure load di RV from pulmo stenosis)
 Most common cyanotic heart disease of infancy  5 of 10.000 live
births (usually assosicated w/ other cardiac defects, e.g. right-sided
aortic arch, ASD, anomalous origin of the left coronary artery)
Pathophysiology
 Pulmonary stenosis  increased pulmonary vascular resistance 
deoxygenated blood dari sirkulasi sistemik (yang ada RV otw ke
pulmonary arteries) terdorong kembali ke RV  VSD  LV  aorta 
systemic circulation
Symptoms
 DOE
 Tet “spells”: biasa abis nangis/makan/capek tiba2 jadi cyanosis (px
biasanya jongkok biar “ngelipet” a. femoralisnya biar tekanan dari a.
femoralis meningkat  darah dikembalikan ke a. iliaca + aorta
meningkat  mengurangi right-to-left shunt soalnya darah dari LV ke
RV via VSD  jadi lebih banyak darah di RV buat dibawa ke a. pulmo)
 Hiperventilasi, cyanosis, syncope, convulsion
PF
 Moderate pulmonary stenosis: mild cyanosis (lips, mucous membrane,
jari tangan)
 Severe pulmonary stenosis: profound cyanosis bbrp hari setelah lahir
 Chronic hypoxemia from right-to-left shunt = finger clubbing
 RVH: palpable heave di left sternal border
 Systolic ejection murmur at the left upper sternal border
Diagnosis
 RVH, decreased size of pulmonary artery  BOOT-SHAPED
APPEARANCE
  Pulmonary vascular markings berkurang  decreased flow to lungs
(pulmo stenosis)
 ECG: RVH + RAD
 Echocardiography: to find the outflow tracts (VSD, RVH, dll)
Treatment

2. Transposition of the Great Arteries (TGA)

Definition
 Each great vessels inappropriately arises from the opposite ventricle
(aorta dari RV, pulmo dari LV)
 7% of congenital heart defect  incidence 40 of 100.000 live births
 Most common etiology of after infancy cyanosis & the most common
cause of cyanosis in neonatal period
 Etiology: unknown  hypothesis: failure of aorticopulmonary septum to
spiral secara normal during fetal development / abnormal growth and
absorption of subpulmonary and subaortic infundibula during the
division of truncus arteriosus (kebalik gitu)
Pathophysiology
 TGA separates pulmonary and systemic circulations by placing the two
circuits in parallel, bukan series.
 Desaturated blood yang dibawa IVC/SVC dari sirkulasi sistemik dibawa
dari RA ke RV (karena TGA, mestinya kan ke RA dulu ke RV buat
dibawa ke a. pulmo)  dari RV lgsg ke aorta  systemic circulation
(neonate nya jadi super hypoxic)
 Oxygenated blood from pulmonary venous dari pulmonary artery 
masuk ke LA  LV  balik ke pulmo artery lg (mestinya dari LV it uke
aorta & sistemik)
 After birth kondisi TGA itu very deadly & lethal, tapi in uetro life: TGA
itu msh gapapa, why?
o Ductus arteriosus + foramen ovale msh kebuka (jadi dua
sirkulasi itu msh ke connect)
 o Oxygenated blood dari placenta  umbilical vein  RA 
foramen ovale  LA  LV  pulmonary artery  ductus
arteriosus  aorta  placenta
Symptoms
 Extremely cyanotic (depends on the parallel circuit)  generalized
cyanosis on the first day after birth  progresses rapidy setelah ductus
arteriosus & foramen ovale terutup
PF
 RV impulse on lower sternal border (systemic pressure di RV
meningkat)
 Auscultation: accentuated S2 (closure of aortic valve yg deket bgt
sama permukaan dada)
Diagnosis
 CXR: egg-on-a-string appearance (The heart appears globular due to
an abnormal convexity of the right atrial border and left atrial
enlargement and therefore appears like an egg.)
 ECG: RVH
 Echocardiography & cardiac catheterization: definitive diagnosis!
Treatment