Junctional Arrythmias

by Armando Faigl
Overview Junctional rhythms occur when the AV node takes over as the primary pacemaker.  Junctional
rhythm usually is associated with a benign course
Mechanism
 SA node has failed
 AV node just wants to be faster and takes over
o Enhanced automaticity in AV nodal cells
Think AVNRT – Seperate condition where a re-entry loop stimulates the
AV node again and again
Aetiology
 Digoxin – classic cause
 Beta-agonists, e.g. isoprenaline, adrenaline
 Myocardial ischaemia
 Myocarditis
 Cardiac surgery
 Hypokalaemia
 Cardiac surgery
Junctional rhythm ECG features
 Narrow QRS (<1.2)
 Ventricular rate 60-100
 Shortened PR interval
 Inverted P wave just before QRS
 P wave buried in QRS
Remember Junctional tachycardia has narrow QRS, absent or inverted P
waves
Differential Diagnoses
 AVRT
 AVNRT
Management
 Usually observation
 Treat underlying cause if needed
 Discontinue causative medications
 Permanent pacemaker

Marfans Syndrome
by Armando Faigl and Sze Nichole

Overview
Overview Marfan’s Syndrome is one of the most common inherited disorders of connective tissue. Marfan
syndrome is a hereditary disease affecting connective tissues in the body, resulting in symptoms such as aortic
dissection and musculoskeletal deformities. However, no family history of Marfan’s does not exclude the
diagnosis as 30% can occur through gene mutation.
Definition
Marfan syndrome: Autosomal dominant disease as a result of
 mutation in the FBN1 gene. FBN1 gene mutation results in decreased
production of fibrillin microfibrils and increased production of TGF-
beta, which produces various clinical symptoms and signs.
Fibrillin microfibrils are large glycoproteins that form part of the
extracellular matrix. Fibrillin microfibrils endow connective tissues
with long-range elasticity

Antoine Bernard- Jean Marfan (1958-1942) a french paediatrician

described a hereditary disorder of connective tissue in a 5 yo girl with
disproportionaly long limbs.

Risk Factors
 Family history of Marfan syndrome
 Family history of aortic dissection or aneurysm
 High parental age (Weak)

Signs and Symptoms

Signs and symptoms of Marfan syndrome varies among patients, they are not generally not presented at birth
and develops overtime with age. For presentations at birth, it is regarded to as neonatal Marfan syndrome.

 Body habitus / musculoskeletal

o High stature, wide arm span – Marfanoid body habitus
o Chest deformity
 Pectus excavatum (Funnel chest)
 Pectus carinatum (Pigeons chest)
 Arachnodactyly (Long and thin digits)
 Scoliosis
 Joint hypermobility
 High arched palate
 Flat feet (pes planus)
 Cardiopulmonary
 Aorta dilatation – causing aortic valve insufficiency
 Aortic aneurysm
 Aortic dissection
 Mitral valve regurgitation
 Cystic medial necrosis (tunica media necrosis of the aorta)
 Pneumothorax due to lung bullae upper lobes
 Ophthalmology
 Ectopia lentis
 Dislocated/subluxed lens
 Myopia with astigmatism (blurred vision as a result of irregularly shaped cornea or lens)

Remember Two cardinal features of Marfan Syndrome is aortic root

dilatation and ectopia lentis. This is used in Diagnosis together with
family history and confirmed FBN1 mutation
 Differential Diagnosis
<20 years old
 Potential Marfans Syndrome
>20 years old
 MASS – Myopia, Mitral valve prolapse, Aortic root dilatation, Aortic aneurysm syndrome, Striae, Skeletal
 Loeys Dietz syndrome
o Hypertelorism, cleft palate/bifid uvula, arterial totuosity, aneurysm and dissection
 Ectopia lentis syndrome
 Ehlers Danlos syndrome (vascular type)
 Marfanoid hypermobility syndrome

Ehlers Danlos Syndrome disorder in connective tissue (collagen),

characterised by joint hypermobility, elastic skin, skin thinning and
bleeding disorders

Investigation and Diagnosis

 Echocardiogram
 Chest X-ray
 CT scan of thorax
 Fibrillin 1 mutation testing
 Slit lamp eye examination
 Ultrasound of abdomen

Ghent Diagnosis (simplified)

Absence of family history
 Aortic root dilatation + Ectopia lentis
 Aortic root dilatation + FBN1 gene mutation
 Aortic root dilatation + systemic score >7points
Presence of family history
 Family history + ectopia lentis
 Family history + systemic score >7points
 Family history + aortic root dilatation

Aetiology
Fibrillin 1 (FBN1) gene mutation, located on chromosome 15

 75% – Inherited the mutated FBN1 gene from one or both parents, autosomal dominant
 25% Sporadic, FBN1 gene underwent mutation in parent germ cells

Pathophysiology
The fibrillin 1 gene is crucial in the production of fibrillin, a glycoprotein which stabilises crosslinks of microfibril
tissues found in connective tissues, such as arteries and skin. Fibrillin either bind microfibril strands to form non-
stretchable tissues (such as tendons), or binds microfibril strands with elastin to form elastic fibres (such as
arteries). It also sequesters transforming growth factor beta (TGF-B), and promotes connective tissue growth.

With the deformed or deficiency in fibrillin proteins due to the gene mutation, connective tissues loss mechanical
integrity.
 Cardiovascular
In the aorta, the lost of integrity in elastic connective tissue causes necrosis of the tunica media, resulting in aortic
dilation, which in turns causes aortic valve insufficiency and heart
failure. There is also increased risk of aneurysm formation and dissection of the aorta, causing mortality related
to Marfan syndrome.
Musculoskeletal
Marfanoid body habitus, scoliosis, kyphoscoliosis and arthropathy are common symptoms present in Marfan
syndrome. Since less fibrillin is present for sequestering the TGF-B, there is a overabundance of such
substances in the systemic circulation, therefore other tissues such as epiphyseal plate responds to the constant
stimulation of TGF-B, resulting in over-production of long bones.
Opthalmopathy
Increased risk of lens dislocation and retinal detachment due to insufficient connective tissue integrity.
Dermatological 
Striae may be seen due to loosening of connective tissues in the dermal layer.
Lungs
Possible bulla formation in the lungs due to lack of mechanical integrity in lung elastic tissues increases risk of
spontaneous pneumothorax.

Management
Treatment of Marfan syndrome is based on symptoms management and improvement of overall life quality of the
patient. Each system affected by the FBN1 gene mutation can be
treated separatly.

 Cardiovascular
o Beta blocker
o Angiotensin II receptor blocker (ARB)
o Prophylaxis for endocarditis and anticoagulants for high risk procedures or surgeries
o Surgical treatment in cfor symptomatic aortic pathology
 Opthalmopathy – surgical for cataracts, myopia, retinal detachment
 Musculoskeletal
o Scoliosis/kyphoscoliosis : Orthopaedic bracing or surgery
o Pectus excavatum or pectus carinatum affecting cardiorespiratory system: Surgery
o Arthropathy: Physiotherapy, analgesics, surgery

Complications and Prognosis

Complications
 Cardiovascular
o Aortic dilation
o Acute aortic dissection/rupture
o Chronic aortic dissection
o Symptomatic aortic regurgitation
o Heart failure
o Infective endocarditis
 Respiratory
o Spontaneous pneumothorax
 Abdominal
o Symptomatic inguinal/incisional/abdominal hernia
Prognosis
 With appropriate medical and surgical intervention addressing the symptoms and complications of Marfan
syndrome, the long term survival rate of most patients are good, most patients can live a normal lifespan with reasonable
quality of life.
 Survival rate is significantly reduced by presentation of aortic dissection even with effective treatment.

References
1. Child. A. and Tome. M. (2019). Marfan syndrome. BMJ Best Practice. https://bestpractice-
bmj-com.wwwproxy1.library.unsw.edu.au/topics/en-gb/514. Last accessed 11 March 2019.

2. Colledge, N. R., Walker, B. R., Ralston, S., & Davidson, S. (2010). Davidson’s principles
and practice of medicine. Edinburgh: Churchill Livingstone/Elsevier.

3. Groenink, M., Lohuis, T. A. J., Tijssen, J. G. P., Naeff, M. S. J., Hennekam, R. C. M., Van
Der Wall, E. E., & Mulder, B. J. M. (1999). Survival and complication free survival in Marfan’s
syndrome: implications of current guidelines. Heart, 82(4), 499-504.

4. Kumar, V., Abbas, A. K., Aster, J. C., & Robbins, S. L. (2013). Robbins basic pathology.
Philadelphia, PA: Elsevier/Saunders. Svensson, L. G., Crawford, E. S., Coselli, J. S., Safi, H. J.,
& Hess, K. R. (1989). Impact of cardiovascular operation on survival in the Marfan patient.
Circulation, 80(3 Pt 1), I233-42.

5. Wright. M. and Connolly. H. M. (2019). Genetics, clinical features, and diagnosis of Marfan
syndrome and related disorders. Up To Date. https://www.uptodate.com/contents/genetics-
clinical-features-and-diagnosis-of-marfan-syndr me-and-related-disorders?search=marfan
%20syndrome&source=search_result&selectedTitl =1~150&usage_type=default&display_rank=1
Last accessed 11 March, 2019.

Angina Pectoris
by Armando HF

Overview
Overview Angina Pectoris refers to the pain caused by myocardial ischaemia. Myocardial Ischaemia is
usually caused by stenosis within the vessel decreasing blood flow to the muscle, but it can be caused by
tachycardia, anaemia, aortic stenosis, left ventricular hypertophy and many other disease. Angina “latin
choking”.
Definitions
Angina Pectoris: Pain caused by myocardial ischaemia (not myocardial
infarction)
Acute myocardial Infarction: Death of myocardial tissue because of
inadequate blood flow
Acute coronary Syndrome: Coronary artery obstruction or rupture can
result in a variety of ischaemic condition which fall under the term of
acute coronary syndrome. This does not include stable angina.
Unstable angina is defined by the absence of biochemical evidence of
myocardial damage. It is characterised by specific clinical findings of
prolonged (>20 minutes) angina at rest; new onset of severe angina;
angina that is increasing in frequency, longer in duration, or lower in
threshold; or angina that occurs after a recent episode of MI.

Risk Factors
Modifiable Non-modifiable
 Smoking Increasing age

Hypertension Male sex

Dyslipidaemia Family history

Diabetes mellitus Ethnicity

Obesity

Physical inactivity

High calorie intake diet

Psychological stress

Types
Angina is classified according to its temporal pattern and its relation to exertion

 Stable angina – angina only on exertion

 Unstable angina – angina on exertion or at rest
 Crescendo angina –
 Decubitus angina – angina by lying flat
Remember Unstable Angina is part of Acute Coronary syndrome.
Stable angina is NOT part of acute coronary syndrome

Signs and Symptoms

Rule out acute coronary syndrome if presenting to Emergency. Stable angina chest pain typically resolves
with rest.

 Chest discomfort (non-sharp pain)

 Dyspnoea
 Fatigue
 Nausea/Vomiting
Remember Myocardial Ischaemia will respond to nitrates almost
immediately

Think Chest wall tenderness suggests musculoskeletal pain and does

not accompany angina

Differential Diagnosis
CAUSES OF CHEST PAIN BY BODY SYSTEMS

Cardiac Respiratory Gastrointestinal Musculoskele Psychogen

 tal ic

Acute Pulmonary Oesophageal Rib Fracture Anxiety

Coronary Embolism rupture
Syndrome

Aortic Pneumothora Pneumomediastin Herpes Zoster Panic

dissection x um attack

Pericarditi Pulmonary Cholecystitis Costochondriti

s Hypertension s

Stable Pneumonia GERD

Angina

Endocardit Lung Peptic ulcer

is Cancer disease

Cardiac Bronchiecta Acute

tamponade sis Pancreatitis

Investigations
 FBC
 EUC
 Blood glucose leves
 Lipid Profile
 Chest X-ray
Test to induce ischaemic chest pain
 Exercise ECG
 Stress echocardiography
 Myocardial perfusion scanning

Management
Non-pharmacological
 Education
 Smoking cessation
 Alcohol limitation
 Lose weight
 Exercise
 Healthy diet
Pharmacological (BANS)
 Betablockers (calcium channel blockers if contraindicated)
 Aspirin
 Nitrates
 Statins
Pharmacology Aspirin is a COX 1/2 inhibitor. It prevents the
production of Prostagladins (inflammation: fever and pain) and
 thromboxane (clotting). It is used to treat fever, osteoarthritis, heart
conditions and stroke. Side effects:
nausea/vomiting, dyspepsia, stomach ulcer or bleeding
problems, headache, dizziness, tinnitus, renal dysfunction and Reye’s
syndrome (particularly in children who have taken aspirin)

Pharmacology Statins are competitive HMG-CoA reductase inhibitors,

an enzyme normally responsible for producing cholesterol. By lowering
the levels, they help prevent heart attacks and stroke. Side
effects: Headache, difficulty sleeping, flushing of the skin, myalgia,
liver enzyme dysfunction, nausea or vomiting, abdominal cramping or
pain, bloating or gas and serious cases myositis and Rhabdomyolysis

Watch Video Acute Coronary Syndrome

Complications
 Acute Coronary Syndrome

References
BMJ
Best Practice
UpToDate

© Armando Hasudungan 2020 | For enquiries, contact me on armandohasudungan@gmail.com | ↑

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Atrial Fibrillation
by Armando HF

Overview
Overview Atrial fibrillation, AF is a chaotic, irregular atrial rhythm at 300-600bpm; the AV node responds
intermittently, hence an irregular ventricular rate. AF is the most common sustained dysrhythmia; characterised
by absent P waves, irregularly irregular heart rate, narrow QRS (typically). It is common in the elderly. The main
risk of AF is embolic stroke either in the mesenteric artery, brain tissue or limb extremities.

Definition
Supraventricular tachyarrhythmia: Arrhythmia arising from the
conduction system above the ventricles, possibly the AV node, atria or
SA node.
Atrial Fibrillation: A supraventricular tachyarrhythmia. It is
characterised by uncoordinated atrial activity on the surface ECG, with
fibrillatory waves of varying shapes, amplitudes, and timing associated
with an irregularly irregular ventricular response when atrioventricular
(AV) conduction is intact.
Acute Atrial Fibrillation: Defined as a new onset or a first detectable
episode of AF, whether symptomatic or not.
Chronic Atrial Fibrillation: is defined as experiencing atrial
fibrillation more than once. It may be paroxysmal, persistent, long-
standing persistent, or permanent.
 Anatomy and Physiology
The cardiomyocytes contract moving blood around and out of the heart. Many factors affect cardiac output, one
of which is heart rate.

Conduction system The heart rate is controlled by the heart’s own special conduction system. The Sino atrial node
(SAN) is the boss and sits high in the lateral right atrium (RA) just below the superior vena cava. The SAN is
richly innervated with sympathetic and parasympathetic fibres, which alter the rate of depolarization hence
controlling the heart rate. This is also the same for the Atrioventricular node which is found in the RA anterior to
the mouth of the coronary sinus and directly above the insertion of the septal leaflet of the tricuspid valve. The AV
node then sends conduction signals to down to the ventricles through the his-purkinje system.

Watch Cardiology – Relationship of conduction system, ventricular

contraction and ECG 

Classification
Three clinical patterns (3Ps) All patterns have a risk of thromboembolism.
 Paroxysmal AF: Defined as recurrent and terminates spontaneously within 24hours -48 without any intervention
 Persistent AF: Defined as AF that is sustained >7 days or lasts <7 days but necessitates pharmacological or
electrical cardioversion
 Permanent (Chronic) AF: refractory to cardioversion or accepted as a final rhythm. A decision has been made not to
pursue restoration of sinus rhythm by any means, including catheter or surgical ablation
Remember Chronic AF is having atrial fibrillation more than once. It
may be paroxysmal, persistent, long-standing persistent, or permanent.

Signs and Symptoms

Clinical Presentation Atrial fibrillation may cause chest pain, palpitations, dyspnea, or faintness. Signs include
irregularly irregular pulse and possible signs of left ventricular heart failure. 30% of patients present with AF as an
incidental finding only
 Think AF may be associated with non-cardiac disease (ie. pneumonia
and hyperthyroidism)

Examination
 Irregular pulse (which if rapid will be faster at the apex than the wrist)
 Variable intensity of the first heart sound
 Absent ‘a’ waves in the JVP

Differential Diagnosis
Palpitations
 Chronic atrial fibrillation
 Paroxysmal atrial tachycardia
 Atrial flutter
 Wolff-Parkinson-White syndrome

Wolff-Parkinson-White syndrome is a condition in which there is an

extra electrical pathway in the heart (accessroy pathway). The condition
can lead to periods of tachycardia

Aetiology
 Heart Failure
 Hypertension
 Post MI
 Mitral Valve disease
 Hyperthyroidism
 Post-Op
 HOCM

Investigations
 ECG – changes in AF or MI

ECG shows absent p waves, irregularly irregular rhythm, QRS (typically narrowed). Rate is usually about 150bpm

 Echocardiogram – valvular heart disease

 Chest x-ray – heart failure
 Troponin – MI?
 ABG – if shocked, hypoxic or signs of acidosis
 For non-cardiac causes
o Thyroid function test
o Full blood count – ↑WCC (pneumonia)
o EUC – hypokalaemia +/- renal impairment
 Other investigations when the patient is stable
o 24-hour ambulatory monitor to assess heart-rate control and look for episodes of symptomatic bradycardia
o Exercise test (or other ischaemia stress test)
o Coronary angiography
o Cardiac magnetic resonance.

Management
The treatment of atrial fibrillation needs to be considered under three seperate headings:

 Diagnose AF stable or unstable?

o If unstable – Cardioversion
 Rate control
 Rhythm control
 Thromboembolic disease prevention
Haemodynamically unstable AF
 ABCD
 Correct underlying electrolyte imbalance
 ECG
 Emergency cardioversion – if unstable AF
o Electrical – 100 joules
o Chemical – Amiodarone OR flecinide
 Identify underlying cause
 Treat underlying cause
Acute AF but haemodynamically stable
 Either Rate control or Rhythm control
 Rate Control – Betablocker or CCB or Digoxin
o Digoxin good in Heart failure
 Consider elective cardioversion
o Tranoesophageal ultrasound before cardioversion to prevent thromboembolism from atrial ‘stunning’ OR
o Start anticoagulants for 3weeks to reduce risk of thromboembolism
 Heparin bridging for Warfarin (INR 2-3) OR
 NOAC – Dapigatran (Factor IIa inhibitor)
 Maintenance therapy after cardioversion or after AF has revereted
 Rhythm control – flecanide OR sotalol OR amiodarone (most effective but crazy side effects)
 no need for antiarrythmic if AF is associated with a transient condition
 Assess risk for embolus using (CHADSVASc SCORE)
 If low emboli risk – stop anticoagulant if sinus rhythm normal and risk of emboli is low.
 If moderate – high emboli risk start prophylaxis
 Aspirin
 Warfarin (INR 2-3) OR NOAC – dapigatran
Chronic AF – ongoing management
 Rate Control – Betablocker or CCB or Digoxin
 Anticoagulation
o Warfarin OR
o NOAC
 Rhythm Control – Sotalol or Amiodarone
 Catheter or surgical ablation if rate and rhythm control ineffective
 Sidenote Warfarin (international normalized ratio (INR) >2) reduces
strokes in AF by 60%, regardless of underlying risk

CHADS2 Scoring system to assess risk of stroke in AF

(nonvalvular)

1 point each 2 point each

Congestive Heart Failure Stroke or TIA previously

Hypertension

Age >75yo

Diabetes

CHA2DS2 – VASc Scoring system to assess risk of stroke in AF

(nonvalvular)

1 point each 2 point each

Congestive Heart Failure V

Hypertension Age >75

Age >65yo Stroke or TIA previously

Diabetes c

Sex – female

Vascular disease

Pharmacology Warfarin is an anticoagulant. It is used for AF, valvular

heart disease and pulmonary embolism. Mechanism of action: Vitamin
K antagonist inhibiting the production of coagulation factors VII, IX, X
(these require Vitamin K to be produced). Side effects: Allergy, acute
bleeding. When bleeding occurs as a result of warfarin this can be
reversed by administering Vitamin K.

Pharmacology Digoxin is used for Heart failure and AF. Digoxin

works by inhibiting the Na/K+ pump in cardiomyoctyes, this causes
↑intracellular Na+ together with Ca+ causes stronger contractions at a
slower rate.  Side effects: Nausea, vomiting, headache,
dizziness/vertigo, loss of appetite, diarrhea, Digoxin toxicity
(arrythmias).

Pharmacology Amiodarone is a K+ channel blocker (class III

 antiarrythmic). It acts on the myocardium to delay repolarization and
increase duration of the action potential. It also decreases SA and AV
conduction rate. Side effects (6Ps): Prolongs action potential
duration, Photosensitivity, Pulmonary fibrosis and
inflammation, Pigmentation of skin, Peripheral neuropathy, Peripheral
conversion of T4 to T3 is inhibited (Hypothyroidism, but it also can
cause hyperthyroidism)

Complications and Prognosis

Complications
 Death
 Bradycardia
 Stroke
 Heart failure
 Hypotension
 Amiodarone toxicity
o Pulmonary Inflammation +/- Fibrosis
o Thyroid Dysfunction

Remember 6Ps of Amiodarone side effects (6Ps): Prolongs action

potential duration, Photosensitivity, Pulmonary fibrosis and
inflammation, Pigmentation of skin, Peripheral neuropathy, Peripheral
conversion of T4 to T3 is inhibited (Hypothyroidism). 
Prognosis depends on several factors, such as the precipitating event, underlying cardiac status, risk of
thromboembolism, and whether the nature of the AF is paroxysmal, persistent, or permanent.

References
UpToDate
Best Practice
Oxford Handbook of Cardiology