Arrhythmogenic right ventricular cardiomyopathy

Question 1

Arrhythmogenic right ventricular cardiomyopathy inheritance

Answer 1

autosomal dominant pattern with variable expression

Question 2

pathophysiology of Arrhythmogenic right ventricular cardiomyopathy inheritance?

Answer 2

right ventricular myocardium is replaced by fatty and fibrofatty tissue

Question 3

presentations of Arrhythmogenic right ventricular cardiomyopathy inheritance

Answer 3

palpitations
syncope
sudden cardiac death

Question 4

DIAGNOSTICS

Answer 4

ECG abnormalities in V1-3, typically T wave inversion.

An epsilon wave is found in about 50% of those with ARV - this is best described as a terminal notch in the QRS complex

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BEST

MRI - magnetic resonance imaging is useful to show fibrofatty tissue

Question 5

echo in diagnostics ?

Answer 5

show an enlarged, hypokinetic right ventricle with a thin free wall

Question 6

management of Arrhythmogenic right ventricular cardiomyopathy

Answer 6

sotalol is the most widely used antiarrhythmic

catheter ablation to prevent ventricular tachycardia

implantable cardioverter-defibrillator

Question 7

autosomal recessive variant of ARVC called ?

Answer 7

Naxos disease

Question 8

clinical features of naxos disease

Answer 8

triad of ARVC,
palmoplantar keratosis,
and woolly hair