What is Arrhythmogenic right ventricular cardiomyopathy?

a form of inherited cardiovascular disease which may present with syncope or sudden cardiac death.

What is the inheritance of Arrhythmogenic right ventricular cardiomyopathy?

autosomal dominant pattern with variable expression

What is the pathophysiology of arrhythmogenic right ventricular cardiomyopathy?

the right ventricular myocardium is replaced by fatty and fibrofatty tissue

How does arrhythmogenic right ventricular cardiomyopathy present?

• palpitations
• syncope
• sudden cardiac death

What ECG changes are seen in Arrhythmogenic right ventricular cardiomyopathy?

ECG abnormalities in V1-3, typically T wave inversion. An epsilon wave is found in about 50% of those with ARV

What echo changes are seen in Arrhythmogenic right ventricular cardiomyopathy?

echo changes are often subtle in the early stages but may show an enlarged, hypokinetic right ventricle with a thin free wall

What is the management of Arrhythmogenic right ventricular cardiomyopathy?

• drugs: sotalol is the most widely used antiarrhythmic
• catheter ablation to prevent ventricular tachycardia
• implantable cardioverter-defibrillator