DR Medhat MRCP

Arrhythmogenic right ventricular cardiomyopathy
- Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a form of inherited
cardiovascular disease which may present with syncope or sudden cardiac death.
- It is generally regarded as the second most common cause of sudden cardiac death
in the young after HOCM.
• Pathophysiology
Inherited in an autosomal dominant pattern with variable expression
The right ventricular myocardium is replaced by fibrofatty tissue
• Presentation
Palpitations
Syncope
Sudden cardiac death
• Investigation
ECG abnormalities :
‣ Typically T wave inversion in V1-3.
‣ An epsilon wave is found in about 50% of those with ARV - this is best
described as a terminal notch in the QRS complex
Echo changes : Enlarged, hypokinetic right ventricle with a thin free wall.
MRI : is useful to show fibrofatty tissue
• Management
Drugs: Sotalol is the most widely used antiarrhythmic
Catheter ablation to prevent ventricular tachycardia
Implantable cardioverter-defibrillator (ICD)
Naxos disease
- An autosomal recessive variant of ARVC
- A triad of ARVC
Palmoplantar keratosis
Woolly hair.
67