ARVD is caused by genetic defects of the desmosomes parts of myocardium. The disease is a type of nonischemic cardiomyopathy that involves the right ventricle. It is characterized by enlarged hypokinetic areas involving the free wall of the right ventricle, with fibrofatty replacement of the right ventricular myocardium, with associated arrhythmias originating in the right ventricle.

Clinical Presentation ; Around 30 yrs of age. Men and women appear to be equally affected. The most common symptoms due to an arrhythmia. Symptoms are usually exercise-related.

ECG ; RBBB frequently. The epsilon wave about 50%. VT shows a LBBB morphology with an inferior axis.

Echo ; Enlarged, hypokinetic right ventricle with a paper-thin RV free wall.

Signal-averaged electrocardiography (SAECG) ; Detection of late potentials and epsilon waves.

MRI ; Fatty infiltration & extreme thinning and akinesis of the RV free wall.

Genetic testing ; Autosomal dominant & about 40% of ARVD patients have a mutation identified in one of several desmosome's genes.

Management ; Beta blocker → Sotalol → Amiodarone → ICD → Catheter ablation

 

Compilation by Dr. Samad Ali Moradi, According to author's work experience and guidelines.