ARVD is a rare form of cardiomyopathy, where the heart muscle of the right ventricle is replaced by fat and/or fibrous tissue, there are forms with biventricular muscle affected. Patients often have abnormal heart rhythms, which can increase the risk of sudden death (SD). Echocardiography is the first-line investigation, and may demonstrate a dilated, hypokinetic right ventricle with prominent apical trabeculae and dilatation of the RV outflow tract, but cardiac MRI is the gold standard tool for diagnosis and could detect the fatty or fibrous-fatty deposits in the myocardium of right ventricle heart muscle.

ARVD is associated with characteristic ECG abnormalities:

• Epsilon wave (most specific finding, seen in 30% of patients)(see image below)

• T wave inversion in V1-3 (85% of patients)
• Prolonged S-wave upstroke of 55ms in V1-3 (95% of patients)
• Localised QRS widening of 110ms in V1-3
• Paroxysmal episodes of ventricular tachycardia with LBBB morphology

Treatment is usually directed at controlling the patient’s abnormal heart rhythms and managing their heart failure. The primary goal of treatment is preventing continued ventricular arrhythmias and/or SD usually treated with medical therapy initially, if not response with radiofrequency ablation and an implantable defibrillator (ICD) usually is needed if high risk of SD. Patients who are thought to be severe HF, after ICD, heart transplant has to be considered.