Role of Imaging in the Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy in Presence of Congenital Heart Disease

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an underdiagnosed entity manifesting with arrhythmia, heart failure and sometimes with sudden cardiac death. Rarely, congenital heart disease (CHD) can co-exist with ARVC which creates a diagnostic challenge. The presence of ARVC in a known case of CHD changes the management plan. We describe three patients with CHD having additional features of ARVC based on proposed criteria in the last 5 years in our institution. The mean age of the patients was 43 years. Two patients had atrial septal defect and one had Ebsteins's anomaly. Cardiac magnetic resonance imaging was done in two patients which showed characteristic features of ARVC and fulfilled the criteria of diagnosis. One patient was labeled as probable ARVC. Medical therapy helped the patients with symptoms of arrhythmia and heart failure. On short-term follow-up, they were doing well. Diagnosis of ARVC in a known case of CHD requires a high index of suspicion. The proposed imaging criteria and holistic approach may help with early diagnosis and appropriate management.