Cardiac arrest secondary to arrhythmogenic right ventricular cardiomyopathy in an adolescent male

• Published in: Indian pacing and electrophysiology journal Vol. 22; no. 5; pp. 241 - 244
• Main Authors: Jan, Meryam, Shillingford, Michael S., Turbendian, Harma K., Ferns, Sunita J.
• Format: Journal Article
• Language: English
• Published: Elsevier B.V 01.09.2022; Elsevier
• Subjects: Case Report
• ISSN: 0972-6292; 0972-6292
• DOI: 10.1016/j.ipej.2022.06.001

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare, genetically-inherited cardiomyopathy that may be fatal. We present the case of a 17 year old male who presented after a witnessed cardiac arrest with indeterminate echocardiogram and electrocardiogram (ECG) findings for a specific etiology. Genetic testing revealed a mutation in the PKP2 and DSC2 genes, consistent with ARVC. This report outlines the presentation of ARVC as an aborted sudden cardiac death episode in a previously asymptomatic teenager, investigations for ARVC and highlights the importance of adequate cardiopulmonary resuscitation in the overall prognosis. Implantable cardiac defibrillator (ICD) placement for secondary prevention is necessary. •The first presentation of ARVC in the young can be a catastrophic arrhythmia.•Initial ECGs and echocardiogram abnormalities can be subtle.•Genetic screening for first degree relatives is important.•First responder CPR plays an important role in the long-term prognosis.