A case of arrhythmogenic right ventricular cardiomyopathy/dysplasia involving the left ventricle / 대한내과학회지

ABSTRACT

Arrhythmogenic right ventricular cardiomyopathy (ARVC)/dysplasia is characterized by fibro-fatty replacement of the right ventricular myocardium. A 19-year-old soldier was admitted with sudden cardiac arrest that presented as ventricular fibrillation requiring cardiopulmonary resuscitation. His electrocardiogram (ECG) showed normal sinus rhythm with inverted T waves from leads V1 to V4 and isolated premature ventricular beats with a left bundle branch block (LBBB) pattern. Echocardiography showed that the right ventricle was enlarged with apical trabecular formation. The coronary angiogram was normal. Cardiovascular magnetic resonance imaging (MRI) showed thinning and fatty substitution of both the right and left ventricular free walls. Ventricular tachycardia (LBBB pattern) occurred frequently despite anti-arrhythmic drug treatment. His drug-resistant ventricular tachycardia was treated with an implanted cardiac defibrillator. Left ventricle involvement in ARVC is less common and more severe. Cardiac MRI is considered the best imaging technique for evaluating the right ventricle and diagnosing ARVC.