ARRHYTHMOGENIC RIGHT-VENTRICULAR DYSPLASIA IN IDENTICAL-TWINS

A rrhythmogenic right ventricular dysplasia (ARVD) is increasingly recognized as representing one of the most frequent causes of sudden cardiac death in the young.1 Refined diagnostic tools such as echocardiography or magnetic resonance imaging allow accurate noninvasive identification of patients with ARVD. These patients typically have symptomatic ventricular arrhythmias, especially exercise-induced sustained ventricular tachycardias with a left bundle branch block pattern in the surface electrocardiogram.1 The characteristic pathologic-anatomic finding of ARVD is fibrolipomatous replacement of the myocardium, which is predominantly confined to the right ventricle. In marked cases the right ventricle is highly thin-walled, and sometimes only the contractions of the hypertrophied right atrium maintain pulmonary circulation. The latter condition is also known as Uhl's anomaly, and several investigators consider this disorder as an extreme form of ARVD.2 Although the etiology of ARVD is still a matter of ongoing debate, familial occurrence has been ascertained by several investigators,3 which points to a genetic background of the disease. The present case report supports this hypothesis, describing 2 identical adult male twins with ARVD and with a different clinical course. © 1994.