Noninvasive recognition of the parchment right ventricle (Uhl's anomaly arrhythmogenic right ventricular dysplasia) syndrome

The case of a 53-year-old man with right heart failure, selective enlargement of the right-sided cardiac chambers, and recurrent sustained ventricular tachycardia is presented. Echocardiographic, radionuclide ventriculographic, and angiographic studies were typical of the right ventricular abnormalities in Uhl's anomaly; electrocardiographic and electrophysiologic findings were those of arrhythmogenic right ventricular dysplasia. Features of this unusual cause of cardiac failure and ventricular arrhythmias in the adult are reviewed, and compared to previous reports of both Uhl's anomaly and arrhythmogenic right ventricular dysplasia. We postulate that these two syndromes are manifestations of a single, presumably congenital, pathophysiologic process--the "parchment right ventricle" syndrome.