Arrhythmogenic right ventricular dysplasia: a generalized cardiomyopathy?

Arrhythmogenic right ventricular dysplasia (ARVD) is a recently described entity characterized by right ventricular myopathic changes and right ventricular tachycardia.The presence or extent of left ventricular dysfunction in ARVD is not known.We assessed right ventricular and left ventricular function and size in six patients with ARVD by echocardiography and radionuclide angiocardiography done in patients at rest and during exercise.All patients had recurrent ventricular tachycardia of left bundle branch block morphology, and right ventricular origin of the ventricular tachycardia was confirmed by endocardial mapping in four patients.The results were compared with those of 10 normal subjects and five patients with Wolff-Parkinson-White syndrome taking amiodarone.The latter group was a control group, since we did not withhold amiodarone therapy in four patients with ARVD.Mean ( + SD) right ventricular ejection fraction (EF) in patients with ARVD was 25 + 1 1% at rest and 26 12% during exercise.In normal subjects right ventricular EF was 51 + 4% at rest and 59 6% during exercise (p .05).In normal subjects, left ventricular EF was 61 + 4% at rest and 72 5% during exercise (p .05) to those of normal subjects.We conclude that right ventricular dysfunction predominates in patients with ARVD but latent left ventricular dysfunction is present more often than is commonly recognized.These findings may have important diagnostic and therapeutic implications.Circulation 68, No. 2, 251-257, 1983.RIGHT VENTRICULAR DYSPLASIA is a pathologic condition in which the right ventricular myocardium is totally or partially replaced by fatty and fibrous tissue.'I The clinical expression of this condition may