[Right ventricular dysplasia (right ventricular cardiomyopathy). Clinical aspects, diagnosis and course in 15 patients from the Zurich area].

Fifteen patients (8 men, 7 women) with right ventricular dysplasia (RVS) from the greater Zürich area are described. Two thirds of these patients were younger than 30 years at first manifestation. 12 presented with ventricular tachycardia of left bundle branch block type. Other forms of arrhythmia (supraventricular tachycardia, sinus node dysfunction) or condition disturbances were documented in 9 subjects. 6 patients had additional symptoms of congestive heart failure; in 3 of them this was the only symptom. 12-lead ECG at rest showed precordial T-negativity (1 pacemaker ECG not interpretable) in 14/15 subjects. Signal averaged ECG revealed late potentials as well as spectral turbulence in the Y or Z leads. Echocardiography yielded typical local abnormalities in the whole study cohort and all but 2 patients showed decreased right ventricular ejection fraction and right heart dilatation. Moreover, left ventricular ejection fraction was concomitantly impaired in 6 subjects. 4 of these 6 individuals suffered from further impairment of left ventricular function within a time period of 19 to 47 months. Recurrent ventricular tachycardia was documented in 11 patients. 2 subjects underwent heart transplantation because of severe progressive right heart failure. One subject died shortly after diagnosis and autopsy confirmed nearly total absence of right ventricular myocardium. This extreme form of right ventricular dysplasia corresponds to Uhl's anomaly. Thus, recurrent ventricular arrhythmias, in particular ventricular tachycardia of left bundle branch block type, together with precordial T-negativity without signs of ischemic heart disease, is highly suggestive of RVD. Echocardiography allows reliable diagnosis. Concomitant left ventricular involvement is frequent. Considering that the etiology and pathogenesis of this disease are unknown, the term right ventricular cardiomyopathy, rather than right ventricular dysplasia, seems more accurate.