RIGHT-VENTRICULAR DYSPLASIA (RIGHT-VENTRI CULAR CARDIOMYOPATHY) - SYMPTOMATOLOGY, DIAGNOSIS AND MONITORING OF 15 CASES IN ZURICH

Fifteen patients (8 men, 7 women) with right ventricular dysplasia (RV S) from the greater Zurich area are described. Two thirds of these pat ients were younger than 30 years at first manifestation. 12 presented with ventricular tachycardia of left bundle branch block type. Other f orms of arrhythmia (supraventricular tachycardia, sinus node dysfuncti on) or condition disturbances were documented in 9 subjects. 6 patient s had additional symptoms of congestive heart failure; in 3 of them th is was the only symptom. 12-lead ECG at rest showed precordial T-negat ivities (1 pacemaker ECG not interpretable) in 14/15 subjects. Signal averaged ECG revealed late potentials as well as spectral turbulence i n the Y or Z leads. Echocardiography yielded typical local abnormaliti es in the whole study cohort and all but 2 patients showed decreased r ight ventricular ejection fraction and right heart dilatation. Moreove r, 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. Re current ventricular tachycardia was documented in 11 patients. 2 subje cts underwent heart transplantation because of severe progressive righ t heart failure. One subject died shortly after diagnosis and autopsy confirmed nearly total absence of right ventricular myocardium. This e xtreme form of right ventricular dysplasia corresponds to Uhl's anomal y. Thus, recurrent ventricular arrhythmias, in particular ventricular tachycardia of left bundle branch block type, together with precordial T-negativities without signs of ischemic heart disease, is highly sug gestive of RVD. Echocardiography allows reliable diagnosis. Concomitan t left ventricular involvement is frequent. Considering that the etiol ogy and pathogenesis of this disease are unknown, the term right ventr icular cardiomyopathy, rather than right ventricular dysplasia, seems more accurate.