Abstract :
‘Progression’ in typical arrhythmogenic right ventricular dysplasia-cardiomyopathy could be mainly demonstrated in case reports and generally implies decreasing left ventricular function; systematic follow-up data are only available on clinical features such as recurrent arrhythmias, late potentials or left ventricular ejection fraction. Due to methodological problems, continuous data on structural and functional right ventricular performance are not available. In order to get some more information about basic mechanisms of possible progression in arrhythmogenic right ventricular dysplasia-cardiomyopathy, the age-dependancy of right ventricular enddiastolic volume, right ventricular ejection fraction, number of bulges, left ventricular involvement and the form of tachycardias was analysed at a single examination after having made the diagnosis of arrhythmogenic right ventricular dysplasia-cardiomyopathy. In the whole cohort of patients (n = 66), age-dependancy of right ventricular volume could be demonstrated using linear regression analysis, chi-square-test and paired t-test (P < 0.01); global right ventricular ejection fraction and the number of right ventricular bulges did not differ significantly in younger and older patients. The same was true for mild left ventricular impairment and sustained or non-sustained ventricular tachycardias, respectively. In a follow-up, two older patients developped dramatic left ventricular dilatation and reduction of left ventricular function requiring heart transplantation. A third older patient died from isolated right heart failure. Increasing age-related right ventricular dilatation is possibly a marker of disease progression as a basis for right heart failure. Dramatic left heart involvement seems to be an event with abrupt onset in most cases due to acute or chronic myocarditis.
