Heart failure in arrhythmogenic right ventricular dysplasia-cardiomyopathy

Sudden arrhythmic death and heart failure are essential factors influencing the prognosis of arrhythmogenic right ventricular dysplasia-cardiomyopathy. Heart failure is a rare, but often lethargic event although little is known about morphology, time course and non-invasive predictors.

In a retrospective study of a consecutive cohort of 121 patients with ARVD over a follow-up period of up to 12 years morphological features of heart failure, time course from the initial diagnosis and standard 12-lead ECG as a non-invasive predictor of developing heart failure were analysed.

Heart failure occurred in 13 patients (11%) with isolated right ventricular dilatation and loss of function in 10 cases (77%) and biventricular failure in three cases (23%). Patients developed NYHA class IV in four cases, class III-IV in two cases and class II in seven cases in 4-8 years. In standard ECG of 12 patients (92%) complete right bundle branch block was present at the time of initial diagnosis (n=6) or in a time interval of 4 years (n=6). Morphological distinction of isolated right and biventricular heart failure could be achieved not only by imaging techniques such as echocardiography or cardioangiography, but also by standard ECG with right atrial hypertrophy and an increased mean precordial QRS dispersion of 47.1+/-18.9 ms in cases of right heart failure and biatrial hypertrophy and a reduced precordial QRS dispersion of 33.0+/-23.1 ms in cases of biventricular heart failure.

Heart failure in ARVD consists of isolated right ventricular and biventricular dilatation and pump failure in a time course of 4-8 years after developing complete right bundle branch block as a strong non-invasive predictor from standard 12-lead ECG.