Dominant right ventricular dilated cardiomyopathy: clinical, echocardiographic and haemodynamic profile.

Ten patients of dominant right ventricular dilated cardiomyopathy (RVDCM) seen over a period of three years in this institution are described. There were six males and four females in the age range of 6.50 years. Presenting features were right ventricular failure (7), paroxysmal supraventricular tachycardia (2), and one patient was detected on routine chest skiagram. All patients had characteristic electrocardiographic and radiologic features. 2D-echocardiographic examination revealed enormous dilatation of right atrium, ventricular cavity and outflow tract, interatrium and ventricular septum concave to the right, pre-systolic pulmonary valve opening, small left atrium and ventricle. Pulsed doppler echocardiogram revealed varying degree of tricuspid incompetence in all. Four patients underwent haemodynamic and cineangiographic studies showing left ventricular involvement in one. The patients have been followed up on digitalis, diuretics and quinidine with variable response to treatment. One patient died of uraemia, and one female patient recovered completely following delivery. Dominant RVDCM is a distinct clinical entity with typical features and with typical features and ill-defined natural history.