[Isolated hypoplasia of the right ventricle. Study of 3 cases]

Right ventricular hypoplasia without additional congenital heart disease is an uncommon abnormality. It is characterized by partial absence of right ventricular inflow tract, both tricuspid and pulmonary valves being normally shaped. We report three cases; a newborn, an infant and a twelve years old boy. All of them had cyanosis and right atrial enlargement and both of the younger patients had electrocardiographic signs suggesting right ventricular hypoplasia. Right atrial mean pressure were raised. A differential diagnosis should be stablished with all sorts of right ventricular hypoplasia with cyanosis and with restrictive myocardiopathyes. Surgery for symptomatic patient should be palliative during first months of life and corrective from age one year onwards.