Hypoplasia of right ventricle and tricuspid valve in three siblings.

A case of hypoplasia of the right ventricle and tricuspid valve is reported in the third affected sibling of a family. The condition, which has a strong familial tendency, usually is responsible for death in infancy. Exceptional patients live to adult life. The functional problem is that of inflow obstruction into the right ventricle. A transatrial right-to-left shunt is characteristic and results in cyanosis. Diminished right ventricular forces in the electrocardiogram represent an important point in differential diagnosis among patients with cyanotic congenital heart disease.

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