Congenital Absence of One Pulmonary Valve Cusp With Other Hypoplastic Cusps.

Congenital absence of a single pulmonary valve cusp is extremely rare. We report a case of a 38-year-old woman with a confirmed congenital absence of a single pulmonary valve cusp associated with dextrocardia. The other 2 leaflets were moderately hypoplastic, and transthoracic echocardiography showed severe pulmonary regurgitation. This combination of lesions has not been reported previously. Pulmonary valve replacement using a composite biologic valved conduit was performed with an excellent outcome.