Pulmonary valve agenesis and supravalvular pulmonary stenosis. Report of a case and review of literature.

: Pulmonary valve agenesis is a rare malformation, and is usually associated with a ventricular septal defect. Occasionally it may be associated also with right ventricular outflow obstruction. Children with this syndrome usually die early in infancy either of intractable heart failure or of severe respiratory distress. Rare instances of association with peripheral pulmonary stenoses have also been reported. We report the case of a three-year-old girl, asymptomatic except for failure to thrive, with pulmonary valve agenesis and coarctation of the pulmonary artery trunk.