Congenital absence of the pulmonary valve leaflets associated with complete transposition of the great arteries.

We describe the clinical and surgical findings of a patient with absence of the pulmonary valve leaflets and complete transposition of the great arteries. The main clinical features were a loud, single second heart sound, a loud pansystolic murmur over the entire precordial area, but no diastolic murmurs were heard. The electrocardiogram suggested right ventricular hypertrophy and right bundle branch block. The roentgenographic study showed cardiomegaly with dilatation of both branches of the pulmonary artery. Cardiac catheterization and angiocardiography showed systemic arterial desaturation, discordant ventriculo-arterial connections and absence of the pulmonary valve leaflets. The patient underwent surgical correction by the Rastelli technique with a favorable clinical course.