Congenital quadricuspid pulmonary valve in an adult patient with double valvular lesions and poststenotic dilatation of the trunk and the left branch of the pulmonary artery: a case presentation and review of the literature.

The quadricuspid pulmonary valve (QPV) is a rare congenital anomaly reported in the general population. There are less than 300 reported cases in the literature to date. It has been found in one in 400 to one in 2000 autopsies. We describe here the case of a 47-year-old patient who presents with a QPV with double valvular lesions (stenosis and insufficiency), causing a poststenotic dilatation of the trunk and the left branch of the pulmonary artery (PA). The diagnosis was made by transesophageal echocardiography (TEE) and confirmed by tomographic angiography (computed tomography). The English as well as the Spanish literature were reviewed. To the best of our knowledge, this is the first case of: (1) congenital QPV with double valvular lesions (stenosis and insufficiency), complicated with (2) aneurysmatic dilatation of the PA trunk as well as left branch, and (3) diagnosed by a TEE.