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.

Lutembacher's syndrome with small atrial septal defect diagnosed by transthoracic and transesophageal echocardiography that underwent mitral valve replacement.

Lutembacher's syndrome is a rare clinical combination of congenital ostium secundum atrial septal defect associated with acquired mitral valve stenosis (usually rheumatic). This unusual cardiac entity is difficult to diagnose clinically because each lesion alters the hemodynamics and clinical characteristics of the other. The resulting clinical manifestations depend chiefly on the size of the defect, the severity of the mitral stenosis, and the compliance of the right ventricle. We present a classic case of Lutembacher's syndrome and illustrate the pitfalls and advantages of echocardiography in the correct diagnosis of the syndrome.