Key result
TEE identifies rare congenital quadricuspid pulmonary valve with mixed dysfunction and pulmonary artery aneurysm.
Why the study?
The quadricuspid pulmonary valve is a rare congenital anomaly with fewer than 300 reported cases, and unique presentations with double valvular lesions and poststenotic dilatation have not been previously described.
Case Report (n=1)
QPV with mixed lesions may cause PA dilatation; leaves open natural history and intervention thresholds in adults.
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.
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Olivares-Reyes et al. (2012) conducted a case report in Congenital quadricuspid pulmonary valve with double valvular lesions (n=1). Transesophageal echocardiography (TEE) was evaluated. Transesophageal echocardiography diagnosed a rare congenital quadricuspid pulmonary valve with stenosis, insufficiency, and aneurysmatic dilatation of the pulmonary artery.
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