Congenital pulmonary veins atresia or stenosis
MONDO:0017864Congenital pulmonary vein (PV) stenosis or atresia is a rare progressive life-threatening great vessels anomaly characterized by narrowing and obstruction of one or more normally positioned PV at their junction with the left atrium, that usually presents during early infancy with dyspnea, tachypnea, and repeated pulmonary infections, and eventually, when all PV of one lung are affected, results in pulmonary hypertension (PH) and consecutive pulmonary arterial hypertension (PAH). It may manifest as an isolated lesion or associated with other cardiac defects such as congenital pulmonary venous return anomaly and septal defects.
Also known as: pulmonary vein stenosis, pulmonary veins stenosis
5 clinical trials for this condition and its sub-types.
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Broader categories
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Rare lung vein condition targeted by novel transplant trial
Disease control TerminatedThis study planned to test a new surgical approach for people with pulmonary vein stenosis, a condition where the veins carrying blood from the lungs to the heart become narrowed. The procedure involved transplanting a donor's left atrium and pulmonary veins, followed by lifelong…
Phase: NA • Sponsor: Boston Children's Hospital • Aim: Disease control
Last updated Jun 27, 2026 12:38 UTC
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New heart solution tested in Kids' surgery: could it offer better protection?
Disease control TerminatedThis study compares two solutions, Custodiol and Custodiol-N, used to protect the heart during surgery for children with congenital heart defects. About 100 children will receive one of the two solutions while on a heart-lung machine. The goal is to see which one is safer and bet…
Phase: PHASE2 • Sponsor: Dr. F. Köhler Chemie GmbH • Aim: Disease control
Last updated Jun 27, 2026 09:03 UTC
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New study aims to predict lung vein disease in kids before it worsens
Knowledge-focused OngoingThis study at Boston Children's Hospital is testing whether a thorough catheterization assessment can predict the outcome of pulmonary vein stenosis in children. Eighty children with known or suspected vein narrowing will undergo four tests during catheterization: angiography, ul…
Phase: NA • Sponsor: Boston Children's Hospital • Aim: Knowledge-focused
Last updated Jun 27, 2026 09:00 UTC