Pulmonary atresia-intact ventricular septum syndrome
MONDO:0009931Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare form of cyanotic congenital heart malformation characterized by severe cyanosis and tachypnea. PA-IVS presents significant morphologic diversity: at the end of the spectrum are patients with a mildly hypoplastic and tripartite right ventricle (RV) and mild tricuspid valve (TV) hypoplasia, and at the other end are patients with severe RV and TV hypoplasia, often with RV-dependent coronary circulation.
Also known as: pulmonary valve atresia with intact ventricular septum, pulmonary atresia with intact ventricular septum
6 clinical trials for this condition and its sub-types, 4 tagged with Pulmonary atresia-intact ventricular septum syndrome itself.
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New app aims to ease Parents' stress over Baby's heart surgery decision
Symptom relief OngoingThis study tests whether a web-based decision aid app can reduce stress and improve decision-making for parents whose baby is diagnosed with a life-threatening congenital heart defect. 135 parents are randomly assigned to use the app alone or with an added values clarification ex…
Sponsor: University of Utah • Aim: Symptom relief
Last updated Jun 27, 2026 09:05 UTC
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Which heart repair lasts longer? a rare defect study seeks answers
Knowledge-focused OngoingThis observational study compares the long-term health of people born with a rare heart defect called pulmonary atresia with intact ventricular septum (PA-IVS). Researchers will look at two groups: those who had surgery to create a single pumping chamber (univentricular circulati…
Sponsor: Vastra Gotaland Region • Aim: Knowledge-focused
Last updated Aug 30, 2026 00:00 UTC