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 heart solution tested in Kids' surgery: could it offer better protection?
Disease control PausedThis 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 2 • Sponsor: Dr. F. Köhler Chemie GmbH • Aim: Disease control
Last updated Jun 27, 2026 09:03 UTC
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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
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Heart defect study tracks kids' outcomes 11 years after surgery
Knowledge-focused OngoingThis study looks at 237 children born with hypoplastic left heart syndrome (HLHS), a serious heart defect. Researchers compare how well the heart works at age 11 in children who received one of two different shunts during their first surgery. The goal is to understand which shunt…
Sponsor: Carelon Research • Aim: Knowledge-focused
Last updated Jun 27, 2026 07:56 UTC