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 trial aims to halve deaths in newborns with severe heart disease
Disease control Recruiting nowThis study compares performing heart surgery on newborns with complex congenital heart disease either before or after 7 days of age. Researchers will track which timing leads to fewer deaths and better recovery. The trial involves 738 babies across multiple hospitals in China.
Sponsor: Central China Fuwai Hospital of Zhengzhou University • Aim: Disease control
Last updated Jun 27, 2026 08:13 UTC
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New study aims to predict heart surgery needs before birth
Knowledge-focused Recruiting nowThis study follows 150 fetuses diagnosed with a rare heart condition called pulmonary atresia with intact ventricular septum. Researchers will track their health before and after birth to develop a model that predicts which babies will need simpler versus more complex surgeries. …
Sponsor: Hospital Universitario 12 de Octubre • Aim: Knowledge-focused
Last updated Jun 26, 2026 17:01 UTC