Hypoplastic right heart syndrome
MONDO:0020291Hypoplastic right-heart syndrome (HRHS) is a rare, cyanotic congenital heart malformation caused by underdevelopment of the right-sided heart structures (tricuspid valve, RV, pulmonary valve, and pulmonary artery) commonly associated with an atrial septal defect, ostium secundum type. Pulmonary blood flow is diminished and right-to-left shunting occurs at the atrial level, leading to dyspnea, fatigue, atrial arrhythmias, right-sided heart failure, hypoxemia, repeated miscarriages that were mostly due to hypoxemia and cyanosis. Two subtypes of HRHS have been characterized: pulmonary atresia-intact ventricular septum and right ventricular hypoplasia.
Also known as: right hypoplastic heart syndrome
5 clinical trials for this condition and its sub-types.
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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.
Phase: NA • 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