hypoplastic right heart syndrome
Findings
No curated finding names hypoplastic right heart syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Hypoplastic 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.
Definition from the Mondo Disease Ontology (MONDO:0020291), read 2026-09-29. CC BY 4.0.
Where it sits
Other names
1 name
Resolves to: hypoplastic right heart syndrome
- Also called
- right hypoplastic heart syndrome