pulmonary atresia with ventricular septal defect
Findings
No curated finding names pulmonary atresia with ventricular septal defect yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Pulmonary atresia with ventricular septal defect (PA-VSD) is a rare cyanotic congenital heart malformation characterized by underdevelopment of the right ventricular outflow tract and atresia of the pulmonary valve, ventricular septal defect (VSD) and pulmonary collateral vessels. Clinical features depend on the anatomic variability of the lesion and patients may be minimally symptomatic, severely cyanotic or may develop congestive heart failure. PA-VSD may represent a severe form of Tetralogy of Fallot.
Definition from the Mondo Disease Ontology (MONDO:0008343), read 2026-09-29. CC BY 4.0.
Where it sits
Other names
1 name
Resolves to: pulmonary atresia with ventricular septal defect
- Also called
- pulmonary valve atresia with ventricular septal defect