You are here: Pulmonary atresia with intact ventricular septum ⇗ Congenital pulmonary atresia ⇗ Congenital anomaly of a ventriculo-arterial valve or adjacent regions ⇗ Structural developmental anomaly of heart or great vessels
|IPCCC Term||Pulmonary atresia with intact ventricular septum|
A congenital cardiovascular malformation in which there are normally aligned great arteries, no opening between the morphologically right ventricle and the pulmonary trunk, and no ventricular level communication.
STS-EACTS-derived IPCCC term
EPCC-derived IPCCC term
Pulmonary atresia with intact ventricular septum
ICD-11 MMS code or crossmap
ICD-10 MMS code or crossmap
Q22.0**: This Foundation term is not in ICD-10. If coding using ICD-10, crossmap to "Congenital pulmonary valve atresia" with code Q22.0
Congenital pulmonary atresia
Pulmonary atresia with intact ventricular septum is a duct-dependent congenital malformation that forms a spectrum of lesions including atresia of the pulmonary valve, a varying degree of right ventricle and tricuspid valve hypoplasia, and anomalies of the coronary circulation. A right ventricular dependent coronary artery circulation is present when coronary artery fistulas are associated with a proximal coronary artery stenosis. Associated Ebstein anomaly of the tricuspid valve can be present.