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IPCCC ICD-11 Congenital Heart Atlas

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
IPCCC Code 01.01.07
ICD-11 MMS LA8A.10
ICD-11 Code 131289265


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

Pulmonary atresia-IVS

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


Coding Notes

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.