ICD-11 code : LA8A.10;
Preferred Label : Pulmonary atresia with intact ventricular septum;
ICD-11 definition : 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. Additional information: 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.;
ICD-11 synonym : pulmonary valve atresia with intact ventricular septum;
Origin ID : 131289265;
Automatic exact mappings (from CISMeF team)
- ICD-10 Mapping
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. Additional information: 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.
