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IPCCC ICD-11 Congenital Heart Atlas

You are here: Hypoplastic left heart syndrome Functionally univentricular heart Structural developmental anomaly of heart or great vessels

IPCCC Term Hypoplastic left heart syndrome
IPCCC Code 01.01.09
ICD-11 MMS LA89.3
ICD-11 Code 1811800027


A spectrum of congenital cardiovascular malformations with normally aligned great arteries without a common atrioventricular junction, characterized by underdevelopment of the left heart with significant hypoplasia of the left ventricle including atresia, stenosis, or hypoplasia of the aortic or mitral valve, or both valves, and hypoplasia of the ascending aorta and aortic arch.


Hypoplasia of the left heart; hypoplasia of the left heart; HLHS; hlh - hypoplastic left heart syndrome; left heart hypoplasia syndrome

STS-EACTS-derived IPCCC term

Hypoplastic left heart syndrome (HLHS)

EPCC-derived IPCCC term

Hypoplastic left heart syndrome

ICD-11 MMS code or crossmap


ICD-10 MMS code or crossmap

Q23.4: Hypoplastic left heart syndrome


Functionally univentricular heart


Coding Notes


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