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IPCCC ICD-11 Congenital Heart Atlas

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

IPCCC Term Functionally univentricular heart
IPCCC Code 01.01.22
ICD-11 Code 5417233


The term “functionally univentricular heart” describes a spectrum of congenital cardiac malformations in which the ventricular mass may not readily lend itself to partitioning that commits one ventricular pump to the systemic circulation, and another to the pulmonary circulation.


Univentricular cardiopathy; Single ventricle; Univentricular heart

STS-EACTS-derived IPCCC term

Single ventricle

EPCC-derived IPCCC term

Functionally univentricular heart

ICD-11 MMS code or crossmap


ICD-10 MMS code or crossmap

Q20.9**: If coding using ICD-10, crossmap to “Congenital malformation of cardiac chambers and connections, unspecified” with code Q20.9


Structural developmental anomaly of heart or great vessels



Coding Notes

A heart may be functionally univentricular because of its anatomy or because of the lack of feasibility or lack of advisability of surgically partitioning the ventricular mass. Common lesions in this category typically include double inlet right ventricle (DIRV), double inlet left ventricle (DILV), tricuspid atresia, mitral atresia, and hypoplastic left heart syndrome. Other lesions which sometimes may be considered to be a functionally univentricular heart include complex forms of atrioventricular septal defect, double outlet right ventricle, congenitally corrected transposition, pulmonary atresia with intact ventricular septum, and other cardiovascular malformations. Specific diagnostic codes should be used whenever possible, and not the term “functionally univentricular heart”.

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