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IPCCC ICD-11 Congenital Heart Atlas

You are here: Double inlet right ventricle Double inlet atrioventricular connection Functionally univentricular heart Structural developmental anomaly of heart or great vessels

IPCCC Term Double inlet right ventricle
IPCCC Code 01.04.03
ICD-11 MMS LA89.0
ICD-11 Code 333513492


A congenital cardiovascular malformation with a univentricular atrioventricular connection wherein both atria connect to a morphologically right ventricle either via two separate atrioventricular valves or a common atrioventricular valve, such that all or nearly all of the total atrioventricular junctional (annular) area is committed to the right ventricular chamber.



STS-EACTS-derived IPCCC term

Single ventricle, DIRV

EPCC-derived IPCCC term

Double inlet right ventricle

ICD-11 MMS code or crossmap

LA89.0*: This Foundation term is not in ICD-11 MMS. If using MMS to code. Crossmap to higher order term "Double inlet atrioventricular connection" with code LA89.0

ICD-10 MMS code or crossmap

Q20.4**: This Foundation term is not in ICD-10. If using ICD-10 to code, crossmap to "Double inlet ventricle" with code Q20.4


Double inlet atrioventricular connection


Coding Notes


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