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IPCCC ICD-11 Congenital Heart Atlas

You are here: Atrioventricular septal defect with communication at atrial level and restrictive communication at ventricular level Common atrioventricular junction with atrioventricular septal defect Common atrioventricular junction Congenital anomaly of an atrioventricular valve or atrioventricular septum Structural developmental anomaly of heart or great vessels

IPCCC Term Atrioventricular septal defect with communication at atrial level and restrictive communication at ventricular level
IPCCC Code 06.06.10
ICD-11 MMS LA87.23
ICD-11 Code 687321516

Definition

A congenital cardiac malformation that is a variant of an atrioventricular septal defect (atrioventricular canal defect) with an interatrial communicationimmediately above the atrioventricular valve, and a restrictive interventricular communication immediately below the atrioventricular valve.

Synonyms/Abbreviations

Intermediate atrioventricular septal defect with atrial and ventricular components and separate atrioventricular valvar orifices; Atrioventricular canal defect associated with a restrictive ventricular septal defect; Atrioventricular septal defect with atrial shunting and restrictive ventricular shunting; Intermediate atrioventricular canal defect with atrial and ventricular components and separate atrioventricular valve; Transitional atrioventricular canal defect with atrial and ventricular components and separate atrioventricular valve; Transitional atrioventricular septal defect with atrial and ventricular components and separate atrioventricular valves

STS-EACTS-derived IPCCC term

AVC (AVSD), Intermediate (transitional) (Primum ASD + restrictive VSD + 2 separate atrioventricular valve orifices + common atrioventricular junction

EPCC-derived IPCCC term

Atrioventricular septal defect (atrioventricular canal defect) with communication at atrial level and restrictive communication at ventricular level (intermediate atrioventricular septal defect)

ICD-11 MMS code or crossmap

LA87.42

ICD-10 MMS code or crossmap

Q21.2**: This Foundation term is not in ICD-10. If using ICD-10 to code, crossmap to higher order term “Atrioventricular septal defect”, ICD-10 code Q21.2

Parent

Common atrioventricular junction with atrioventricular septal defect

Siblings

Coding Notes

This term is used to identify hearts with a restrictive interventricular communication. If there is a single atrioventricular valvar orifice this term should still be used. If theventricular component of the atrioventricular septal defectis unrestrictive (no interventricular pressure gradient), this malformation should not be coded as "Atrioventricular septal defect (atrioventricular canal defect) with communication at atrial level and restrictive communication at ventricular level" and instead the term "Atrioventricular septal defect (atrioventricular canal defect) with communication at atrial level and unrestrictive communication at ventricular level" should be used.