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IPCCC ICD-11 Congenital Heart Atlas

You are here: Scimitar syndrome Partial anomalous pulmonary venous connection of Scimitar type Anomalous pulmonary venous connection Congenital anomaly of pulmonary vein Congenital anomaly of mediastinal vein Structural developmental anomaly of heart or great vessels

IPCCC Term Scimitar syndrome
IPCCC Code 03.02.23
ICD-11 MMS LA86.22
ICD-11 Code 1321054364

Definition

A congenital cardiopulmonary malformation with “partial anomalous pulmonary venous connection of Scimitar type” and one or more of the following: hypoplasia of the right lung with bronchial anomalies, dextrocardia, hypoplasia of the right pulmonary artery, lobar lung sequestration, and anomalous systemic arterial supply to the lower lobe of the right lung directly from the aorta or its main branches.

Synonyms/Abbreviations

Pulmonary venolobar syndrome

STS-EACTS-derived IPCCC term

Partial anomalous pulmonary venous connection (PAPVC), Scimitar type with Scimitar syndrome

EPCC-derived IPCCC term

Scimitar syndrome

ICD-11 MMS code or crossmap

LA86.22

ICD-10 MMS code or crossmap

Q26.8**: This Foundation term is not in ICD-10. If using ICD-10 to code, crossmap to "Other congenital malformation of great veins" with code Q26.8 which includes "Scimitar sybdrome"

Parent

Partial anomalous pulmonary venous connection of Scimitar type

Coding Notes

None

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