Scimitar syndrome

Description

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.

Index terms

Scimitar syndrome
pulmonary venolobar syndrome

Exclusions from above levels

http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

LA86.20 – Total anomalous pulmonary venous connection
LA86.21 – Partial anomalous pulmonary venous connection
LA86.2Y – Other specified anomalous pulmonary venous connection
LA86.2Z – Anomalous pulmonary venous connection, unspecified

Foundation entity

Scimitar syndrome – http://id.who.int/icd/entity/1321054364

Part of: LA86.2: Anomalous pulmonary venous connection › LA86: Congenital anomaly of mediastinal vein › 20: Developmental anomalies