Scimitar syndrome
ORPHA:185· ICD-10 Q26.8
Definition
A rare congenital malformation characterized by a combination of cardiopulmonary anomalies including partial anomalous pulmonary venous connection of the right lung to the inferior caval vein leading to the creation of a left-to-right shunt, and varying degrees of right lung hypoplasia, along with a systemic arterial supply to the right lung.
- Prevalence
- Unknown
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Antenatal, Childhood, Infancy, Neonatal