Scimitar Syndrome
Pronunciation: SIM-ih-tar
A rare form of partial anomalous pulmonary venous return where the right pulmonary veins drain into the inferior vena cava, creating a characteristic curved radiographic appearance.
Full Definition
Scimitar syndrome is a complex congenital heart defect characterized by partial anomalous pulmonary venous return of the right lung to the inferior vena cava or right atrium. The condition derives its name from the characteristic curved radiographic shadow resembling a Turkish sword (scimitar) seen on chest X-rays. Associated features often include hypoplasia of the right lung, dextroposition of the heart, and systemic arterial supply to the right lower lobe. The syndrome may present with recurrent respiratory infections, failure to thrive, or heart failure in infancy, though some cases remain asymptomatic until adulthood.
Usage
Usage note: The term 'scimitar sign' refers specifically to the radiographic appearance, not the syndrome itself.
In Context
- "The chest radiograph revealed the pathognomonic scimitar sign, confirming the diagnosis of scimitar syndrome." — Radiology report
- "Surgical repair of scimitar syndrome involved rerouting the anomalous vein to the left atrium using an intra-atrial baffle." — Operative report