Pediatric scimitar syndrome with an intrahepatic venous-venous shunt: A rare imaging case report.

Authors

Marjeta Tanka, Albert Koja, Numila Kuneshka, Ermela Celaj, Fjorda Tuka, Erjona Abazaj, Kostandin Tanka, Vasiana Tanka

Published in

Radiology case reports. Volume 21. Issue 10. Pages 5027-5031. Epub Jul 30, 2026.

Abstract

Scimitar syndrome (pulmonary veno-lobular syndrome) is a rare congenital anomaly characterized by abnormal pulmonary venous drainage. The coexistence of this syndrome with a veno-venous shunt connecting an accessory hepatic vein to the main hepatic vein is exceptionally rare; to our knowledge, this is the first such case reported in an asymptomatic patient. A pediatric patient was referred to cardiology following the incidental discovery of a heart murmur during a routine exam. While the patient was asymptomatic, an echocardiogram revealed mitral valve regurgitation and right-sided heart dilation. A chest X-ray showed the classic "scimitar" sign (a curved paracardiac opacity). Subsequent CT angiography confirmed that the entire right lung drained into the inferior vena cava and identified an incidental veno-venous shunt between the inferior right accessory hepatic vein and the main hepatic vein. This report highlights a unique variant of Scimitar syndrome involving an unusual hepatic vascular shunt. It underscores the critical role of advanced imaging in detecting latent vascular anomalies, even when clinical symptoms are absent.

PMID:
42571424
Bibliographic data and abstract were imported from PubMed on 09 Aug 2026.

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