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

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.