The Role of Advanced Imaging in the Diagnosis and Management of Scimitar Syndrome in Pediatric Patients

This imaging review highlights the spectrum of Scimitar Syndrome (SS) as seen on cardiac computed tomography (CT) and cardiovascular magnetic resonance (CMR), illustrating its variants and linking imaging findings with clinical presentation and management strategies. SS is a rare congenital cardiopulmonary anomaly characterized by partial or total right anomalous pulmonary venous drainage to the hepatic vein or inferior vena cava (IVC), hypoplastic right lung and pulmonary artery, abdominal aortopulmonary collateral(s) to the right lower lobe and other cardiac and bronchial malformations. Management options range from surgical lobe resection, rerouting of the anomalous pulmonary vein to the left atrium, catheter-based embolization of aorto-pulmonary collaterals, and intracardiac shunt device closure, to conservative observation. While surgical outcomes are generally favorable, postoperative complications can be substantial, and the indications for surgery—especially in asymptomatic patients—remain unclear and should be individualized. A multimodal imaging approach is essential for accurate diagnosis, hemodynamic assessment, and follow-up, enabling tailored treatment planning. This manuscript presents role of cardiac CT and CMR using representative imaging examples, highlighting the diagnostic value and discussing implications of the findings for clinical management and longitudinal follow-up. • Scimitar syndrome shows a broad spectrum of anatomical variants. • CT and CMR provide complementary anatomical and functional data. • CT enables rapid, high-resolution anatomical assessment in infants. • CMR enables longitudinal follow-up and hemodynamic assessment.