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.

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Publication Details

Journal
International Journal of Cardiology Congenital Heart Disease
Published
2026-04-17
DOI
https://doi.org/10.1016/j.ijcchd.2026.100678
Primary Topic
Fetal and Pediatric Neurological Disorders
Type
article
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article

The role of advanced imaging in the diagnosis and management of scimitar syndrome in pediatric patients

Simon Padley, Inga Voges, Thomas R. Semple, Cemil İzgi et al.
International Journal of Cardiology Congenital Heart Disease
Fetal and Pediatric Neurological Disorders
article

The role of advanced imaging in the diagnosis and management of scimitar syndrome in pediatric patients

Simon Padley, Inga Voges, Thomas R. Semple, Cemil İzgi, Sylvia Krupičková, Dudley J. Pennell, Hicran Gül Emral, S Y Ho, Thao V.N. Nguyen, Raad Mohiaddin, Rigby L. Michael, Piers E.J. Daubeney, Edward Nicol
article en

Abstract

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.

International Journal of Cardiology Congenital Heart DiseaseVol. 24
King's College London (GB), Guy's and St Thomas' NHS Foundation Trust (GB), Royal Brompton Hospital (GB), Children's Hospital 2 (VN), University Hospital Schleswig-Holstein (DE), German Centre for Cardiovascular Research (DE), Children's Hospital 1 (VN), Imperial College London (GB), University of Lübeck (DE)
Openalex Percentile: Top 25%
Fetal and Pediatric Neurological Disorders
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