Long-Term Outcomes and Imaging Characteristics of Patients with Scimitar Syndrome

Scimitar syndrome is a rare congenital cardiopulmonary anomaly with marked anatomical and clinical heterogeneity, and long-term outcome data remain limited. We reviewed our single-center experience over a 33-year period. Patients evaluated between 1992 and 2025 were retrospectively analyzed. A total of 104 patients were included, with female predominance (63, 60.6%). The median age at first presentation was 0.4 years (IQR 0.0-16.7; range 1 day-68 years) with 59 patients (56.7%) presenting during infancy. At last follow-up, the median age was 18.5 years (IQR 8.7-30.6; range 60 days-70 years), with a median follow-up duration of 9.5 years (IQR 3.7-16.1). Dextrocardia was observed in 76 patients (73.1%). The most common associated defect was atrial septal defect (37 patients, 35.6%), while 23 patients (22.1%) had no additional cardiac defects. Respiratory manifestations predominated at presentation and follow-up. However, 18 patients (17.3%) were asymptomatic at diagnosis and 44 (42.3%) at follow-up. Partial anomalous pulmonary venous drainage was present in 87 patients (83.6%), and aorto-pulmonary collaterals in 70 (67.3%). Cardiac catheterization was performed in 78 patients (75.0%), and 47 (45.2%) underwent surgery. At last documented follow-up, 101 of 104 patients (97.1%) were alive, with three deaths occurring during follow-up.