Methods: Twelve patients (8 g irls, 4 b oys; m ean age: 4.0±4.8 years; range 1 month to 16 years) with a mean weight of 16.7±21.8 kg who were diagnosed with Scimitar syndrome in our clinic between January 2003 and December 2011 were included. Clinical findings of the patients, physical examination, telecardiography, echocardiography, cardiac catheterization, and surgical findings and follow-up data were retrospectively reviewed.

Results: Of 12 patients, five (42%) were considered with infantile type and seven (58%) with child/adult type Scimitar syndrome. All patients of infantile type presented with tachypnea and cardiac failure findings, while one patient with child/adult type had a history of recurrent lower respiratory tract infection. Additional cardiac defects were atrial septal defect in seven, coarctation in one, patent ductus arteriosus in two, and ventricular septal defect with pulmonary atresia in one patient. Bilateral Scimitary syndrome was present in one patient. All infantile type patients had severe pulmonary hypertension, while only one of the child/adult type patients had mild pulmonary hypertension. Six of nine patients with aortopulmonary collateral artery underwent transcatheter closure of the artery. Surgery was performed in 10 patients (83%). One patient with infantile type in the early postoperative period and another patient with bilateral Scimitar syndrome who was not operated during follow-up died due to pulmonary infection.

Conclusion: Scimitar syndrome is a rare syndrome which may present with different clinical presentations. Patients with child/ adult type disease are often asymptomatic, however, may present with recurrent pulmonary infections. Although aortopulmonary collateral occlusion may provide symptomatic relief, surgery is indicated in patients with obstructed Scimitar vein and additional cardiac anomalies, in particular.