Background: Scimitar syndrome is a rare congenital heart disease. To evaluate the surgical results of this malformation we have embarked on a multi-centric Italian study involving 7 different Centres.
Methods: From June 1982 to December 2007, 28 patients who had scimitar syndrome and underwent surgery were included. Primary outcome was the efficacy of repair at follow-up.
Results: Median age at surgery was 7 years (IQR 47 days-64 years). Twenty-one patients (75%) presented with symptoms including upper respiratory tract infections (n=14), recurrent pneumonia (n=10), cardiac failure (n=6), and cyanosis (n=2). Associated cardiac anomalies included: atrial septal defect (ASD) in 18 patients (64%), dextrocardia in 9 (32%), right lung hypoplasia in 9 (32%) and aorto-pulmonary collaterals in 5 (18%). Surgical repair included: intra-atrial baffle repair in 18 patients (69%)(group 1) and re-implantation of the “scimitar vein” into left atrium in 8 patients (31%)(group 2). Two patients (6.4%) had a right pneumectomy and ASD closure (group 3). One patient died in hospital (3.5%). Median follow-up time was 4 years (IQR 1.8 -9.7 years). There were 2 late deaths (7.4%). Four patients (16%) presented an occlusion of the scimitar drainage (2 in group 1, 12% and 2 in group 2, 25%) and three patients (12%) required balloon dilation/stenting for stenosis of the scimitar vein (1 in group 1, 6% and 2 in group 2, 25%).
Conclusions: The intra-atrial baffle repair is the most used surgical technique in our population and seems to offer better results in the mid-term.