The Scimitar Syndrome: An European Multicentric Study

Background: Scimitar syndrome is a rare congenital heart disease. In order to evaluate the surgical results of managing this malformation, we have embarked on a multi-centric European study involving 19 different Centres and reporting the widest so far published series in the medical literature. Methods: From January 1997 to December 2007, 68 patients with scimitar syndrome who underwent surgical correction, were included. Primary outcomes include hospital mortality and the efficacy of repair at the follow-up. Results: Median age was 1.32 years (IQR 0.28 –7.87). Forty-two patients (62%) presented with symptoms including upper respiratory tract infections (n=25), cardiac failure (n=24), recurrent pneumonia (n=9), and cyanosis (n=4). Surgical repair included: intra-atrial baffle technique in 38 patients (56%)(group 1), re-implantation of the "scimitar vein" onto left atrium in 21 patients (31%)(group 2). Nine patients (13%) underwent a right pneumectomy together with atrial septal defect closure (group 3). Four patients died in hospital (5.9%)(1 in group 1, 2.6% and 3 in group 3, 33%)(p=0.008). Postoperative complications were more frequently reported in group 3 (7/9, 78%)(p=0.02, group 3 vs. all) if compared to group 2 (14/21, 67%) and group 1 (14/38, 37%)(p=0.02, group 2 vs. group and related to the presence of preoperative congestive heart failure (OR=13). Median follow-up time was 4.5 year (IQR 1.6 – 8.3). Nine of 58 patients who survived the corrective procedure (15.5%) showed a stenosis of the scimitar drainage (6/37 in group 1, 16% and 3/21, 14.3% in group Four patients (7%) required a reoperation, all in group 1; three patients (5.3%) required balloon dilation/stenting for scimitar vein stenosis (2 in group 1, 6% and 1 in group 2, 4.8%). There were 2 late deaths (3.1%) due to severe pulmonary arterial hypertension (1 in group 1, 2.7% and 1 in group 2, 4.7%). Conclusions: Surgical repair of scimitar syndrome is safe and effective, however, a relatively high incidence of stenosis of the scimitar venous drainage is present at follow-up. The intra-atrial baffle technique carries a lower incidence of postoperative complications, but it seems to relates to a higher incidence of reoperations for scimitar vein stenosis.