The Scimitar Syndrome: An Italian Multicenter Study

Background: Scimitar syndrome is a rare congenital heart disease. To evaluate the results of managing this malformation surgically, we have embarked on a multicenter Italian study involving seven different centers and reporting the largest published series in the medical literature. Methods: From January 1997 to December 2007, 26 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 11 years (interquartile range, 1.8 to 19.9 years). Nineteen patients (73%) presented with symptoms including upper respiratory tract infections (n = 13), recurrent pneumonia (n = 10), cardiac failure (n = 4), and cyanosis (n = 2). Associated cardiac anomalies were present in 16 patients (63%). Surgical repair included intraatrial baffle repair in 18 patients (69%; group 1), and reimplantation of the "scimitar vein" onto the left atrium in 8 patients (31%; group 2). One patient died in hospital (3.8%; group 1). Postoperative complications were less frequently reported in group 1 (4 of 18 patients, 22%) compared with group 2 (5 of 8 patients, 62%). Median follow-up time was 4 years (interquartile range, 1.8 to 9.7 years). There was 1 late death (1/25 patients, 4%; group 2). Four patients (16%) showed a complete occlusion of the scimitar drainage (2 in group 1, 12%; 2 in group 2, 25%) and 3 patients (12%) required balloon dilation or stenting for scimitar vein stenosis (1 in group 1, 6%; 2 in group 2, 25%). Conclusions: The intraatrial baffle repair seems to have a lower incidence of postoperative complications and a better patency rate, at last follow-up, than the reimplantation of the scimitar vein onto the left atrium.