Scimitar Syndrome: European Study

Circulation. 2010;122:1159-
Scimitar Syndrome. A European Congenital Heart Surgeons Association (ECHSA) Multicentric Study
Vladimiro L. Vida, MD, PhD; Massimo A. Padalino, MD, PhD; Giovanna Boccuzzo, MPH; Erjon Tarja, MD; Hakan Berggren, MD; Thierry Carrel, MD; Sertaç Çiçek, MD; Giancarlo Crupi, MD; Duccio Di Carlo, MD; Roberto Di Donato, MD; José Fragata, MD; Mark Hazekamp, MD; Viktor Hraska, MD; Bohdan Maruszewski, MD; Dominique Metras, MD; Marco Pozzi, MD; Rene Pretre, MD; Jean Rubay, MD; Heikki Sairanen, MD; George Sarris, MD, PhD; Christian Schreiber, MD; Bart Meyns, MD; Tomas Tlaskal, MD; Andreas Urban, MD; Gaetano Thiene, MD; Giovanni Stellin, MD

Background— Scimitar syndrome is a rare congenital heart disease. To evaluate the surgical results, we embarked on the European Congenital Heart Surgeons Association (ECHSA) multicentric study.
Methods and Results— From January 1997 to December 2007, we collected data on 68 patients who underwent surgery for scimitar syndrome. Primary outcomes included hospital mortality and the efficacy of repair at follow-up. Median age at surgery was 1.4 years (interquartile range, 0.46 to 7.92 years). Forty-four patients (64%) presented with symptoms. Surgical repair included intraatrial baffle in 38 patients (56%; group 1) and reimplantation of the scimitar vein onto the left atrium in 21 patients (31%; group 2). Eight patients underwent right pneumectomy, and 1 had a right lower lobe lobectomy (group 3). Four patients died in hospital (5.9%; 1 patient in group 1, 2.6%; 3 patients in group 3, 33%). Median follow-up time was 4.5 years. There were 2 late deaths (3.1%) resulting from severe pulmonary arterial hypertension. Freedom from scimitar drainage stenosis at 13 years was 83.8% in group 1 and 85.8% in group 2. Four patients in group 1 were reoperated, and 3 patients (2 in group 1 [6%] and 1 in group 2 [4.8%]) required balloon dilation/stenting for scimitar drainage stenosis.
Conclusions— The surgical treatment of this rare syndrome is safe and effective. The majority of patients were asymptomatic at the follow-up control. There were a relatively high incidence of residual scimitar drainage stenosis that is similar between the 2 reported corrective surgical techniques used.