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Vida, V.L.; Padalino, M.A.; Boccuzzo, G.; Tarja, E.; Berggren, H.; Carrel, T.; ... ; Stellin, G. (2010)

Scimitar Syndrome A European Congenital Heart Surgeons Association (ECHSA) Multicentric Study

Article / Letter to editor
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...Show moreBackground-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. (Circulation. 2010;122:1159-1166.)Show less
congenital heart disease multicenter study scimitar syndrome pulmonary-hypertension surgical-treatment venous drainage infancy pneumonectomy children sequestration childhood vein form
All authors
Vida, V.L.; Padalino, M.A.; Boccuzzo, G.; Tarja, E.; Berggren, H.; Carrel, T.; Cicek, S.; Crupi, G.; Carlo, D. di; Donato, R. di; Fragata, J.; Hazekamp, M.; Hraska, V.; Maruszewski, B.; Metras, D.; Pozzi, M.; Pretre, R.; Rubay, J.; Sairanen, H.; Sarris, G.; Schreiber, C.; Meyns, B.; Tlaskal, T.; Urban, A.; Thiene, G.; Stellin, G.
Date
2010-09-21
Journal
Circulation
Volume
122
Issue
12
Pages
1159 - 1166
DOI
doi:10.1161/CIRCULATIONAHA.109.926204