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Vida, V L; Padalino, M A; Boccuzzo, G; Tarja, E; Berggren, H; Carrel, T; Ciçek, S; Crupi, G; Di Carlo, D; Di Donato, R; Fragata, J; Hazekamp, M; Hraska, V; Maruszewski, B; Metras, D; Pozzi, M; Prêtre, René; Rubay, J; Sairanen, H; Sarris, G; Schreiber, C; Meyns, B; Tlaskal, T; Urban, A; Thiene, G; Stellin, G (2010). Scimitar syndrome: a European Congenital Heart Surgeons Association (ECHSA) multicentric study. Circulation, 122(12):1159-1166.

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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.


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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic for Cardiovascular Surgery
Dewey Decimal Classification:610 Medicine & health
Deposited On:20 Jan 2011 10:13
Last Modified:05 Apr 2016 14:36
Publisher:Lippincott Wiliams & Wilkins
Free access at:Publisher DOI. An embargo period may apply.
Publisher DOI:10.1161/CIRCULATIONAHA.109.926204
PubMed ID:20823387

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