title: Scimitar syndrome: a European Congenital Heart Surgeons Association (ECHSA) multicentric study.
abstract: 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.
  
  
  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.
authors.affiliation: Pediatric and Congenital Cardiac Surgery Unit, University of Padua, Via Giustiniani 2, Padua, Italy.
authors.forename: Vladimiro L
authors.initials: VL
authors.lastname: Vida
authors.forename: Massimo A
authors.initials: MA
authors.lastname: Padalino
authors.forename: Giovanna
authors.initials: G
authors.lastname: Boccuzzo
authors.forename: Erjon
authors.initials: E
authors.lastname: Tarja
authors.forename: Hakan
authors.initials: H
authors.lastname: Berggren
authors.forename: Thierry
authors.initials: T
authors.lastname: Carrel
authors.forename: Sertaç
authors.initials: S
authors.lastname: Ciçek
authors.forename: Giancarlo
authors.initials: G
authors.lastname: Crupi
authors.forename: Duccio
authors.initials: D
authors.lastname: Di Carlo
authors.forename: Roberto
authors.initials: R
authors.lastname: Di Donato
authors.forename: José
authors.initials: J
authors.lastname: Fragata
authors.forename: Mark
authors.initials: M
authors.lastname: Hazekamp
authors.forename: Viktor
authors.initials: V
authors.lastname: Hraska
authors.forename: Bohdan
authors.initials: B
authors.lastname: Maruszewski
authors.forename: Dominique
authors.initials: D
authors.lastname: Metras
authors.forename: Marco
authors.initials: M
authors.lastname: Pozzi
authors.forename: Rene
authors.initials: R
authors.lastname: Pretre
authors.forename: Jean
authors.initials: J
authors.lastname: Rubay
authors.forename: Heikki
authors.initials: H
authors.lastname: Sairanen
authors.forename: George
authors.initials: G
authors.lastname: Sarris
authors.forename: Christian
authors.initials: C
authors.lastname: Schreiber
authors.forename: Bart
authors.initials: B
authors.lastname: Meyns
authors.forename: Tomas
authors.initials: T
authors.lastname: Tlaskal
authors.forename: Andreas
authors.initials: A
authors.lastname: Urban
authors.forename: Gaetano
authors.initials: G
authors.lastname: Thiene
authors.forename: Giovanni
authors.initials: G
authors.lastname: Stellin
mesh_terms: D013504:Cardiovascular Surgical Procedures / Q000009:adverse effects / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D003251:Constriction, Pathologic / Q000453:epidemiology; D005060:Europe; D005260:Female; D005500:Follow-Up Studies; D017052:Hospital Mortality; D006801:Humans; D015994:Incidence; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D012189:Retrospective Studies; D012587:Scimitar Syndrome / Q000401:mortality / Q000601:surgery*; D016896:Treatment Outcome