Scimitar syndrome: a European Congenital Heart Surgeons Association (ECHSA) multicentric study.
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.
Vida
Vladimiro L
VL
Pediatric and Congenital Cardiac Surgery Unit, University of Padua, Via Giustiniani 2, Padua, Italy.
Padalino
Massimo A
MA
Boccuzzo
Giovanna
G
Tarja
Erjon
E
Berggren
Hakan
H
Carrel
Thierry
T
Ciçek
Sertaç
S
Crupi
Giancarlo
G
Di Carlo
Duccio
D
Di Donato
Roberto
R
Fragata
José
J
Hazekamp
Mark
M
Hraska
Viktor
V
Maruszewski
Bohdan
B
Metras
Dominique
D
Pozzi
Marco
M
Pretre
Rene
R
Rubay
Jean
J
Sairanen
Heikki
H
Sarris
George
G
Schreiber
Christian
C
Meyns
Bart
B
Tlaskal
Tomas
T
Urban
Andreas
A
Thiene
Gaetano
G
Stellin
Giovanni
G
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