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

2010Journal article Open access
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dc.contributor.authorVida, V
dc.contributor.authorPadalino, M
dc.contributor.authorBoccuzzo, G
dc.contributor.authorTarja, E
dc.contributor.authorBerggren, H
dc.contributor.authorCarrel, T
dc.contributor.authorÇiçek, S
dc.contributor.authorCrupi, G
dc.contributor.authorCarlo, D
dc.contributor.authorDonato, R
dc.contributor.authorFragata, J
dc.contributor.authorHazekamp, M
dc.contributor.authorHraska, V
dc.contributor.authorMaruszewski, B
dc.contributor.authorMetras, D
dc.contributor.authorPozzi, M
dc.contributor.authorPretre, R
dc.contributor.authorRubay, J
dc.contributor.authorSairanen, H
dc.contributor.authorSarris, G
dc.contributor.authorSchreiber, C
dc.contributor.authorMeyns, B
dc.contributor.authorTlaskal, T
dc.contributor.authorUrban, A
dc.contributor.authorThiene, G
dc.contributor.authorStellin, G
dc.date.accessioned2021-10-13T11:44:38Z
dc.date.available2021-10-13T11:44:38Z
dc.date.issued2010
dc.description.abstractBackground: 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.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationCirculation. 2010 Sep 21;122(12):1159-66.pt_PT
dc.identifier.doi10.1161/CIRCULATIONAHA.109.926204pt_PT
dc.identifier.urihttp://hdl.handle.net/10400.17/3875
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherAmerican Heart Associationpt_PT
dc.subjectHSM CCTpt_PT
dc.subjectCardiovascular Surgical Procedures / adverse effectspt_PT
dc.subjectCardiovascular Surgical Procedures / methods*pt_PT
dc.subjectChildpt_PT
dc.subjectEuropept_PT
dc.subjectFemalept_PT
dc.subjectMalept_PT
dc.subjectHumanspt_PT
dc.subjectChild, Preschoolpt_PT
dc.subjectConstriction, Pathologic / epidemiologypt_PT
dc.subjectHospital Mortalitypt_PT
dc.subjectIncidencept_PT
dc.subjectFollow-Up Studiespt_PT
dc.subjectInfantpt_PT
dc.subjectKaplan-Meier Estimatept_PT
dc.subjectRetrospective Studiespt_PT
dc.subjectScimitar Syndrome / mortalitypt_PT
dc.subjectScimitar Syndrome / surgery*pt_PT
dc.subjectTreatment Outcomept_PT
dc.titleScimitar Syndrome: a European Congenital Heart Surgeons Association (ECHSA) Multicentric Studypt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.endPage1166pt_PT
oaire.citation.startPage1159pt_PT
oaire.citation.titleCirculationpt_PT
oaire.citation.volume122pt_PT
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT

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