Vladimiro L. Vida, Massimo A. Padalino, Giovanna Boccuzzo, Erjon Tarja, Hakan Berggren, Thierry Carrel, Sertaç Çiçek, Giancarlo Crupi, Duccio Di Carlo, Roberto Di Donato, José Fragata, Mark Hazekamp, Viktor Hraska, Bohdan Maruszewski, Dominique Metras, Marco Pozzi, Rene Pretre, Jean Rubay, Heikki Sairanen, George Sarris, Christian Schreiber, Bart Meyns, Tomas Tlaskal, Andreas Urban, Gaetano Thiene, Giovanni Stellin
University of Padua. Queen Silvia Children’s Hospital. University Hospital. Anadolu Medical Center. Ospedali Riuniti, Bergamo. Ospedale Pediatrico Bambino Gesù. Hospital de Santa Marta and Unidade Cardiáca Pediátrica. Leiden University Medical Center, Asklepios Clinic Sankt. Children’s Memorial Health Institute. Hopital de la Timone. Ospedali Riuniti. University Children’s Hospital. Université Catholique de Louvain. Hospital for Children and Adolescents and University of Helsinki. Mitera Pediatric and Hygea Hospital. Munich Clinic of Cardiovascular Surgery. University Hospital.
Italy, Sweden, Switzerland, Turkey, Portugal, Netherlands, Germany, Poland, France, Belgium, Finland, Greece and Czech Republic
Circulation
Circulation 2010; 122: 1159-1166
DOI: 10.1161/CIRCULATIONAHA.109.926204
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.
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.
Category
Stenosis or Obstruction of Pulmonary Veins Following Surgical Repair of Anomalous Pulmonary Venous Connections
Incidence or Prevalence of Disease
Catheter-mediated Interventions: Efficacy or Lack of Efficacy
Surgical Interventions for Pulmonary Venous Obstruction After the Onset of Disease
Year of Publication: 2010
Age Focus: Pediatric
Article Type: Retrospective Observational Cohort Studies (>10 patients)
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: Yes