Scimitar syndrome: a European Congenital Heart Surgeons Association (ECHSA) multicentric study

Circulation. 2010 Sep 21;122(12):1159-66. doi: 10.1161/CIRCULATIONAHA.109.926204. Epub 2010 Sep 7.


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.

Publication types

  • Multicenter Study

MeSH terms

  • Cardiovascular Surgical Procedures / adverse effects
  • Cardiovascular Surgical Procedures / methods*
  • Child
  • Child, Preschool
  • Constriction, Pathologic / epidemiology
  • Europe
  • Female
  • Follow-Up Studies
  • Hospital Mortality
  • Humans
  • Incidence
  • Infant
  • Kaplan-Meier Estimate
  • Male
  • Retrospective Studies
  • Scimitar Syndrome / mortality
  • Scimitar Syndrome / surgery*
  • Treatment Outcome