AI Article Synopsis
- Scimitar syndrome is a rare congenital heart defect, and a study involving 68 patients treated surgically from 1997 to 2007 assessed the outcomes of different surgical methods.
- The majority of patients experienced positive results post-surgery, showing safety and effectiveness, with a low hospital mortality rate of 5.9% and a median follow-up of 4.5 years.
- Despite a high success rate, the study found that many patients experienced residual scimitar drainage stenosis, with similar rates regardless of the surgical technique employed.
Article 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.
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| http://dx.doi.org/10.1161/CIRCULATIONAHA.109.926204 | DOI Listing |
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