Scimitar Syndrome Repair in Adults: Intermediate-Term Results Using an Extracardiac Conduit.

Background: Scimitar syndrome may be corrected using different techniques. Repair using an extracardiac conduit has rarely been performed. This study assessed the intermediate-term outcomes of this technique in adults.

Methods: From January 2000 to June 2011, 7 adult patients underwent correction with a ringed polytetrafluoroethylene conduit used to connect the scimitar vein (SV) to the left atrium, posterior to the inferior vena cava (IVC). Preoperative and perioperative data were reviewed retrospectively.

Results: All patients (32 ± 10.6 years old) were symptomatic (3 patients were New York Heart Association [NYHA] functional class II, 4 had recurrent pneumonia), with a ratio of pulmonary to systemic blood flow greater than 2, without significant pulmonary hypertension. In all patients, the SV drained the entire right lung venous return to the IVC below the diaphragm. Surgical repair was performed by sternotomy, normothermic cardiopulmonary bypass, and aortic cross-clamping. Four patients required additional closure of an atrial septal defect. Mean conduit diameter was 14 mm (range, 12 to 16 mm). Patients received long-term platelet suppression therapy with aspirin. There were no deaths and no reoperations. Mean follow-up time was 9.1 ± 3.6 years. Postoperative morbidity was nil. No evidence of subclinical stroke or embolization was found in postoperative magnetic resonance imaging of the brain. No thrombi on the prosthesis or in the left atria were detected at the latest echocardiogram, with laminar flow from the SV to the left atrium. At last follow-up, all patients were in NYHA class I.

Conclusions: Correction of scimitar syndrome with an extracardiac conduit can be easily and safely performed in adults, with excellent intermediate-term durability, without graft thrombi or stenosis. This technique avoids deep hypothermic circulatory arrest when the SV is short or enters the IVC in an unusually caudad location.