Surgical management of scimitar syndrome: an alternative approach

The scimitar syndrome is a congenital anomaly that consists in part of total or partial anomalous venous drainage of the right lung to the inferior vena cava. Surgical approaches to the scimitar syndrome have varied according to the anatomic and pathologic features presented in each case. The aim of this study was to present an alternative approach to the surgical correction of scimitar syndrome.

Nine patients with the scimitar syndrome were operated on between 1990 and 2000. They comprised 1 male and 8 female patients (mean age 11.5 +/- 17.6 years). All patients had symptoms, with recurrent pneumonia or respiratory tract infections and pulmonary/systemic flow ratios greater than 1.5:1.0. None of the patients had pulmonary hypertension or an atrial septal defect. All patients underwent repair of the anomalous scimitar vein by direct reimplantation into the left atrium without cardiopulmonary bypass. Two patients underwent concomitant resection of a right lower lobe sequestration. Follow-up was complete in all cases.

There were no operative or late deaths, and no patients have required reoperation. At the time of follow-up (mean 55 +/- 46 months), echocardiography demonstrated a patent anastomosis in all patients without any evidence of restenosis.

This clinical experience indicates that an alternative surgical approach to scimitar syndrome is direct anastomosis of the scimitar vein to the posterior aspect of the left atrium using a right thoracotomy without cardiopulmonary bypass. This procedure is safe and effective and obviates the need for long intra-atrial baffles and the use of the extracorporeal circuit.