Surgery for scimitar syndrome: the Melbourne experience

To evaluate the outcomes associated with surgical repair of scimitar syndrome.

From 1974 to 2012, 21 patients with scimitar syndrome underwent corrective surgery. Outcomes included mortality, reoperation rate, hospital length of stay and long-term functional status.

The mean age of surgery was 5.4 years (2.5 months to 16.7 years). Sixteen (76.2%) patients were symptomatic at presentation. Fifteen (71.4%) patients presented with the infantile form and 6 (28.6%) with the adult form of scimitar syndrome. Operative techniques included baffle repair in 15 (71.4%) patients, reimplantation in 5 (23.8%) patients and pneumonectomy in 1 (4.8%) patient. Six (28.6%) patients were operated in infancy at a mean of 4 months (2.5-9.6 months) of age. There was 1 (4.8%) hospital death and 3 (15%) late deaths. The risk factors for mortality included preoperative pulmonary hypertension (P = 0.006) and surgery during infancy (P = 0.003). The incidence of postoperative pulmonary vein stenosis was 23.8% (n = 5). Three (14.3%) patients underwent reoperation for pulmonary vein stenosis. Neither baffle (P = 0.6) nor reimplantation (P = 0.55) surgical techniques influenced the rate of stenosis. The follow-up was complete in 15 (88%) survivors at a mean of 13.7 years (1.3-38.5 years). All surviving patients were asymptomatic and had a New York Heart Association functional status Class I.

Mortality was highest in patients who had preoperative pulmonary hypertension and those who were operated on before 1 year of age. Surgical technique did not influence the occurrence of pulmonary vein stenosis. Survivors were asymptomatic at the long-term follow-up.