Surgery for scimitar syndrome: the Melbourne experience

Abstract

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

Methods: 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.

Results: 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.

Conclusions: 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.

Keywords: Congenital heart disease; Infant; Pulmonary hypertension; Pulmonary veins; Scimitar syndrome.