Background: Thirty-two patients with scimitar syndrome were seen in the period between 1975 and 1995. There were 11 male and 21 female patients. Median age at diagnosis was 7 months (mean 7.7 years, range 1 day to 70 years). Patients in whom the diagnosis was made during the first year of life (infantile group, n = 19) had more severe symptoms and had a higher incidence of heart failure (11/19 vs 0/13) and of pulmonary hypertension (11/19 vs 1/13) than did the patients in whom the diagnosis was made after age 1 year (adult group, n = 13). In 17 patients the anomalous pulmonary venous drainage was repaired by baffling the vein to the left atrium. The median age at this operation was 5.8 years (mean 14.8 years, range 6 months to 70 years).
Results: No deaths occurred in this surgical group during a mean follow-up period of 8.9 years (range 1.6 to 17 years). Eight patients (47%), however, had evidence of pulmonary venous stenosis after repair, and two required reoperation for pulmonary venous obstruction. All six children in the infantile group had postoperative pulmonary venous stenosis, compared with two of 11 older patients. Postoperative quantitative pulmonary perfusion scans performed in 15 patients demonstrated reduced flow to the right lung (24%, range 0% to 59%).
Conclusion: We conclude that age at detection of scimitar syndrome is important in predicting outcome. Surgical repair seldom results in normal blood flow to the right lung but abolishes left-to-right shunt. Postoperative pulmonary venous obstruction is prevalent, especially in the infants.