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J Thorac Cardiovasc Surg 1996;112:1161-1169
© 1996 Mosby, Inc.

SURGERY FOR CONGENITAL HEART DISEASE

SCIMITAR SYNDROME: TWENTY YEARS' EXPERIENCE AND RESULTS OF REPAIR

From the Divisions of Cardiovascular Surgery and Cardiology, Department of Pediatrics and Surgery, Hospital for Sick Children, and University of Toronto Faculty of Medicine, Toronto, Ontario, Canada.

Received for publication May 6, 1996 Revisions requested June 6, 1996; revisions received June 21, 1996 Accepted for publication June 24, 1996. Address for reprints: William G. Williams, MD, Chief, Cardiovascular Surgery, Division of Cardiovascular Surgery, The Hospital for Sick Children, 555 University Ave., Toronto, Ontario M5G 1X8, Canada.

Abstract

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. (J THORACCARDIOVASCSURG1996;112:1161-9)

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