Related Experiment Videos
Scimitar syndrome: twenty years' experience and results of repair
H K Najm1, W G Williams, J G Coles
1Department of Pediatrics and Surgery, Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Age at diagnosis significantly impacts scimitar syndrome outcomes. Surgical repair of scimitar syndrome can lead to pulmonary venous obstruction, particularly in infants, affecting lung perfusion.
Area of Science:
- Cardiology
- Pediatric Surgery
- Thoracic Surgery
Background:
- Scimitar syndrome affects pulmonary venous drainage, with diagnosis timing influencing severity.
- Infantile scimitar syndrome presents with more severe symptoms, including heart failure and pulmonary hypertension.
- Adult diagnosis of scimitar syndrome is associated with less severe clinical manifestations.
Purpose of the Study:
- To analyze the outcomes of surgical repair for scimitar syndrome.
- To evaluate the impact of age at diagnosis on patient prognosis.
- To assess the incidence of postoperative complications, specifically pulmonary venous obstruction.
Main Methods:
- Retrospective review of 32 scimitar syndrome patients diagnosed between 1975 and 1995.
- Surgical repair via baffling anomalous pulmonary venous drainage to the left atrium in 17 patients.
- Postoperative follow-up including clinical assessment and pulmonary perfusion scans.
Main Results:
- No surgical mortality occurred during a mean follow-up of 8.9 years.
- Pulmonary venous stenosis was observed in 47% of surgically treated patients, with infants being more affected (6/6).
- Reduced right lung perfusion was noted in 24% of patients post-surgery, indicating persistent flow abnormalities.
Conclusions:
- Age at scimitar syndrome detection is a critical factor for predicting patient outcomes.
- Surgical repair effectively corrects the left-to-right shunt but rarely normalizes pulmonary blood flow.
- Postoperative pulmonary venous obstruction is a significant complication, especially in the infantile scimitar syndrome population.
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.