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Surgery for scimitar syndrome: the Melbourne experience.
Johann Brink1, Matthew S Yong1, Yves d'Udekem2
1Royal Children's Hospital, Melbourne, Australia.
Interactive Cardiovascular and Thoracic Surgery
|October 8, 2014
Summary
Surgical repair of scimitar syndrome shows good long-term outcomes, but mortality is higher in infants and those with pulmonary hypertension. Survivors of scimitar syndrome repair achieve excellent functional status.
Area of Science:
- Cardiology
- Pediatric Surgery
- Congenital Heart Disease
Background:
- Scimitar syndrome is a rare congenital anomaly characterized by anomalous pulmonary venous return.
- Surgical intervention is often necessary to correct the anatomical defects and improve patient outcomes.
Purpose of the Study:
- To evaluate the long-term outcomes of surgical repair for scimitar syndrome.
- To identify risk factors associated with mortality and complications after surgery.
Main Methods:
- A retrospective review of 21 patients with scimitar syndrome who underwent corrective surgery between 1974 and 2012.
- Outcomes assessed included mortality, reoperation rates, hospital length of stay, and long-term functional status.
Main Results:
- The mean age at surgery was 5.4 years. Sixteen patients (76.2%) were symptomatic, and most had the infantile form of the syndrome.
- Hospital mortality was 4.8%, with late deaths occurring in 15%. Risk factors for mortality included preoperative pulmonary hypertension and surgery in infancy.
- Postoperative pulmonary vein stenosis occurred in 23.8% of patients, necessitating reoperation in 14.3%. Surgical technique did not influence stenosis rates.
Conclusions:
- Surgical repair of scimitar syndrome can lead to excellent long-term functional status in survivors.
- Preoperative pulmonary hypertension and early surgical intervention (before 1 year) are significant risk factors for mortality.
- Pulmonary vein stenosis is a notable complication, but surgical approach did not impact its incidence.
