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Surgical management of pulmonary artery sling in children
Matthew S Yong1, Yves d'Udekem2, Christian P Brizard2
1Royal Children's Hospital, Melbourne, Australia; University of Melbourne, Melbourne, Australia.
Insights
Pulmonary artery sling repair outcomes depend on tracheal involvement. While tracheal surgery increases mortality, the slide tracheoplasty technique has improved survival rates for these complex congenital cases.
Area of Science:
- Cardiovascular Surgery
- Pediatric Surgery
- Thoracic Surgery
Background:
- Pulmonary artery (PA) sling is a rare vascular anomaly.
- It is frequently associated with congenital tracheal stenosis, leading to poor natural history.
- Early surgical intervention is often required for affected children.
Purpose of the Study:
- To describe the surgical repair experience for PA sling.
- To analyze outcomes and identify factors influencing mortality.
- To evaluate the impact of tracheal surgery and evolving techniques.
Main Methods:
- Retrospective review of 21 patients undergoing PA sling repair (1984-2011).
- Assessment of tracheal compression and need for concomitant tracheal surgery.
- Analysis of operative mortality, late deaths, and long-term follow-up.
Main Results:
- Operative mortality was 14.3%, with all deaths in patients requiring tracheal repair.
- Introduction of slide tracheoplasty since 2004 correlated with zero operative mortality.
- Survivors (100%) remain asymptomatic, with 50% of those needing tracheal repair requiring further intervention.
Conclusions:
- PA sling repair outcomes are excellent for patients without tracheal surgery.
- The need for tracheal surgery is the primary determinant of mortality.
- Slide tracheoplasty has significantly reduced mortality, offering an excellent prognosis for survivors beyond one year.
Objectives:
Pulmonary artery (PA) sling is a rare vascular anomaly associated with congenital tracheal stenosis. The natural history is poor and these patients often require early surgical intervention. We describe our experience with repair of this condition.
Methods:
From 1984 to 2011, 21 patients with PA sling underwent repair at the Royal Children's Hospital (median age, 5.9 months). PA sling was associated with compression of the trachea in all patients. Tracheal surgery was required in 12 (57.1%) patients. All patients had an echocardiogram, and concomitant repair of coexisting cardiac anomalies was performed in 6 (28.6%, 6/21) patients.
Results:
Operative mortality was 14.3% (3/21), occurring at 19 days, 4.4 months, and 5 months after surgery. Operative mortality for the first 10 years was 22.2% (1984-1993; 2/9), the next 10 years was 14.3% (1994-2003; 1/7), and 0% for the most recent 7 years (2004-2011; 0/5). All deaths occurred in patients requiring tracheal repair (25%, 3/12). No deaths have occurred since 2004 with introduction of the slide tracheoplasty technique. One (5.6%, 1/18) late death occurred at 8 months after repair. After tracheal repair, intervention for excessive granulations and tracheomalacia was necessary in 6 (50%, 6/12) patients. Median follow-up was 8 years (mean, 8.6 ± 6.4 years; range, 5 months to 20.6 years), and all survivors (100%, 17/17) remain asymptomatic.
Conclusions:
Children with PA sling who do not require tracheal surgery have excellent outcomes. Mortality is determined by the need for tracheal surgery. However, with the advent of the slide tracheoplasty technique, mortality can be reduced. Survival beyond 1 year after surgery offers excellent prognosis.
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