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Pulmonary artery sling. Results of surgical repair in infancy
C L Backer1, F S Idriss, L D Holinger
1Division of Cardiovascular-Thoracic Surgery, Children's Memorial Hospital, Chicago, IL 60614.
Insights
Pulmonary artery sling, a rare congenital anomaly, can be surgically repaired in infants with low mortality. This procedure involves re-implanting the left pulmonary artery, achieving excellent long-term patency.
Area of Science:
- Cardiovascular Surgery
- Pediatric Surgery
- Thoracic Surgery
Background:
- Pulmonary artery sling is a rare congenital vascular anomaly.
- It causes tracheal and bronchial compression, leading to severe respiratory distress in infants.
- Associated complete tracheal rings are common.
Purpose of the Study:
- To review surgical outcomes for pulmonary artery sling repair in infants.
- To evaluate the efficacy of surgical techniques and associated procedures.
Main Methods:
- Retrospective review of 12 infants (1953-1990) undergoing surgical repair.
- Surgical repair involved left pulmonary artery re-implantation anterior to the trachea.
- Bronchoscopy, pulmonary angiography, CT, and MRI were used for diagnosis and assessment.
Main Results:
- No operative deaths occurred among 12 infants.
- Two late deaths occurred postoperatively.
- Of 10 long-term survivors, 78% (7/9) had patent left pulmonary artery anastomoses.
- Simultaneous pericardial patch tracheoplasty was performed in 3 patients for complete tracheal rings.
Conclusions:
- Pulmonary artery sling repair in infancy has low operative mortality.
- Re-implantation of the left pulmonary artery provides excellent long-term patency.
- Simultaneous pericardial patch tracheoplasty is recommended for associated complete tracheal rings.
- Early surgical repair at diagnosis, using median sternotomy and extracorporeal circulation, is advised.
Abstract:
Pulmonary artery sling is a rare congenital vascular anomaly in which the left pulmonary artery originates from the right pulmonary artery and encircles the right main-stem bronchus and distal trachea before entering the hilum of the left lung. This causes compression of the trachea and right main-stem bronchus, and most infants with this anomaly have severe respiratory distress within the first year of life. Between 1953 and 1990 12 infants (nine male, three female) underwent surgical repair of pulmonary artery sling. Ages ranged from 8 days to 9 months (mean age 5 months). Bronchoscopic examination was performed in all patients. Complete tracheal rings were the most common associated lesion (five patients). Nine patients had pulmonary angiography. Most recently, computed tomography and magnetic resonance imaging have been used to diagnose pulmonary artery sling and associated complete tracheal rings when present. Surgical repair consisted of transection of the left pulmonary artery at its origin and implantation into the main pulmonary artery anterior to the trachea via right thoracotomy (one), left thoracotomy (six), or median sternotomy (five). Three patients had simultaneous pericardial patch tracheoplasty for complete tracheal rings. There were no operative deaths. Two late deaths occurred, at 7 months and 2.5 years postoperatively. Of 10 long-term survivors nine have had postoperative studies to determine the patency of the left pulmonary artery. Seven anastomoses were patent (78%). Pulmonary artery sling can be repaired in infancy with low operative mortality and excellent long-term patency of the left pulmonary artery by dividing the left pulmonary artery and implanting it into the main pulmonary artery anterior to the trachea. Simultaneous pericardial patch tracheoplasty should be performed if complete tracheal rings are associated. We recommend repair at the time of diagnosis with median sternotomy and extracorporeal circulation.