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[Pulmonary artery sling with tracheal stenosis--primary repair in infancy]
Y Oshima1, M Yamaguchi, H Ohashi
1Kobe Children's Hospital, Japan.
Insights
This study highlights successful surgical repair for infants with pulmonary artery sling and severe congenital tracheal stenosis. Early, aggressive surgical intervention, including concomitant repair of airway and heart defects, improves outcomes.
Area of Science:
- Pediatric Surgery
- Cardiovascular Surgery
- Thoracic Surgery
Background:
- Pulmonary artery sling is a rare congenital anomaly often associated with severe tracheal stenosis, leading to significant respiratory distress in infants.
- Complete tracheal rings and other airway abnormalities frequently coexist, complicating surgical management.
- Intracardiac anomalies are also common, necessitating a comprehensive approach to treatment.
Observation:
- Five infants with pulmonary artery sling and severe congenital tracheal stenosis underwent surgical repair between 1984 and 1996.
- All patients presented with severe respiratory distress, with three requiring preoperative mechanical ventilation.
- Associated anomalies included complete tracheal rings (100%), right upper lobe tracheal bronchus (60%), and intracardiac defects (60%).
Findings:
- Surgical repair involved reimplantation of the left pulmonary artery and tracheoplasty (using costal cartilage grafts or resection and anastomosis).
- Simultaneous repair of intracardiac anomalies was performed in three patients.
- No hospital mortality occurred; all survivors were weaned from ventilation, with three of four showing no postoperative stridor.
Implications:
- Early and aggressive surgical repair of pulmonary artery sling with tracheal stenosis is advocated.
- Concomitant repair of tracheal and intracardiac lesions, when feasible, improves outcomes.
- The use of cardiopulmonary bypass (CPB) or extracorporeal membrane oxygenation (ECMO) can facilitate complex airway management, and tracheal resection with end-to-end anastomosis should be prioritized when possible.
Abstract:
Between 1984 and 1996 five infants underwent surgical repair of pulmonary artery sling associated with severe congenital tracheal stenosis. All infants had symptoms of severe respiratory distress and three of them required ventilator support preoperatively ages ranged from 2 to 11 months (mean age 6 months). Complete tracheal rings were present in all patients as an associated lesion and right upper lobe tracheal bronchus in 3 patients. The length of tracheal stenosis ranged from 18 to 45 mm (median 40 mm). Three had associated intracardiac anomalies (Scimitar syndrome (1), VSD (1), double-outlet right ventricle with VSD (1), double-outlet right ventricle with pulmonary hypertension (1)). Surgical intervention was carried out through a right thoracotomy (1) or median sternotomy (4). Cardiopulmonary bypass (CPB) was used in 3 patients and extracorporeal membrane oxgenator (ECMO). In 1. All infants had reimplantation of the left pulmonary artery into the main pulmonary artery left anterior to the trachea. Four patients underwent simultaneous tracheoplasty using costal cartilage grafts and one had complete resection of obstructed trachea between the right upper lobe tracheal bronchus and carina. The length of resected trachea was about 30% of the entire length of the trachea. Three infants underwent simultaneous intracardiac repair. There was no hospital death. All were weaned from ventilatory support and extubated on 1 to 16 months (mean 4, 5 months) postoperatively. AS an additional procedure, aortopexy, removal of granulation tissue or balloon dilatation of the trachea were carried out in one patient each following tracheoplasty using cartilage grafts. There was one late death at 1 year postoperatively. Three of 4 survivors are doing well with no stridor. We adonostridor. We adovocate 1) early aggressive primary repair of pulmonary artery sling with tracheal stenosis, 2) concomitant repair of tracheal lesion and intracardiac anomalies whenever possible, 3) application of CPB or ECMO to avoid cumbersome intubation technique, and 4) utmost effort to perform tracheal resection and end-to-end anastomosis.