Surgical Treatment of Pulmonary Artery Sling and Tracheal Stenosis

Background

Pulmonary artery sling is a rare vascular ring and is commonly associated with tracheal stenosis. Symptomatic newborns and infants with these complex lesions have a high mortality rate without surgical intervention. The ideal operation remains controversial, with debate focusing on the need for left pulmonary artery for reimplantation and the technique of tracheal reconstruction.

Methods

From 1983 to 2003, 14 patients with pulmonary artery sling (mean age, 7 months; range, 6 days to 27 months) underwent repair of pulmonary artery sling alone (6 patients), tracheoplasty alone (1 patient), and pulmonary artery sling repair with tracheoplasty (7 patients). Preoperatively, 7 patients were intubated, 2 had VATER (vertebral, anal, tracheal, esophageal, and radial anomalies) syndrome, and 2 patients had agenesis of the right lung. The left pulmonary artery was reimplanted at the ductal insertion site in 13 patients. One patient had left pulmonary artery translocation. Tracheoplasty employing extracorporeal circulation consisted of autologous pericardial patch (6 patients) or slide tracheoplasty (2 patients). Six patients with pulmonary artery sling and mild tracheal stenosis required only left pulmonary artery reimplantation. Concomitant procedures included closure of atrial septal defect (4 patients), ventricular septal defect (4 patients), and shunt for Fallot's tetralogy (2 patients).

Results

There were 2 hospital deaths (2 of 14;14%) from abdominal sepsis (1) and renal failure (1). Reoperations included diaphragm plication (2), tracheostomy (1), and bronchoscopy with laser resection of granulation tissue (5 patients: 2 slide, 3 pericardium). Follow-up was complete in all patients (mean, 42 months) with 1 late death from fungal sepsis. At follow-up, all left pulmonary artery anastomoses were patent by echocardiography, and no patient has required reoperation for trachea reconstruction.

Conclusions

These data demonstrate that tracheal repair is not always necessary in the presence of pulmonary artery sling; that agenesis of the right lung is not a contraindication to successful complete repair; and that simultaneous correction of intracardiac defects can be safely performed in selected patients. This study suggests that in newborns and infants, pericardial patch and slide tracheoplasty are effective methods for trachea reconstruction.

Fig 1. The anomalous left pulmonary artery (LPA) is seen arising from the right pulmonary artery (RPA) and coursing between the esophagus and the trachea. Associated trachea stenosis and the ductus arteriosus are illustrated. (MPA = main pulmonary artery.)

Fig 2. The ductus arteriosus is divided and the anomalous left pulmonary artery (LPA) is detached from the right pulmonary artery (RPA) and reimplanted into the main pulmonary artery (MPA) using absorbable suture.

Fig 3. Autologous pericardial patch tracheoplasty. The pericardial patch is secured to the trachea using continuous absorbable suture avoiding the tracheal mucosa

Fig 4. Slide tracheoplasty. (A) The trachea is divided and the most stenotic segment is excised. (B) The proximal and distal limbs are opened and spatulated as illustrated. The arrows represent the technique of sliding the proximal and distal trachea segments over each other. (C) The spatulated end-to-end anastomosis is performed with interrupted absorbable suture taking bites to avoid the mucosa (see inset).