Pulmonary artery sling: Current results with cardiopulmonary bypass

Division of Cardiovascular Thoracic Surgery, Children's Memorial Hospital, Chicago, Ill 60614, USA.
The Journal of thoracic and cardiovascular surgery (Impact Factor: 4.17). 11/2011; 143(1):144-51. DOI: 10.1016/j.jtcvs.2011.09.038
Source: PubMed


We have used cardiopulmonary bypass with left pulmonary artery reimplantation for pulmonary artery sling repair since 1985. This review presents our current results with this technique, emphasizing the importance of diagnosis and treatment of frequently associated tracheal stenosis.
Since 1985, 34 patients have undergone pulmonary artery sling repair using a median sternotomy and cardiopulmonary bypass. Age ranged from 9 days to 43 years (mean 2.1 ± 7.5 years, median 0.2 years). Twenty-seven patients (79%) had tracheal stenosis secondary to complete cartilage tracheal rings. All patients had preoperative airway imaging with rigid bronchoscopy, and since 2000 all patients have had computed tomography imaging of the chest with 3-dimensional reconstruction (n = 14). Tracheal repair has included pericardial patch tracheoplasty (n = 7), tracheal autograft (n = 10), tracheal resection (n = 4), and slide tracheoplasty (n = 5). All patients had an echocardiogram, and cardiac lesions repaired simultaneously included atrial septal defect (4), tetralogy of Fallot (2), and ventricular septal defect (1). One patient had a severely hypoplastic right lung, and 3 patients had an absent right lung. In these patients, the left pulmonary artery was translocated anterior to the trachea. In all other patients, the left pulmonary artery was reimplanted into the main pulmonary artery.
There have been no early deaths or complications related to the use of cardiopulmonary bypass. Median hospital stay was 24 days. There have been 4 late deaths. Two late deaths were the result of complications of tracheal surgery (1 pericardial patch [6 months postoperatively] and 1 autograft [1.7 years postoperatively]). One child died of biliary atresia (0.2 years postoperatively), and 1 child died of pneumonia (5.8 years postoperatively). All recent tracheal stenosis repairs have been with slide tracheoplasty. All left pulmonary arteries are patent with a mean percent flow by perfusion scan of 41% ± 13%. Older patients (n = 2) have noted a significant improvement in exercise tolerance.
Pulmonary artery sling is best repaired with median sternotomy, cardiopulmonary bypass, and left pulmonary artery reimplantation. This resulted in uniformly patent left pulmonary arteries in all patients. Preoperative computed tomography imaging, echocardiography, and bronchoscopy are essential for precise operative planning. The frequently associated tracheal stenosis is best repaired with slide tracheoplasty.

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    ABSTRACT: 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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    ABSTRACT: Pulmonary artery sling frequently accompanies various degrees of tracheal stenosis. The authors reviewed their surgical outcomes for pulmonary artery sling from two institutions. From 1997 to September 2011, 16 patients with pulmonary artery sling underwent surgical treatment. Their median age was 4.6 months and their body weight 6.2 kg at the time of the operation. Of the 16 patients, 12 showed preoperative respiratory symptoms such as recurrent airway infection or dyspnea, with three of these patients receiving ventilator therapy preoperatively. Most of the patients showed various degrees of tracheal luminal stenosis and stenotic length, except for three patients who did not show significant airway stenosis. Left pulmonary artery reimplantation was performed for all the patients, but an accompanying tracheoplasty was performed for only two patients (one patch augmentation and one sliding tracheoplasty). After surgery, early extubation and intensive lung care were performed. Two operative mortalities occurred: one due to cardiac tamponade associated with postoperative bleeding and one due to aggravation of preexisting multiorgan failure. Two late mortalities occurred: one due to airway obstruction by repetitive granulation tissue growing at the tracheoplasty site and one due to pneumonia. The 12 living patients, including 11 patients who did not undergo tracheal surgery, showed no clinically significant airway problems at their last follow-up visit. The surgical outcomes for pulmonary artery sling without tracheoplasty were acceptable. The authors think tracheoplasty may be avoided by intensive postoperative airway management for a significant portion of the patients with pulmonary artery sling and tracheal stenosis.
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    ABSTRACT: Pulmonary artery sling is frequently associated with tracheal and/or bronchial stenosis. A number of patients receive only re-implantation or relocation of the left pulmonary artery (LPA) while other patients may require tracheoplasty for stenosis of the airway. This study used computer tomography (CT) scans for the evaluation of airways and to evaluate the trachea diameter and the clinical outcomes with or without tracheoplasty. A total of 15 patients with PA sling who received CT scans were included in this study. We reviewed the clinical outcomes and the severity of trachea stenosis, and evaluated various surgical strategies. Among the 15 patients, one received only conservative treatment, 14 (93%) patients received LPA re-implantation, and eight (53%) received tracheoplasty. For the eight patients that received LPA reimplantation and tracheoplasty, five received slide tracheoplasty and all survived, two patients had pericardial patch augmentation and both died, and one patient received a resection and end-to-end anastomosis and survived. The diameter of the trachea in survivors without tracheoplasty was significantly larger than those who died or received tracheoplasty (3.2 ± 0.3 mm vs. 2.2 ± 0.4 mm, P = 0.003). Establishing 3.0 mm as the threshold, the positive predictive value for tracheoplasty or mortality was 89% and the negative predictive value was 83%. In this study cohort, approximately 60% of the patients with PA sling received tracheoplasty. A diameter of the trachea <3 mm is associated with the use of tracheoplasty or poor outcomes. Slide tracheoplasty provided acceptable good results.
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