Surgical management of pulmonary artery sling in children

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 AE 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.

Partial lung resection of supernumerary tracheal bronchus combined with pulmonary artery sling in an adult: report of a case

“…PAS is a rare vascular anomaly in which the left pulmonary artery arises from the right pulmonary artery. It is sometimes combined with tracheal anomalies and causes compression of Miyazaki 5 the lower trachea [1]. Most PAS cases are identified with severe respiratory distress in infants.…”

“…Yong et al [1] reported surgical management of pulmonary artery sling in 21 children. Operative mortality was 14.3%, and it was determined by the need for tracheal surgery.…”

“…It is sometimes combined with tracheal anomalies and causes compression of the lower trachea [1]. However, there are few reports about PAS detected in adulthood.…”

Pulmonary Artery Sling Causing Tracheal Stenosis in a Neonate

“…This approach can substantially reduce postoperative ventilation time and respiratory sequelae in comparison with autologous pericardial patch tracheoplasty. 2,3 Of note, the tracheal autograft technique is typically used to treat tracheal stenosis when the stenotic segment is less than 30% of the trachea's length. …”

Assessment of Extracardiac and Intracardiac Anatomy by MD-CT

“…In patients with congenital heart disease, anomalous vessels and previous surgical operations may cause airway obstruction [3,6,27,28]. MD-CT can provide information on not only abnormal arteries or veins but also the structures of the trachea, bronchi, and coronary arteries.…”