Pulmonary Artery Sling Treatment & Management
- Author: Stuart Berger, MD; Chief Editor: Steven R Neish, MD, SM more...
Medical Care
In patients with pulmonary artery sling, medical care is supportive until the patient can undergo definitive surgical correction. Hypoxemia and respiratory distress should be treated with supplemental oxygen and endotracheal (ET) intubation if indicated. Treat pneumonia with appropriate antibiotics. Stabilize the patient and make arrangements for surgery as soon as possible. Infants without airway obstruction and with minimal symptoms may not require surgical intervention. However, this scenario is the rare exception.
Surgical Care
Survival of symptomatic infants is unlikely without early surgical intervention. In 1954, Potts and Hollinger reported the first description of surgical repair of this lesion.[4] Surgery involves division of the anomalous left pulmonary artery and reanastomosis to the main pulmonary artery anterior to the trachea. Although this can be performed from a left thoracotomy, approach from a midline sternotomy with cardiopulmonary bypass may offer more control. The findings from one study, which presented a large series of successful patient management experiences, emphasize the advantage of cardiopulmonary bypass as the treatment of choice.[5]
Mortality varies and has been reported to be relatively high in previous studies. The major contributor to postoperative mortality is the high frequency of bronchial and tracheal abnormalities in this group of patients. Early, aggressive intervention may minimize the mortality caused by these associated lesions. If airway stenosis is severe, patients may require surgical reconstruction of the obstructed bronchi or trachea at the time of reimplantation of the anomalous left pulmonary artery.
Surgical survivors are usually free of significant symptoms at long-term follow-up. However, some degree of airway obstruction may persist in as many as 45% of patients.
Consultations
- Pediatric cardiologist
- Cardiovascular surgeon experienced in congenital heart defect surgery
- Radiologist
Diet
No specific dietary considerations are needed.
Activity
Normal activity is indicated after surgical repair unless significant airway obstruction persists.
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Potts W, Holinger P. Anomalous left pulmonary artery causing obstruction to the right main stem bronchus. JAMA. 1954;155:1409.
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