Medical Care
In patients with pulmonary artery sling, medical care is supportive until the patient can undergo definitive surgical correction. Inpatient care in patients with pulmonary artery sling may be required for symptoms of airway obstruction and/or pneumonia.
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.
After surgical repair, these symptoms of airway obstruction and/or pneumonia may persist, although they should improve over time.
Consultations
Obtain consultations with the following specialists:
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Pediatric cardiologist
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Cardiovascular surgeon experienced in congenital heart defect surgery
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Radiologist
Transfer
Patients hospitalized with significant stridor, in whom a vascular ring or pulmonary artery sling is suspected, should be transferred to a facility that can provide pediatric cardiology and pediatric cardiovascular surgery services.
Diet and activity
No specific dietary considerations are needed.
Normal activity is indicated after surgical repair unless significant airway obstruction persists.
Surgical Care
Survival of symptomatic infants with pulmonary artery sling is unlikely without early surgical intervention. In 1954, Potts and Hollinger reported the first description of surgical repair of this lesion. [8] 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. [9]
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. For example, tracheal stenosis associated with pulmonary artery sling is often repaired using sling tracheoplasty. [9] See the video below.
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.
Follow-up
Close follow-up care after surgery is required, particularly monitoring for persistent airway obstruction.
Although airway symptoms should improve, follow patients closely, especially during times of upper respiratory infections.
Similarly, monitor this group of patients closely for the possibility of left pulmonary artery stenosis after reimplantation of the vessel. This can be noninvasively assessed with echocardiography but rarely may require follow-up pulmonary artery angiography.
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Slide tracheoplasty and left PA sling repair. Procedure performed by Giles Peek MD, FRCS, CTh, FFICM, The Children’s Hospital at Montefiore, Bronx, NY. Video courtesy of Montefiore.
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Aberrant left pulmonary artery or pulmonary artery sling.
