This scenario, however, is the rare exception. Survival of symptomatic infants is unlikely without early surgical intervention. Potts, in 1954, reported the first description of surgical repair of this lesion. Surgery involves division of the anomalous left pulmonary artery and reanastomosis to the main pulmonary artery anterior to the trachea. While this can be performed from a left thoracotomy, approach from a midline sternotomy with cardiopulmonary bypass may offer more control.
Mortality is variable 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. Pneumonia requires appropriate antibiotics. After surgical repair, these same symptoms may persist, although they should improve over time.