Arterial switch operation for complex d-transposition of the great arteries with aortopulmonary window

A term female neonate was born with a prenatal diagnosis of presumed double outlet right ventricle. On day of life 1, she was transferred from an outside hospital to our institution where she underwent echocardiography. Her postnatal imaging showed complex d-TGA with AP window (type II) and outlet VSD. The patient maintained normal respirations with oxygen saturations in the high 90s and did not require prostaglandin E. At age 6 days, she experienced an episode of acute lactic acidosis that resolved with intubation.

Preoperative 3-dimensional computed tomography reconstruction illustrates the AP window, aberrant left subclavian artery, and right-sided aortic arch (Figure 1). Computed tomography imaging also revealed the patient's coronary artery anatomy: 1RL, 2Cx according to the Leiden convention (Figure 2). On day of life 10 weighing 3.0 kg, she underwent an arterial switch operation under deep hypothermic circulatory arrest. Intraoperatively, the AP window was divided, followed by transection of the aorta and pulmonary arteries at the distal wall of the AP window. The coronary buttons were excised, and the outlet VSD was repaired with a decellularized patch. The coronary arteries were anastomosed to the neoaorta. Next, the branch pulmonary arteries were mobilized toward the hilum, and the LeCompte maneuver was performed. The aorta was reanastomosed to construct the left ventricular outflow tract, followed by right atriotomy and primary closure of the patent foramen ovale. Two U-shaped patches of extracellular matrix were fashioned to complete the neopulmonary root where the coronary buttons were excised. The distal anastomosis with the branch pulmonary arteries was performed to complete the right ventricular outflow tract (Video 1). Cardiopulmonary bypass time was 105 minutes, and aortic crossclamp time was 93 minutes. The patient was transferred to the cardiovascular intensive care unit where she was extubated on postoperative day 6. She is growing and developing well 9 months postoperatively.

d-TGA with AP window is an uncommon form of congenital heart disease. Patients with d-TGA and aortopulmonary window are at increased risk for pulmonary vascular remodeling, which is exacerbated by pulmonary overcirculation. Surgical repair of this condition can be achieved through the arterial switch operation. Different techniques can be applied depending on the size of the AP window and the presence of associated anomalies. Some surgeons report exclusion of the AP window with a homograft or pericardial patch during arterial switch, similar to the repair of a typical AP window.^, For patients with a VSD or PFO, others have chosen to use a patch with a 4-mm fenestration instead of closing the defect completely. These modified patches were intended to reduce the risk of pulmonary hypertension postoperatively by decreasing pulmonary blood flow (fenestrated patent foramen ovale) or enabling decompression of the right ventricle (fenestrated VSD).^, Chen and colleagues indicated that their fenestrated VSD had closed spontaneously after 12 months, and the systolic pulmonary artery pressure was 27 mm Hg. Although these techniques were not utilized in our repair, their physiologic implications should not be overlooked. Perhaps the most variable step in the arterial switch is translocation of the coronary arteries to the neoaorta. Several different approaches have been described in the literature and each is specific to the origin of the coronary arteries. Although d-TGA with AP window represents a small subset of patients, the arterial switch operation remains the gold standard for surgical repair.