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Single Stage Repair of Type A Interrupted Aortic Arch With Type III Aortopulmonary Window in a Preterm Child
Interrupted aortic arch occurring with an aortopulmonary window is an extremely rare anomaly with very few cases described in the current literature. This video demonstrates a single stage repair in a preterm very-low birth weight neonate, using autologous pericardium to reconstruct the aorta.
The patient was a one-week-old late preterm neonate with a birth weight of 1.7 kg, delivered by cesarean section at 36 weeks. He developed respiratory distress soon after delivery. Upon further evaluation, he was diagnosed with a type A interrupted aortic arch and a type III aortopulmonary window. The child was started on a prostaglandin infusion at 0.05 mcg/kg/min and stabilized for a week. Surgery was performed by establishing cardiopulmonary bypass (CPB) with two arterial cannulation, of the ascending and descending aorta, and right atrium cannulation. The child was cooled to 18 degrees Celsius using the alpha-stat pH method. Anterior cerebral perfusion was established by advancing the aortic cannula into the innominate artery, and the patent ductus arteriosus was ligated after removing the descending aortic cannulation. The aortopulmonary window was closed using an autologous pericardial patch. The descending thoracic aorta was mobilized further and posteriorly it was anastomosed directly to the ascending aorta. The arch was reconstructed anteriorly using an autologous pericardial patch and the anastomosis was completed.
The patient came off CPB with no gradient across the reconstructed arch. The child had a delayed chest closure on the second postoperative day and was sent home on the fifth postoperative day. The patient had an uneventful recovery and was doing well after two months. He had no gradient across the ascending and descending aorta and no narrowing of the main pulmonary branch or pulmonary arteries.