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Double Switch Operation (Senning and Rastelli)
The patient was a 23-month-old, 8.6 kg girl with complex congenital heart disease consisting of dextro-mesocardic position, double outlet right ventricle, subaortic ventricular septal defect, pulmonary atresia, atrioventricular discordance, and left atrial isomerism. In addition, she had left superior vena cava connecting to right innominate vein, a midline inferior vena cava, and right aortic arch with mirror image branching.
The patient was palliated in the neonatal period with a 4 mm left modified Blalock-Taussig shunt and PDA ligation and division on DOL four via a left thoracotomy. Indication for surgery at that point was ductal dependent pulmonary blood flow in the setting of pulmonary atresia. The patient had a right aortic arch with PDA inserting into the proximal right pulmonary artery, thus BT shunt was placed on the left.
Subsequent cath at four months of age showed discontinuous pulmonary arteries, and the patient underwent right modified BT shunt via right thoracotomy. It was found at this time that her proximal right pulmonary artery had been inadvertently ligated (instead of her ductus) during her first operation. Thus, her ductus supplied her right pulmonary artery with flow and allowed it to grow. The patient’s left and right pulmonary artery became discontinuous when her ductus spontaneously closed.
The patient was determined to be a poor candidate for a biventricular repair at OSH. She was transferred to Cincinnati Children’s Hospital Medical Center for a second opinion. After reviewing her hemodynamics, she was considered to be a candidate for biventricular repair consisting of double switch that involved Senning and Rastelli (right ventricular outflow reconstruction with 18 mm pulmonary homograft), VSD enlargement, unifocalization, and patch arterioplasty of bilateral branch pulmonary arteries.
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