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We report a rare case of interrupted aortic arch and a right aortic arch associated with DiGeorge syndrome, in neonate. Through a median sternotomy bypass was established placing an arterial perfusion cannula both in the ascending aorta, and in the main pulmonary artery. The right and left pulmonary arteries were temporarily occluded, while this pulmonary cannula perfused the lower part of the body. The arch reconstruction was performed during profound hypothermic total circulatory arrest. The right descending aorta had an adequate length and direct anastomosis was carried out without any tension. The VSD was repaired through a right atrial approach. The patient had hypocalcemia and thymic abnormalities which was consistent with the DiGeorge syndrome. He was treated with calcium gluconate and alfacalcidol, but no serious infection due to immunodeficiency was seen after operation. Post operative catheterization revealed no pressure gradient at the site anastomosis of the aortic arch and satisfactory results.
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