The surgical management of the aortic arch pathologies is controversial. Primary anastomosis and patch aortoplasties combined with end-to-end anastomosis have some complications like recurrence and aneurysm formation. Surgical repair of apical muscular (Swiss-cheese) defects is also still under debate. A 6-year-old patient with diagnosis of type A aortic arch interruption and Swiss-cheese ventricular septal defect (VSD) underwent successful intracardiac repair and aortic arch reconstruction. Aortic arch reconstruction was done by end-to-side anastomosis of distal aortic archus and thoracic aorta without cardiopulmonary bypass. The anterior side of the anastomosis was augmented by using pulmonary autograft patch and this patch was extended to the inferior surface of the archus aorta. Swiss-cheese VSD was repaired with a single patch using septal obliteration technique via transatrial approach. Pulmonary autograft patch aortoplasty and end-to-side anastomosis may be an alternative surgical management for surgical repair and it may be done without the need for cardiopulmonary bypass. In these patients associated multiple apical muscular VSDs can be repaired with a single patch, septal obliteration technique.
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