We report a challenging case of a 32-year-old patient in New York Heart Association (NYHA) class IV with pulmonary atresia, ventricular septal defect, a left aortopulmonary collateral artery, a right modified Blalock-Taussing shunt, and a gigantic aneurysm of the ascending aorta with severe aortic valve insufficiency. A combined Rastelli and Bentall procedure was performed by a joint adult and pediatric cardiovascular surgery team. One year after the operation, the patient is in NYHA class I, working full time.
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