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The interrupted aortic arch (IAA) is rare congential heart defect and entails a very poor prognosis without surgical treatment.We describe a young adult patient with IAA associated with severe pulmonary hypertension and discuss the perioperative anesthetic management of single-stage surgical correction in this patient.Preoperative anesthetic preparation with better understanding of the malformation,intraoperative hemodynamic monitoring,and adequate alveolar ventilation in the perioperative period were all important factors contributing to a successful outcome.