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.