Perioperative management of a patient with anomalous origin of the left coronary artery from the pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital disease, also known as Bland-White-Garland (BWG) syndrome. Mitral valve regurgitation secondary to papillary muscle dysfunction due to myocardial ischemia and progressive left ventricular dysfunction are major clinical manifestations of BWG syndrome. We report perioperative management of a five-year-old patient with BWG syndrome who underwent the Takeuchi procedure making an ¡baffle using anterior pulmonary artery wall. General anesthesia was maintained with inhalation of sevoflurane in 25-40% oxygen and continuous infusion of remifentanil. Since a decrease in pulmonary artery pressure could induce coronary steal phenomenon, we ventilated the patient with minimally required F_IO2 to maintain Sp_O2 98-100%, and maintained Pa_CO2 between 40 and 50 mmHg to avoid myocardial ischemia before the induction of cardiopulmonary bypass (CPB). We started continuous infusions of nitroglycerin and milrinone after the initiation of CPB, which were continued through the post-CPB period. The patient was transferred to the ICU and the postop-erative course was uneventful. There are two key points in the management of BWG syndrome : the balance between systemic and pulmonary artery pressure in pre-CPB period, and left ventricular support including both inotropes and vasodilators in post-CPB period. key words : anomalous origin of the left coronary artery from the pulmonary artery, Bland-White-Garland syndrome, mitral valve regurgitation, Takeuchi procedure.