[Anesthetic Management of Single-staged Definitive Repair of Pulmonary Atresia with Ventricular Septum Defect and Major Aorto-pulmonary Collateral Arteries (PA/VSD, MAPCA)in an Adult].

A 25-year-old woman with unrepaired pulmonary atresia, ventricular septal defect and major aorto-pul- monary collateral artery was scheduled for single- staged definitive repair. She was complicated with mod- erate pulmonary hypertension, and had 2 MAPCAs arising from the descending artery. Cardiac catheter- ization demonstrated that right ventricular pressures were approximately equal to the left ventricular pres- sure. Pre-bypass, we maintained her PVR not too high in order to maintain her pulmonary blood flow. Post- bypass, we used hemodynamic support with dopamine, olprinone and nitroglycerin, maintaining her PVR opti- mally low. We inserted a catheter introducer in her jugular vein to deal with massive hemorrhage. After the repair, her right ventricular pressures were 7/10 of systemic pressure, and her postoperative course was uneventful.