[General Anesthesia for Septal Myectomy in a Patient with Noonan Syndrome, Severe Hypertrophic Obstructive Cardiomyopathy and Right Ventricular Outflow Tract Obstruction].

We report a case of an 18-year-old man with Noonan syndrome, severe hypertrophic obstructive cardiomy- opathy and right ventricular outflow tract obstruction who underwent septal myectomy under general anes- thesia. In our case, the ventricular outflow tract pres- sure gradients were 108 mmHg at left and 79 mmHg at right. General anesthesia was induced deliberately by fentanyl, midazolam and sevoflurane. Anesthesia was maintained with sevoflurane. For treatment of hypotension, we performed volume loading and admin- istration of phenylephrine. We did not use drugs that increase heart rate or contractility. Preload and after- load were well maintained, and the operation and gen- eral anesthesia were completed without serious prob- lem.

[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.