[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.