[Anesthetic management of a child with Noonan syndrome and hypertrophic obstructive cardiomyopathy].

Noonan syndrome is characterized by facial, skeletal and cardiovascular anomalies. We describe the anesthetic management of a one-year-old boy with Noonan syndrome and hypertrophic obstructive cardiomyopathy scheduled for tonsillectomy and adenoidectomy under general anesthesia. He had a history of congestive heart failure at 5 months of age. Preoperative echocardiogram revealed a hypertrophied septum (12.8 mm) with concentric hypertrophy of the left ventricle. Mild mitral regurgitation was also noted. Our anesthetic goal was set to maintaining adequate preload and afterload as well as adequate anesthesia depth to avoid LV outflow tract obstruction. Intravenous fluid was started the day before surgery. Anesthesia was induced with fentanyl 30 microg and midazolam 2 mg and maintained with propofol 8 mg x kg(-1) x hr(-1), fentanyl 30 microg, ketamine 4 mg, and sevoflurane 1-2%. Intraoperative monitoring included 12-lead electrocardiogram and direct measurement of arterial pressure. Intra- and post-operative course was uneventful.