[Anesthetic management of a child with dilated cardiomyopathy associated with congenital fiber-type disproportion].

: A 3-year-old girl, who presented with dilated cardiomyopathy in conjunction with congenital fiber-type disproportion, underwent open reduction for congenital dislocation of the hip. Preoperative echocardiography demonstrated left ventricular dilatation with an ejection fraction (EF) of 0.33. Anesthesia was induced with intravenous ketamine and fentanyl, and maintained with fentanyl administered incrementally to a total dose of 10 micrograms.kg-1 and 1-1.5% isoflurane. During operation, we continuously monitored left ventricular wall motion and measured left ventricular diastolic dimension (LVDd), systolic dimension (LVDs), cardiac output (CO), EF, and fractional shortening (FS) with transesophageal echocardiography (TEE). At the end of surgery, preload (LVDd) and LV contractility (CO, EF, FS) decreased, but LV wall motion remained almost stable throughout the procedure. In conclusion, TEE was useful for intraoperative management of a child with dilated cardiomyopathy.