ECG changes during emergence from anesthesia in a patient with LEOPARD syndrome

: LEOPARD syndrome is a rare congenital disease characterized by seven features. Besides the original clinical manifestations, hypertrophic cardiomyopathy was reported as an additional laboratory finding recently. We gave general anesthesia to a child of this syndrome, and encountered ECG changes during emergence from anesthesia. The diagnosis was made in her infancy, and had been receiving beta-blocker treatment ever since. The preoperative examination showed remarkable right axis deviation in ECG, and asymmetric ventricular septal hypertrophy in echocardiography. After the operation, several kinds of arrhythmia (i.e. atrioventricular junctional rhythm, atrioventricular dissociation, etc), were observed and they continued for a few minutes. Careful planning for the perioperative management of this syndrome is mandatory. The cardiomyopathy must be examined beforehand. We made an effort to prevent the extreme changes of pre- and after-load during the general anesthesia.