[Anesthetic management of a patient with Fontan circulation with pacemaker failure].

We describe the anesthetic management of a 23-year-old female patient with severe bradycardia due to pacemaker lead disconnection. The pacemaker had been placed due to complete AV block following an extracardiac Fontan operation. She was scheduled for lead repair under general anesthesia. The preoperative HR was only 37 beats x min(-1) of idioventricular rhythm. As further bradycardia was a major concern, we prepared a percutaneous cardiac pacemaker and the catheterization laboratory for transatrial cardiac pacing. After placement of transcutaneous cardiac pacing pads on the chest, we administered isoproterenol at 0.01 microg x kg(-1) x min(-1). We confirmed an increase in HR to 50 beats x min(-1) and induced anesthesia, after which isoproterenol was administered at 0.015 microg x kg(-1) x min(-1). No bradycardia was observed perioperatively. Transvenous cardiac pacing, part of the last step in the ACLS bradycardia algorithm, is inappropriate except in patients with anatomic Fontan circulation. Furthermore, in the case of bradycardia, percutaneous or transesophageal cardiac pacing can be used; however, these are not always effective. Transatrial cardiac pacemakers are probably most effective for bradycardia with Fontan circulation but they should be placed by a catheter specialist, and emergency placement can be difficult. As described here, a special management protocol is necessary to control bradycardia in the face of Fontan circulation.