Treatment of tachyarrhythmias in a patient with the long QT syndrome by autotransplantation of the heart and sinus node-triggered atrial pacing.

The case history of a 37-year-old woman with the long QT syndrome and drug-refractory paroxysmal ventricular tachyarrhythmias is reported. She was resuscitated eight times between 1980 and 1987. The duration of these attacks increased from a few minutes to 8 hours and the interval between them decreased from 2 years to 4 weeks. An autotransplantation was performed of the anatomically and hemodynamically normal heart. She has had normal QT intervals and has been free of tachyarrhythmias since the operation in June 1987. Because of a slow escape low atrial rhythm, she was treated with a dual-chamber pacemaker programmed in the VDD mode with a coupling interval of 15 msec. The pacemaker wires were connected to the nontransplanted atrial cuff ("atrial" wire) and the transplanted atrium ("ventricular" wire). The hemodynamic effect of the resultant sinus node-driven low atrial pacing was restoration of normal values. This is the first report of successful autotransplantation of the heart for ventricular tachyarrhythmias caused by the long QT syndrome.