Ten patients with the
long QT syndrome and recurrent
syncope and/or
cardiac arrest caused by ventricular arrhythmias underwent left stellate (one patient) or left cervicothoracic
sympathectomy (nine patients) after failing to respond to high-dose beta-blocker
therapy. The syndrome was familial in four and idiopathic in six. All patients had a prolonged resting QT interval (548 +/- 51 msec, mean +/- SD) and corrected QT interval (QTc) (556 +/- 43 msec). After
sympathectomy the mean QTc shortened significantly from 556 +/- 43 to 508 +/- 65 msec (p less than .05) but the QTc remained abnormal in all but one patient. Over a mean follow-up period of 38.6 +/- 19 months, eight patients developed recurrent symptoms that included
cardiac arrest in three (one fatal, two nonfatal),
syncope in four, and
presyncope in six. The addition of beta-blockers was ineffective in suppressing the recurrent symptoms. The control of symptoms required more extensive
sympathectomy (three patients), chronic atrial pacing (three patients), and implantation of an automatic internal
defibrillator (one patient). Only one patient has remained asymptomatic without
drug or pacemaker
therapy. In conclusion, left cervicothoracic
sympathectomy proved inadequate for long-term control of symptoms in most patients with the
long QT syndrome. These patients usually required concomitant drugs, more extensive surgery, or long-term cardiac pacing for symptomatic relief.