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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.

Efficacy of left cardiac sympathectomy in the treatment of patients with the long QT syndrome.