THERAPY AND PREVENTION Efficacy of left cardiac sympathectomy in the treatment of patients with the long QT syndrome

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 /3-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 < .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 /3-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. Circulation 70, No. 6, 1018-1023, 1984. THE ASSOCIATION of idiopathic prolongation of the QT interval with recurrent attacks of syncope and cardiac arrest is known as the long QT syndrome. 6 The mortality rate in untreated patients is thought to be as high as 78%.) Although /3-blockers have proved valuable in the long-term management of patients with this syndrome, at least 6% of patients fail to respond to 13-blockade.,23.5 Left cervicothoracic sympathectomy has been reported to be the treatment of choice for patients with this syndrome who do not respond to pharmacologic treatment.7-' Although the available data suggest that sympathectomy is highly effective in suppressing episodes of syncope and cardiac arrest caused by ventricular tachycardia, long-term follow-up data for patients with the long QT syndrome who have undergone sympathectomy are scanty. In prior reports, changes in the QT interval after sympathectomy were variable and were not predictive of clinical outcome. From the Department of Medicine and the Cardiovascular Research Institute, University of California, San Francisco, and Stanford University Medical Center, Stanford; the University of Utah Medical Center, Salt Lake City; and the University of Michigan, Ann Arbor. Address for correspondence: Anil K. Bhandari, M.D., University of Southern California School of Medicine, Section of Cardiology, 2025 Zonal Ave., Los Angeles, CA 90033. Received April 30, 1984; revision accepted Aug. 23, 1984. 1018 This report deals with 10 consecutive patients with the long QT syndrome who underwent sympathectomy over a 6 year period. The study aimed to assess the long-term effects of sympathectomy on changes in the corrected QT interval (QTc), ventricular arrhythmias, and symptoms. Materials and methods The study was a retrospective analysis of 10 patients with the long QT syndrome who underwent left cervicothoracic sympathectomy during the years 1977 to 1982 at either the University of California Medical Center, San Francisco (six patients), or Stanford Medical Center (four patients). During this period we evaluated a total of 18 symptomatic patients with a definite diagnosis of the long QT syndrome: eight responded to the /blockers but 10 required sympathectomy because of a poor response to the /3-blockers or severe manifestations of the syndrome. The study population comprised the latter 10 patients. The clinical characteristics of the 10 patients are summarized in table 1. There were nine women and one man, with an age range of 13 to 56 years (30.5 + 12). The syndrome was familial in four and idiopathic in six. All patients had a history of cardiac arrest (nine patients), syncope (nine patients), or both (eight patients). Polymorphous ventricular tachycardia or fibrillation was documented as the cause of symptoms in each patient. Based on the history, physical examination, electrocardiogram, chest x-ray, two-dimensional echocardiogram (three patients), and cardiac catheterization with coronary angiography (three patients), seven patients had no demonstrable structural heart disease, two had mild systemic hypertension without left ventricular hypertrophy, and one had mitral valve prolapse. Seven CIRCULATION by gest on A ril 4, 2017 http://ciajournals.org/ D ow nladed from THERAPY AND PREVENTION-ARRHYTHMIA