(Circulation. 1999;100:2431.)
© 1999 American Heart Association, Inc.
Clinical Investigation and Reports |
Long-Term Follow-Up of Patients With Long-QT Syndrome Treated With ß-Blockers and Continuous Pacing
From the Divisions of Electrophysiology and Pediatric Cardiology, University of California, San Francisco.
Correspondence to Parvin C. Dorostkar, MD, Division of Pediatric Cardiology, Rainbow Babies and Children’s Hospital, 11100 Euclid Ave, Cleveland, OH 44106.
Background—The long-QT syndrome is associated with sudden cardiac death. Combination of ß-blocker and pacing therapy has been proposed for treatment of drug-resistant patients. The purpose of this study was to summarize our long-term experience with combined therapy in patients with long-QT syndrome.
Methods and Results—A total of 37 patients with idiopathic long-QT syndrome were treated with combined therapy consisting of continuous cardiac pacing and maximally tolerated ß-blocker therapy and followed up for 6.3±4.6 years (mean±SD). The group consisted of 32 female and 5 male patients with a mean age of 31.6 years. The mean paced rate was 82±7 bpm (range, 60 to 100 bpm). On follow-up, recurrent symptoms caused by pacemaker malfunction were documented in 3 patients. Four patients died during the follow-up period: 2 adolescents stopped ß-blocker therapy, 1 patient died suddenly while treated with combined therapy, and 1 patient died of unrelated causes. In addition, 3 patients had resuscitated cardiac arrest while on combined therapy, and 1 patient had repeated, appropriate implantable cardioverter-defibrillator discharges on follow-up.
Conclusions—Because 28 of 37 patients remain without symptoms with ß-blocker therapy and continuous pacing, combined therapy appears to provide reasonable, long-term control for this high-risk group. However, the incidence of sudden death and aborted sudden death (24% in all patients and 17% in compliant patients) strongly suggests the use of a "back-up" defibrillator, particularly in noncompliant adolescent patients. Implantable cardioverter-defibrillator therapy, however, may be associated with recurrent shocks in susceptible patients.
Key Words: syncope • death, sudden • cardioversion • defibrillation • pacemakers
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