Long-term follow-up of patients with long-QT syndrome treated with beta-blockers and continuous pacing

Background-The long-QT syndrome is associated with sudden cardiac death. Co mbination of P-blocker and pacing therapy has been proposed for treatment o f drug-resistant patients. The purpose of this study was to summarize our l ong-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 beta-blocker therapy and followed up for 6.3+/-4.6 years (mean+/-SD). The group consisted of 32 female and 5 male patients wi th 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 malfuncti on were documented in 3 patients. Four patients died during the follow-up p eriod: 2 adolescents stopped beta-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 beta-blo cker therapy and continuous pacing, combined therapy appears to provide rea sonable, 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 c ompliant 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.