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. Combination of beta-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.


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 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 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-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.
Dorostkar
P C
PC
Divisions of Electrophysiology and Pediatric Cardiology, University of California, San Francisco, USA.
Eldar
M
M
Belhassen
B
B
Scheinman
M M
MM
D000293:Adolescent; D000319:Adrenergic beta-Antagonists / Q000008:administration & dosage*; D000328:Adult; D000368:Aged; D001262:Atenolol / Q000008:administration & dosage; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003645:Death, Sudden; D004554:Electric Countershock; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008133:Long QT Syndrome / Q000188:drug therapy* / Q000401:mortality*; D008297:Male; D008790:Metoprolol / Q000008:administration & dosage; D008875:Middle Aged; D009248:Nadolol / Q000008:administration & dosage; D010138:Pacemaker, Artificial; D011379:Prognosis; D011433:Propranolol / Q000008:administration & dosage*; D018570:Risk Assessment; D016019:Survival Analysis; D016896:Treatment Outcome
D000319:Adrenergic beta-Antagonists; D009248:Nadolol; D001262:Atenolol; D011433:Propranolol; D008790:Metoprolol