Long-Term Follow-Up of Patients With Long-QT Syndrome Treated With β-Blockers and Continuous Pacing

Abstract

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.

Genotype-Specific Onset of Arrhythmias in Congenital Long-QT Syndrome

“…15,16 This distinction may reflect different electrophysiological mechanisms and affect the therapeutic efficacy of ␤-blockers. Identification of those patients who have pause-dependent TdP gains significance because these patients may benefit from ancillary treatment with pacemakers, [17][18][19] which use algorithms to prevent bradycardias and pauses. Accordingly, we studied whether differences exist in pause dependence of TdP onset between LQT1, LQT2, and LQT3.…”

Electrical heart disease: Genetic and molecular basis of cardiac arrhythmias in normal structural hearts

“…Undoubtedly, the use of betablockers has proven effective in reducing cardiac events in patients with genetically proven LQT1 and LQT2. However, breakthrough events may still occur, particularly in patients with events before treatment (31,32), and frank discussion of the option of an implantable cardioverter-defibrillator is always reasonable.…”

Primary Prevention of Sudden Death With Implantable Defibrillator Therapy in Patients With Cardiac Disease

“…44 On the basis of these and other data, clinicians may elect to choose an ICD for LQTS patients with a prior cardiac arrest or in asymptomatic patients with LQTS and a worrisome family history. 43 The presence of specific genetic mutations (eg, LQT 3) may provide prognostic information related to the risk of SCD 45 but remains to be confirmed.…”