Abstract 14217: Long Term Efficacy of Atrial Based Pacing and Beta Blocker Therapy to Prevent Sudden Cardiac Death in Congenital Long Qt Syndrome
Introduction: Conflicting data exists for congenital long QT syndrome (LQTS) treated with a combination of beta blockade and continuous pacing. High risk patients are usually treated with an implantable cardiac defibrillator (ICD) exposing them to the risk of inappropriate shocks and potentially more frequent generator replacements. Appropriate therapy also has been shown to trigger ICD storm in many cases due to increased sympathetic discharge. We sought to examine the outcome of a cohort of patients at our institution treated with the combination of a beta blocker and atrial based pacing at a higher rate than previously described.
Methods: The long term clinical course of patients with LQTS treated with beta blockers and atrial pacing were examined to identify recurrent syncope, cardiac arrest, sudden cardiac death or the need to upgrade to a defibrillator.
Results: A total of 30 patients with LQTS (age 26.8 ± 21.3 years; 21 females) were identified with varying presentations - syncope (n=16); torsade de pointes (n=7); cardiac arrest (n=6); family history (n=1). All patients had a pacemaker implanted and arrhythmia diagnostics turned on. Lower rate was set to a mean of 89 ± 9 bpm [ ≥ 90 (n=20); ≥80 (n=9); 70 (n=1)]. 21 patients were programmed in the AAI mode, with the rest in the DDD (n=6) or DDI (n=3) modes. Over a mean follow up of 9.7 ± 6.6 years, no patient had recurrent syncope, cardiac arrest or sudden cardiac death. None of the patients required upgrade to an ICD. 4 patients died during follow up from known unrelated causes.
Conclusion: A combination of beta blocker therapy and atrial based pacing at a high rate is highly effective in preventing sudden death in patients with congenital LQTS. This approach may be particularly beneficial in the young population, in whom, implantation of a pacemaker rather than an ICD may enhance device longevity and avoid inappropriate ICD therapies.
- © 2013 by American Heart Association, Inc.