February 15, 2000Circulation844 citationsOpen Access
Effectiveness and Limitations of β-Blocker Therapy in Congenital Long-QT Syndrome
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Key Result
Beta-blocker therapy in congenital long-QT syndrome significantly reduced cardiac events from 0.97 to 0.31 events per year in probands, though symptomatic patients remained at high risk of recurrence.
Key Points
- To evaluate the clinical effectiveness and limitations of beta-blocker therapy in reducing cardiac events in patients with congenital long-QT syndrome.
- Analyzed 869 patients with congenital long-QT syndrome (LQTS) receiving prescribed beta-blocker therapy.
- Evaluated outcomes during matched 5-year periods before and after therapy initiation and performed survivorship analyses to determine predictors of on-treatment events.
- Beta-blocker initiation significantly decreased annual cardiac event rates from 0.97±1.42 to 0.31±0.86 in probands and from 0.26±0.84 to 0.15±0.69 in affected family members (both P<0.001).
- Patients with cardiac symptoms prior to therapy (n=598) had a significantly higher risk of recurrent events during treatment (HR 5.8; 95% CI, 3.7 to 9.1), with a 5-year recurrence rate of 32%.
- Patients with prior aborted cardiac arrest (n=113) faced substantial risk of recurrent arrest or death on therapy (HR 12.9; 95% CI, 4.7 to 35.5), with 14% having a recurrence within 5 years.
Study Design
Type
Cohort (n=869)
Structured PICO
Does beta-blocker therapy reduce the rate of cardiac events in patients with congenital long-QT syndrome?
P
Population
869 patients with congenital long-QT syndrome (LQTS)
I
Intervention
Beta-blocker therapy
C
Comparator
Matched periods before starting beta-blocker therapy (within-patient comparison)
O
Outcome
Rate of cardiac events (syncope, aborted cardiac arrest, or death) during 5-year matched periodscomposite
Beta-blockers significantly reduce cardiac events in congenital LQTS, but previously symptomatic patients remain at substantial risk for recurrent life-threatening events.
Main Result
Absolute Event Rate: 0.31% vs 0.97%
p-value: p=<0.001
Abstract
BACKGROUND: beta-blockers are routinely prescribed in congenital long-QT syndrome (LQTS), but the effectiveness and limitations of beta-blockers in this disorder have not been evaluated. METHODS AND RESULTS: The study population comprised 869 LQTS patients treated with beta-blockers. Effectiveness of beta-blockers was analyzed during matched periods before and after starting beta-blocker therapy, and by survivorship methods to determine factors associated with cardiac events while on prescribed beta-blockers. After initiation of beta-blockers, there was a significant (P<0.001) reduction in the rate of cardiac events in probands (0.97+/-1.42 to 0.31+/-0.86 events per year) and in affected family members (0. 26+/-0.84 to 0.15+/-0.69 events per year) during 5-year matched periods. On-therapy survivorship analyses revealed that patients with cardiac symptoms before beta-blockers (n=598) had a hazard ratio of 5.8 (95% CI, 3.7 to 9.1) for recurrent cardiac events (syncope, aborted cardiac arrest, or death) during beta-blocker therapy compared with asymptomatic patients; 32% of these symptomatic patients will have another cardiac event within 5 years while on prescribed beta-blockers. Patients with a history of aborted cardiac arrest before starting beta-blockers (n=113) had a hazard ratio of 12.9 (95% CI, 4.7 to 35.5) for aborted cardiac arrest or death while on prescribed beta-blockers compared with asymptomatic patients; 14% of these patients will have another arrest (aborted or fatal) within 5 years on beta-blockers. CONCLUSIONS: beta-blockers are associated with a significant reduction in cardiac events in LQTS patients. However, syncope, aborted cardiac arrest, and LQTS-related death continue to occur while patients are on prescribed beta-blockers, particularly in those who were symptomatic before starting this therapy.
Cite This Study
Moss et al. (2000) conducted a cohort in Congenital long-QT syndrome (LQTS) (n=869). beta-blockers vs. matched periods before starting beta-blocker therapy was evaluated on rate of cardiac events in probands (events per year) (p=<0.001). Beta-blocker therapy in congenital long-QT syndrome significantly reduced cardiac events from 0.97 to 0.31 events per year in probands, though symptomatic patients remained at high risk of recurrence.