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. 2004 Sep 15;292(11):1341-4.
doi: 10.1001/jama.292.11.1341.

Association of long QT syndrome loci and cardiac events among patients treated with beta-blockers

Silvia G Priori  1 , Carlo Napolitano, Peter J Schwartz, Massimiliano Grillo, Raffaella Bloise, Elena Ronchetti, Cinzia Moncalvo, Chiara Tulipani, Alessia Veia, Georgia Bottelli, Janni Nastoli
Affiliations

Association of long QT syndrome loci and cardiac events among patients treated with beta-blockers

Silvia G Priori et al. JAMA. .

Abstract

Context: Data on the efficacy of beta-blockers in the 3 most common genetic long QT syndrome (LQTS) loci are limited.

Objective: To describe and assess outcome in a large systematically genotyped population of beta-blocker-treated LQTS patients.

Design, setting, and patients: Consecutive LQTS-genotyped patients (n = 335) in Italy treated with beta-blockers for an average of 5 years.

Main outcome measures: Cardiac events (syncope, ventricular tachycardia/torsades de pointes, cardiac arrest, and sudden cardiac death) while patients received beta-blocker therapy according to genotype.

Results: Cardiac events among patients receiving beta-blocker therapy occurred in 19 of 187 (10%) LQT1 patients, 27 of 120 (23%) LQT2 patients, and 9 of 28 (32%) LQT3 patients (P<.001). The risk of cardiac events was higher among LQT2 (adjusted relative risk, 2.81; 95% confidence interval [CI], 1.50-5.27; P =.001) and LQT3 (adjusted relative risk, 4.00; 95% CI, 2.45-8.03; P<.001) patients than among LQT1 patients, suggesting inadequate protection from beta-blocker therapy. Other important predictors of risk were a QT interval corrected for heart rate that was more than 500 ms in patients receiving therapy (adjusted relative risk, 2.01; 95% CI, 1.16-3.51; P =.01) and occurrence of a first cardiac event before the age of 7 years (adjusted RR, 4.34; 95% CI, 2.35-8.03; P<.001).

Conclusion: Among patients with genetic LQTS treated with beta-blockers, there is a high rate of cardiac events, particularly among patients with LQT2 and LQT3 genotypes.

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