1. High Efficacy of β-Blockers in Long-QT Syndrome Type 1
- Author
-
Candice Bithell, Silvia G. Priori, Carlo Napolitano, Li Zhang, Heikki Swan, Carla Spazzolini, Peter J. Schwartz, Isabelle Denjoy, G. Michael Vincent, Kirsi Piippo, Villain E, Lia Crotti, Jean Marc Lupoglazoff, Vincent, G, Schwartz, P, Denjoy, I, Swan, H, Bithell, C, Spazzolini, C, Crotti, L, Piippo, K, Lupoglazoff, J, Villain, E, Priori, S, Napolitano, C, and Zhang, L
- Subjects
Adult ,Male ,medicine.medical_specialty ,Adolescent ,Drug-Related Side Effects and Adverse Reactions ,Romano-Ward Syndrome ,Long QT syndrome ,Adrenergic beta-Antagonists ,030204 cardiovascular system & hematology ,Treatment failure ,Young Adult ,03 medical and health sciences ,0302 clinical medicine ,Physiology (medical) ,Internal medicine ,medicine ,Humans ,Treatment Failure ,030212 general & internal medicine ,Patient compliance ,long qt syndrome, beta-blockers, drugs, long qt syndrome type 1 ,Retrospective Studies ,Syndrome type ,business.industry ,Follow up studies ,Retrospective cohort study ,MED/11 - MALATTIE DELL'APPARATO CARDIOVASCOLARE ,Middle Aged ,medicine.disease ,3. Good health ,Romano–Ward syndrome ,Pharmaceutical Preparations ,Anesthesia ,Mutation ,Cardiology ,Patient Compliance ,Female ,Cardiology and Cardiovascular Medicine ,business ,Follow-Up Studies - Abstract
Background— β-Blocker efficacy in long-QT syndrome type 1 is good but variably reported, and the causes of cardiac events despite β-blocker therapy have not been ascertained. Methods and Results— This was a retrospective study of the details surrounding cardiac events in 216 genotyped long-QT syndrome type 1 patients treated with β-blocker and followed up for a median time of 10 years. Before β-blocker, cardiac events occurred in 157 patients (73%) at a median age of 9 years, with cardiac arrest (CA) in 26 (12%). QT-prolonging drugs were used by 17 patients; 9 of 17 (53%) had CA compared with 17 of 199 nonusers (8.5%; odds ratio, 12.0; 95% confidence interval, 4.1 to 35.3; P P P =0.001). None of the 26 patients with CA before β-blocker had CA/sudden death on β-blockers. Conclusions— β-Blockers are extremely effective in long-QT syndrome type 1 and should be administered at diagnosis and ideally before the preteen years. β-Blocker noncompliance and use of QT-prolonging drug are responsible for almost all life-threatening “β-blocker failures.” β-Blockers are appropriate therapy for asymptomatic patients and those who have never had a CA or β-blocker therapy. Routine implantation of cardiac defibrillators in such patients does not appear justified.
- Published
- 2009