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Publication Details
AFRICAN RESEARCH NEXUS
SHINING A SPOTLIGHT ON AFRICAN RESEARCH
medicine
High efficacy of β-blockers in long-QT syndrome type 1: Contribution of noncompliance and QT-prolonging drugs to the occurrence of β-blocker treatment "failures"
Circulation, Volume 119, No. 2, Year 2009
Notification
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Description
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<0.001). After β-blocker, 75% were asymptomatic, and cardiac events were significantly reduced (P<0.001), with a median event count (quartile 1 to 3) per person of 0 (0 to 1). Twelve patients (5.5%) suffered CA/sudden death, but 11 of 12 (92%) were noncompliant (n=8), were on a QT-prolonging drug (n=2), or both (n=1) at the time of the event. The risk for CA/sudden death in compliant patients not taking QT-prolonging drugs was dramatically less compared with noncompliant patients on QT-prolonging drugs (odds ratio, 0.03; 95% confidence interval, 0.003 to 0.22; 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. © 2009 American Heart Association, Inc.
Authors & Co-Authors
Vincent, G. Michael
United States, Salt Lake City
The University of Utah
Schwartz, Peter J.
Italy, Pavia
Fondazione Irccs Policlinico San Matteo
Italy, Pavia
Università Degli Studi Di Pavia
Italy, Milan
Irccs Istituto Auxologico Italiano
South Africa, Cape Town
University of Cape Town
Denjoy, Isabelle
France, Paris
Hôpital Lariboisiere Ap-hp
Swan, Heikki
Finland, Helsinki
Helsingin Yliopisto
Bithell, Candice
United States, Salt Lake City
The University of Utah
Spazzolini, Carla
Italy, Pavia
Fondazione Irccs Policlinico San Matteo
CROTTI, L.
Italy, Pavia
Fondazione Irccs Policlinico San Matteo
Piippo, Kirsi
Finland, Helsinki
Helsingin Yliopisto
Lupoglazoff, Jean Marc
France, Paris
Hôpital Robert-debré Ap-hp
Villain, Elisabeth
France, Paris
Hôpital Necker Enfants Malades
Priori, Silvia G.
Italy, Pavia
Università Degli Studi Di Pavia
Italy, Pavia
Istituti Clinici Scientifici Maugeri Spa – sb
Napolitano, Carlo
Italy, Pavia
Istituti Clinici Scientifici Maugeri Spa – sb
Zhang, Li
United States, Salt Lake City
The University of Utah
Statistics
Citations: 254
Authors: 13
Affiliations: 10
Identifiers
Doi:
10.1161/CIRCULATIONAHA.108.772533
ISSN:
00097322
e-ISSN:
15244539
Research Areas
Noncommunicable Diseases
Study Design
Cohort Study
Case-Control Study