OBJECTIVES: The aims of this study were: 1) to evaluate risk factors influencing the clinical course of mutation-confirmed adult patients with long QT syndrome (LQTS), 2) to study life-threatening cardiac events as a specific end point in adults, and 3) to examine the protective effect of beta-blocker therapy on cardiac events in adult LQTS patients with known cardiac channel mutations. BACKGROUND: The clinical course and risk factors for cardiac events in genotype-confirmed adult patients with LQTS have not been previously investigated. METHODS: The clinical characteristics of 812 mutation-confirmed LQTS patients age 18 years or older were studied with both univariate and multivariate analyses to determine the genotype-phenotype factors that influence the clinical course of adult patients with this disorder. RESULTS: Female gender, corrected QT (QTc) interval, LQT2 genotype, and frequency of cardiac events before age 18 years were associated with increased risk of having any cardiac events between the ages of 18 and 40 years. Female gender, QTc interval > or =500 ms, and interim syncopal events during follow-up after age 18 years were associated with significantly increased risk of life-threatening cardiac events in adulthood. Beta-blockers provided a 60% reduction in risk of any cardiac event and life-threatening events, with somewhat greater effect in higher-risk subjects. CONCLUSIONS: The severity of LQTS in adulthood can be risk stratified with information regarding genotype, gender, QTc duration, and history of cardiac events. Beta-blockers effectively reduce but do not eliminate the risk of both syncopal and life-threatening cardiac events in adult patients with mutation-confirmed LQTS.
OBJECTIVES: The aims of this study were: 1) to evaluate risk factors influencing the clinical course of mutation-confirmed adult patients with long QT syndrome (LQTS), 2) to study life-threatening cardiac events as a specific end point in adults, and 3) to examine the protective effect of beta-blocker therapy on cardiac events in adult LQTS patients with known cardiac channel mutations. BACKGROUND: The clinical course and risk factors for cardiac events in genotype-confirmed adult patients with LQTS have not been previously investigated. METHODS: The clinical characteristics of 812 mutation-confirmed LQTS patients age 18 years or older were studied with both univariate and multivariate analyses to determine the genotype-phenotype factors that influence the clinical course of adult patients with this disorder. RESULTS: Female gender, corrected QT (QTc) interval, LQT2 genotype, and frequency of cardiac events before age 18 years were associated with increased risk of having any cardiac events between the ages of 18 and 40 years. Female gender, QTc interval > or =500 ms, and interim syncopal events during follow-up after age 18 years were associated with significantly increased risk of life-threatening cardiac events in adulthood. Beta-blockers provided a 60% reduction in risk of any cardiac event and life-threatening events, with somewhat greater effect in higher-risk subjects. CONCLUSIONS: The severity of LQTS in adulthood can be risk stratified with information regarding genotype, gender, QTc duration, and history of cardiac events. Beta-blockers effectively reduce but do not eliminate the risk of both syncopal and life-threatening cardiac events in adult patients with mutation-confirmed LQTS.
Authors: Peter A Noseworthy; Aki S Havulinna; Kimmo Porthan; Annukka M Lahtinen; Antti Jula; Pekka J Karhunen; Markus Perola; Lasse Oikarinen; Kimmo K Kontula; Veikko Salomaa; Christopher Newton-Cheh Journal: Circ Cardiovasc Genet Date: 2011-04-21
Authors: Lars Anneken; Stefan Baumann; Patrick Vigneault; Peter Biliczki; Corinna Friedrich; Ling Xiao; Zenawit Girmatsion; Ina Takac; Ralf P Brandes; Stefan Kissler; Inka Wiegratz; Sven Zumhagen; Birgit Stallmeyer; Stefan H Hohnloser; Thomas Klingenheben; Eric Schulze-Bahr; Stanley Nattel; Joachim R Ehrlich Journal: Eur Heart J Date: 2015-08-12 Impact factor: 29.983
Authors: Ilan Goldenberg; Samuel Horr; Arthur J Moss; Coeli M Lopes; Alon Barsheshet; Scott McNitt; Wojciech Zareba; Mark L Andrews; Jennifer L Robinson; Emanuela H Locati; Michael J Ackerman; Jesaia Benhorin; Elizabeth S Kaufman; Carlo Napolitano; Pyotr G Platonov; Silvia G Priori; Ming Qi; Peter J Schwartz; Wataru Shimizu; Jeffrey A Towbin; G Michael Vincent; Arthur A M Wilde; Li Zhang Journal: J Am Coll Cardiol Date: 2011-01-04 Impact factor: 24.094
Authors: Arthur A M Wilde; Arthur J Moss; Elizabeth S Kaufman; Wataru Shimizu; Derick R Peterson; Jesaia Benhorin; Coeli Lopes; Jeffrey A Towbin; Carla Spazzolini; Lia Crotti; Wojciech Zareba; Ilan Goldenberg; Jørgen K Kanters; Jennifer L Robinson; Ming Qi; Nynke Hofman; David J Tester; Connie R Bezzina; Marielle Alders; Takeshi Aiba; Shiro Kamakura; Yoshihiro Miyamoto; Mark L Andrews; Scott McNitt; Bronislava Polonsky; Peter J Schwartz; Michael J Ackerman Journal: Circulation Date: 2016-08-26 Impact factor: 29.690
Authors: James E Tisdale; Heather A Jaynes; Joanna R Kingery; Brian R Overholser; Noha A Mourad; Tate N Trujillo; Richard J Kovacs Journal: Circ Cardiovasc Qual Outcomes Date: 2014-05-06