The elusive link between LQT3 and Brugada syndrome: the role of flecainide challenge.
作者： Silvia G. Priori , Carlo Napolitano , Peter J. Schwartz , Raffaella Bloise , Lia Crotti
关键词: Internal medicine 、 Sodium channel blocker 、 ST segment 、 Flecainide 、 Heart block 、 Heart disease 、 Anesthesia 、 Medicine 、 Cardiology 、 Long QT syndrome 、 QT interval 、 Brugada syndrome
摘要: Background—Defects of the SCN5A gene encoding cardiac sodium channel are associated with both LQT3 subtype long-QT syndrome and Brugada (BS). The typical manifestations (QT interval prolongation) BS (ST segment elevation in leads V1 through V3) may coexist same patients, which raises questions about actual differences between BS. Intravenous flecainide is standard provocative test used to unmask individuals concealed forms disease, oral has been proposed as a treatment option for patients because it shorten their QT interval. Methods Results—We tested possibility that some might not only interval, but also produce an ST segment. A total 13 from 7 families received intravenous using protocol As expected, QT, QTc, JT, JTc shortening was observed 12 and...
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sci-hub.se 参考文章(16)
Qiuyun Chen, Glenn E. Kirsch, Danmei Zhang, Ramon Brugada, Josep Brugada, Pedro Brugada, Domenico Potenza, Angel Moya, Martin Borggrefe, Günter Breithardt, Rocio Ortiz-Lopez, Zhiqing Wang, Charles Antzelevitch, Richard E. O'Brien, Eric Schulze-Bahr, Mark T. Keating, Jeffrey A. Towbin, Qing Wang, Genetic basis and molecular mechanism for idiopathic ventricular fibrillation Nature. ,vol. 392, pp. 293- 296 ,(1998) , 10.1038/32675
Douglas P. Zipes, José Jalife, Cardiac Electrophysiology : From Cell to Bedside ,(1990)
P J Schwartz, A J Moss, G M Vincent, R S Crampton, Diagnostic criteria for the long QT syndrome. An update. Circulation. ,vol. 88, pp. 782- 784 ,(1993) , 10.1161/01.CIR.88.2.782
Silvia G. Priori, Carlo Napolitano, Peter J. Schwartz, Low Penetrance in the Long-QT Syndrome Clinical Impact Circulation. ,vol. 99, pp. 529- 533 ,(1999) , 10.1161/01.CIR.99.4.529
G. Michael Vincent, Katherine W. Timothy, Mark Leppert, Mark Keating, The Spectrum of Symptoms and QT Intervals in Carriers of the Gene for the Long-QT Syndrome New England Journal of Medicine. ,vol. 327, pp. 846- 852 ,(1992) , 10.1056/NEJM199209173271204
Silvia G. Priori, Carlo Napolitano, Francesco Cantù, Arthur M. Brown, Peter J. Schwartz, Differential Response to Na+ Channel Blockade, β-Adrenergic Stimulation, and Rapid Pacing in a Cellular Model Mimicking the SCN5A and HERG Defects Present in the Long-QT Syndrome Circulation Research. ,vol. 78, pp. 1009- 1015 ,(1996) , 10.1161/01.RES.78.6.1009
Duangrurdee Wattanasirichaigoon, Mark R. Vesely, Priya Duggal, Jami C. Levine, Elizabeth D. Blume, Grace S. Wolff, Sam B. Edwards, Alan H. Beggs, Sodium channel abnormalities are infrequent in patients with long QT syndrome: identification of two novel SCN5A mutations. American Journal of Medical Genetics. ,vol. 86, pp. 470- 476 ,(1999) , 10.1002/(SICI)1096-8628(19991029)86:5<470::AID-AJMG13>3.0.CO;2-Y
Robert Dumaine, Qing Wang, Mark T. Keating, Hali A. Hartmann, Peter J. Schwartz, Arthur M. Brown, Glenn E. Kirsch, Multiple Mechanisms of Na+ Channel– Linked Long-QT Syndrome Circulation Research. ,vol. 78, pp. 916- 924 ,(1996) , 10.1161/01.RES.78.5.916
Peter J. Schwartz, Silvia G. Priori, Emanuela H. Locati, Carlo Napolitano, Francesco Cantù, Jeffrey A. Towbin, Mark T. Keating, Hicham Hammoude, Arthur M. Brown, Ling-Sing K. Chen, Thomas J. Colatsky, Long QT syndrome patients with mutations of the SCN5A and HERG genes have differential responses to Na+ channel blockade and to increases in heart rate : implications for gene-specific therapy Circulation. ,vol. 92, pp. 3381- 3386 ,(1995) , 10.1161/01.CIR.92.12.3381
Ramon Brugada, Josep Brugada, Charles Antzelevitch, Glenn E. Kirsch, Domenico Potenza, Jeffrey A. Towbin, Pedro Brugada, Sodium Channel Blockers Identify Risk for Sudden Death in Patients With ST-Segment Elevation and Right Bundle Branch Block but Structurally Normal Hearts Circulation. ,vol. 101, pp. 510- 515 ,(2000) , 10.1161/01.CIR.101.5.510