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• W2771709793 abstract "HomeCirculationVol. 136, No. 23Letter by Jin-shan and Xue-bin Regarding Article, “Genotype-Phenotype Correlation of SCN5A Mutation for the Clinical and Electrocardiographic Characteristics of Probands With Brugada Syndrome: A Japanese Multicenter Registry” Free AccessLetterPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessLetterPDF/EPUBLetter by Jin-shan and Xue-bin Regarding Article, “Genotype-Phenotype Correlation of SCN5A Mutation for the Clinical and Electrocardiographic Characteristics of Probands With Brugada Syndrome: A Japanese Multicenter Registry” He Jin-shan, MD and Li Xue-bin, MD He Jin-shanHe Jin-shan Department of Cardiology, Beijing Key Laboratory of Early Prediction and Intervention of Acute Myocardial Infarction, Peking University People’s Hospital, Beijing, China. Search for more papers by this author and Li Xue-binLi Xue-bin Department of Cardiology, Beijing Key Laboratory of Early Prediction and Intervention of Acute Myocardial Infarction, Peking University People’s Hospital, Beijing, China. Search for more papers by this author Originally published5 Dec 2017https://doi.org/10.1161/CIRCULATIONAHA.117.030553Circulation. 2017;136:2287–2288To the Editor:We read with great interest the article by Yamagata et al1 about the genotype-phenotype correlation of SCN5A mutations for the clinical and electrocardiographic characteristics of probands with Brugada syndrome, which indicates that patients with Brugada syndrome with SCN5A mutations exhibit more conduction abnormalities on ECG and have a higher risk for cardiac events. This study has a great effect on making the genotype-phenotype correlation of SCN5A mutations as a predictor of cardiac events in Brugada syndrome, which can improve the risk stratification systems for Brugada syndrome. However, we have 3 questions about this study. First, patients with SCN5A mutations have a higher risk for cardiac events, but there is a trend toward a lower rate of VF/VT inducibility in them during the EP study. What is the reason for this discrepancy? Why are patients with SCN5A mutations difficult to induce malignant arrhythmia in with rigorous stimulation but are prone to cardiac events in real life? Second, patients with SCN5A mutations exhibit more conduction abnormalities on ECG and have a higher risk for cardiac events in this study. What is the relationship between conduction abnormalities and higher cardiac event risks? Are they both the presentations of SCN5A mutations? Or do SCN5A mutations cause conduction abnormalities first and then conduction abnormalities make cardiac events risk higher? Third, SCN5A mutations are related to Brugada syndrome as well as other inherited cardiomyopathies such as dilated cardiomyopathy, long QT syndrome type 3, and arrhythmogenic right ventricular dysplasia.2,3 Do these patients also have a higher risk for cardiac events? Or is this effect confined only to Brugada syndrome? We hope to have a profound understanding of Brugada syndrome with these questions.He Jin-shan, MDLi Xue-bin, MDDisclosuresNone.FootnotesCirculation is available at http://circ.ahajournals.org.References1. Yamagata K, Horie M, Aiba T, Ogawa S, Aizawa Y, Ohe T, Yamagishi M, Makita N, Sakurada H, Tanaka T, Shimizu A, Hagiwara N, Kishi R, Nakano Y, Takagi M, Makiyama T, Ohno S, Fukuda K, Watanabe H, Morita H, Hayashi K, Kusano K, Kamakura S, Yasuda S, Ogawa H, Miyamoto Y, Kapplinger JD, Ackerman MJ, Shimizu W. Genotype-phenotype correlation of SCN5A mutation for the clinical and electrocardiographic characteristics of probands with Brugada syndrome: a Japanese multicenter registry.Circulation. 2017; 135:2255–2270. doi: 10.1161/CIRCULATIONAHA.117.027983.LinkGoogle Scholar2. McNair WP, Ku L, Taylor MR, Fain PR, Dao D, Wolfel E, Mestroni L; Familial Cardiomyopathy Registry Research Group. SCN5A mutation associated with dilated cardiomyopathy, conduction disorder, and arrhythmia.Circulation. 2004; 110:2163–2167. doi: 10.1161/01.CIR.0000144458.58660.BB.LinkGoogle Scholar3. Remme CA. Cardiac sodium channelopathy associated with SCN5A mutations: electrophysiological, molecular and genetic aspects.J Physiol. 2013; 591:4099–4116. doi: 10.1113/jphysiol.2013.256461.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetails December 5, 2017Vol 136, Issue 23 Advertisement Article InformationMetrics © 2017 American Heart Association, Inc.https://doi.org/10.1161/CIRCULATIONAHA.117.030553PMID: 29203569 Originally publishedDecember 5, 2017 PDF download Advertisement SubjectsCardiomyopathyRisk Factors" @default.
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