High prevalence of early repolarization in short QT syndrome

Watanabe, Hiroshi; Makiyama, Takeru; Koyama, T; Kannankeril, Prince J.; Seto, Shinji; Okamura, Kazuki; Oda, Hirotaka; Itoh, Hideki; Okada, Masahiko; Tanabe, Naohito; Yagihara, Nobue; Kamakura, Shiro; Horie, Minoru; Aizawa, Yoshifusa; Shimizu, Wataru

doi:10.1016/j.hrthm.2010.01.012

Cited by 152 publications

(84 citation statements)

References 46 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…18,39 Third, ERP has been observed as a feature of other genetic arrhythmia syndromes such as Brugada syndrome [40][41][42] and short-QT syndrome. 43 Furthermore, some genetic variants reported to be associated with ERP have been associated with other arrhythmia syndromes, including Brugada syndrome. 44,45 Indeed, rare genetic variants in genes governing cardiac repolarization have been observed in candidate gene screening studies performed in affected individuals, including KCNJ8, 30,45 SCN5A, 32 and L-type calcium channel subunits.…”

Section: Biological Basis and Geneticsmentioning

confidence: 99%

Electrocardiographic Early Repolarization

Patton¹,

Ellinor²,

Ezekowitz³

et al. 2016

Circulation

102

View full text Add to dashboard Cite

Section: Biological Basis and Geneticsmentioning

confidence: 99%

Electrocardiographic Early Repolarization

Patton¹,

Ellinor²,

Ezekowitz³

et al. 2016

Circulation

102

View full text Add to dashboard Cite

“…96 A recent clinical study reported a high prevalence of early repolarization in patients with SQTS associated with arrhythmic events. 98 An implantable cardioverter-defibrillator is the most reliable therapy for secondary prevention in SQTS patients with a history of VF or aborted sudden cardiac death. As an adjunctive medication, quinidine has been reported to normalize the QT interval and T-wave morphology and to suppress the induction of VF during electrophysiological study in patients with SQT1 99 ; however, it is not clear whether the specific efficacy of quinidine observed in SQT1 patients was genotype specific or mutation specific.…”

Section: Genotype-phenotype Correlations In Sqtsmentioning

confidence: 99%

Phenotypic Manifestations of Mutations in Genes Encoding Subunits of Cardiac Potassium Channels

Shimizu

Horie

2011

Circulation Research

Self Cite

View full text Add to dashboard Cite

Abstract:Since 1995, when a potassium channel gene, hERG (human ether-à-go-go-related gene), now referred to as KCNH2, encoding the rapid component of cardiac delayed rectifier potassium channels was identified as being responsible for type 2 congenital long-QT syndrome, a number of potassium channel genes have been shown to cause different types of inherited cardiac arrhythmia syndromes. These include congenital long-QT syndrome, short-QT syndrome, Brugada syndrome, early repolarization syndrome, and familial atrial fibrillation. Genotype-phenotype correlations have been investigated in some inherited arrhythmia syndromes, and as a result, gene-specific risk stratification and gene-specific therapy and management have become available, particularly for patients with congenital long-QT syndrome. In this review article, the molecular structure and function of potassium channels, the clinical phenotype due to potassium channel gene mutations, including genotype-phenotype correlations, and the diverse mechanisms underlying the potassium channel gene-related diseases will be discussed. (Circ Res. 2011;109:97-109.)

show abstract

“…47 A high prevalence of ER in patients with short QT syndrome has been reported (65%). 48 In multivariate models, ER was associated with arrhythmic events. Another study reported that among patients with idiopathic VF and ER, the QT interval was shorter compared with those with idiopathic VF but without ER, postulating an association between ER and QT interval shortening.…”

Section: Early Repolarization Modifying Risk Of Underlying Cardiac Pamentioning

confidence: 98%