J‐Wave syndromes expert consensus conference report: Emerging concepts and gaps in knowledge

Antzelevitch, Charles; Yan, Gan Xin; Ackerman, Michael J.; Borggrefe, Martin; Corrado, Domenico; Guo, Jihong; Gussak, Ihor; Hasdemir, Can; Horie, Minoru; Huikuri, Heikki V.; Ma, Changsheng; Morita, Hiroshi; Nam, Gi Byoung; Sacher, Frédéric; Shimizu, Wataru; Viskin, Sami; Wilde, Arthur A.M.

doi:10.1016/j.joa.2016.07.002

Cited by 230 publications

(464 citation statements)

References 354 publications

(553 reference statements)

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“…Syncope is a relatively common presentation in Brugada syndrome (BrS), an inheritable primary arrhythmia syndrome. Considered one of the J wave syndromes, BrS has several clinical and genetic similarities with early repolarization syndrome (ERS) [54]. The region generally most affected in BrS is the anterior right ventricular outflow tract; in ERS, it is the inferior region of the left ventricle [54].…”

Section: Brugada Syndromementioning

confidence: 99%

Syncope and the risk of sudden cardiac death: Evaluation, management, and prevention

Koene

Adkisson

Benditt

2017

Journal of Arrhythmia

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Syncope is a clinical syndrome defined as a relatively brief self-limited transient loss of consciousness (TLOC) caused by a period of inadequate cerebral nutrient flow. Most often the trigger is an abrupt drop of systemic blood pressure. True syncope must be distinguished from other common non-syncope conditions in which real or apparent TLOC may occur such as seizures, concussions, or accidental falls. The causes of syncope are diverse, but in most instances, are relatively benign (e.g., reflex and orthostatic faints) with the main risks being accidents and/or injury. However, in some instances, syncope may be due to more worrisome conditions (particularly those associated with cardiac structural disease or channelopathies); in such circumstances, syncope may be an indicator of increased morbidity and mortality risk, including sudden cardiac death (SCD). Establishing an accurate basis for the etiology of syncope is crucial in order to initiate effective therapy. In this review, we focus primarily on the causes of syncope that are associated with increased SCD risk (i.e., sudden arrhythmic cardiac death), and the management of these patients. In addition, we discuss the limitations of our understanding of SCD in relation to syncope, and propose future studies that may ultimately address how to improve outcomes of syncope patients and reduce SCD risk.

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Section: Brugada Syndromementioning

confidence: 99%

Syncope and the risk of sudden cardiac death: Evaluation, management, and prevention

Koene

Adkisson

Benditt

2017

Journal of Arrhythmia

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“…The J wave appears as a J point elevation or ST‐segment elevation when partially buried in the R wave, which is termed an early repolarization pattern. Arrhythmias associated with early repolarization are considered to represent a continuous spectrum of phenotypic expression referred to as J wave syndrome 2,3 . When the J wave appears in hypothermia, it might contribute to the genesis of ventricular arrhythmias 4 .…”

Section: Introductionmentioning

confidence: 99%

The AMP‐activated protein kinase modulates hypothermia‐induced J wave

Chen

et al. 2020

Eur J Clin Investigation

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Background:The mechanism underlying the occurrence of the J wave in low temperature remains unclear. However, low temperature is associated with metabolic disorder and 5' AMP-activated protein kinase (AMPK), which modulates ionic currents and cardiac metabolism. This study investigated whether AMPK regulation can modulate the occurrence of the J wave at low temperature. Methods: Unipolar and bipolar leads were used to record monophasic action potential (the endocardium and epicardium) and pseudo-electrocardiograms (inferior leads) to study the cardiac electrical activity. Measurements were taken in isolated Langendorff rabbit hearts at both 30℃ and 37℃ before and after administration of 4-aminopyridine (an ultrarapid delayed rectifier potassium current inhibitor, I Kur , 50 µmol L −1 ), PF06409577 (an AMPK activator, 1 µmol L −1 ), compound C (an AMPK inhibitor, 10 µmol L −1 ) and glibenclamide (an ATP-sensitive inward rectifier potassium channel inhibitor, I KATP , 20 µmol L −1 ). Results: The amplitude of the J wave (2.46 ± 0.34 mV vs. 1.11 ± 0.23 mV, P < .01) at 30℃ (n = 15) was larger than that at 37℃ (n = 15). PF06409577 (1 µmol L −1 ) increased the J waves at both 30℃ and 37℃. In contrast, compound C (10 µmol L −1 ) reduced J wave at both 37℃ and 30℃. Low-temperature-induced J waves were individually suppressed by 4-AP (50 µmol L −1 ) and glibenclamide (20 µmol L −1 ). Conclusions: AMPK inhibition reduces low-temperature-induced J waves and possible ventricular arrhythmogenesis by modulating I KATP and I Kur channels.

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“…The electrocardiographic hallmark of type 1 Brugada syndrome, which may be spontaneous or drug-induced, is a coved-type ST elevation in V1–V3 with an elevation ≥2 mm [5]. The initial ECG of the index patient had discrete J-point elevation in V1–V3, the ST-segment slope was slightly elevated, and the repolarization was abnormal with pronounced biphasic T-waves.…”

Section: Discussionmentioning

confidence: 99%

A Novel <b><i>SCN5A</i></b> Variant Associated with Abnormal Repolarization, Atrial Fibrillation, and Reversible Cardiomyopathy

et al. 2018

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A variety of life-threating arrhythmias are caused by mutations in the cardiac voltage-gated sodium channel encoded by the SCN5A gene. In this study, we report a novel loss-of-function SCN5A variant, p.Ile1343Val (c.4027A>G), identified in a 42-year-old proband who presented with an unusual ECG with abnormal repolarization with biphasic T-waves in anteroseptal leads, persistent atrial fibrillation (AF), intermittent left bundle branch block (LBBB), and reversible cardiomyopathy. The patient did not meet the diagnostic criteria for Brugada syndrome, long QT syndrome, or any other known SCN5A-associated phenotype. Characterization of the biophysical properties of the variant by in vitro patch clamp experiments revealed a reduced Na⁺ current with no effect on the inactivation kinetics of the channel. This loss-of-function of Na⁺ current could explain the intermittent LBBB as well as the AF. In conclusion, we describe a unique combination of electrical and structural abnormalities associated with a novel SCN5A variant. Our findings broaden the spectrum of cardiac phenotypes associated with SCN5A channelopathy, underlining the complex clinical manifestations of genetic variations within this gene.

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J‐Wave syndromes expert consensus conference report: Emerging concepts and gaps in knowledge

Cited by 230 publications

References 354 publications

Syncope and the risk of sudden cardiac death: Evaluation, management, and prevention

Syncope and the risk of sudden cardiac death: Evaluation, management, and prevention

The AMP‐activated protein kinase modulates hypothermia‐induced J wave

A Novel <b><i>SCN5A</i></b> Variant Associated with Abnormal Repolarization, Atrial Fibrillation, and Reversible Cardiomyopathy

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