Brugada syndrome with atypical ECG: downsloping ST-segment elevation in inferior leads

Pérez‐Riera, Andrés Ricardo; Ferreira, Celso; Schapachnik, Edgardo; Sanches, Paulo Cesar R.; Moffa, Paulo Jorge

doi:10.1016/j.jelectrocard.2004.01.002

Cited by 70 publications

(32 citation statements)

References 19 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…By contrast, there are >20 case reports showing the ST-segment elevation in the inferior but not right precordial leads. [9][10][11][12][13][14] Although loss-offunction type SCN5A mutations could be identified in ~20% of patients with classical BS 7 , there is only a single report 15 on the genetic background of the atypical type BS with the ST-elevation in the inferior leads. Potet et al 15 identified an SCN5A missense mutation, G752R, in a family who had ST-segment elevation in the inferior leads.…”

Section: Discussionmentioning

confidence: 99%

“…8 In contrast, there is a variant type of the syndrome showing the ST-segment elevation in the inferior but not right precordial leads. [9][10][11][12][13][14] Although a single case with a SCN5A mutation, G752R, has been reported to display the ST-segment elevation in the inferior leads, 15 it still remains unclear as to whether this variant is a latent form of SCN5A-positive BS. We here report on a 67-year-old male with recurrent VF in whom we identified a novel nonsense SCN5A mutation, R179X.…”

mentioning

confidence: 99%

See 1 more Smart Citation

Dynamic Change in ST-Segment and Spontaneous Occurrence of Ventricular Fibrillation in Brugada Syndrome With a Novel Nonsense Mutation in the SCN5A Gene During Long-Term Follow-up

Kawamura

Ozawa

Yao

et al. 2009

Circ J

View full text Add to dashboard Cite

A 67-year-old male underwent genetic testing under the diagnosis of Brugada syndrome because of recurrent ventricular fibrillation with coincident ST-segment elevation in either right precordial, inferior leads or both since the age of 55 years. Screening of gene mutations using denaturing high-performance liquid chromatography (DHPLC) and direct sequencing identified a novel nonsense mutation (R179X) of SCN5A in a heterozygous manner. The functional assay for the identified mutation, using a whole-cell patch clamp in the heterologous expression system, revealed that the nonsense mutation, located in the second transmembrane segment of the first domain (DI-S2) of the α-subunit, failed to synthesize the complete structure of the cardiac sodium channel, thus causing the non-functional channel. Coding effects by the gene mutation was altered during the 12-year follow-up, which might affect the clinical features of the patient through the ion channel density in the ventricle, dynamics of repolarization abnormality and conduction disturbance. (Circ J 2009; 73: 584 -588)

show abstract

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

Dynamic Change in ST-Segment and Spontaneous Occurrence of Ventricular Fibrillation in Brugada Syndrome With a Novel Nonsense Mutation in the SCN5A Gene During Long-Term Follow-up

Kawamura

Ozawa

Yao

et al. 2009

Circ J

View full text Add to dashboard Cite

show abstract

“…Negative genetic screening (mutations are identifiable in only 20% to 30% of cases affected by true BrS) Recently, Alper et al 1 showed the first case of BrP with atypical type 1 Brugada ECG pattern located in inferior leads, emphasizing that Brugada-like syndromes have been reported to present as ST elevation in inferior leads. 3 The potential mechanisms and pathophysiology underlying BrP remain unclear. BrPs have been reported under a multitude of clinical circumstances in the following distinct etiologic categories 1,2 : metabolic conditions, endocrine disease (ie, hypopituitarism), electrolyte imbalances, mechanical compression (mediastinal tumors, pectus excavatum), ischemia-induced (the present case), myocardial and pericardial disease, acute pulmonary embolism, and others.…”

Section: Brugada Phenocopy Emerging As a New Conceptmentioning

confidence: 99%

Unusual ST-Segment Elevation in the Anterolateral Precordial Leads

et al. 2017

View full text Add to dashboard Cite

The patient is a 65-year-old white male with history of type 2 diabetes mellitus, hypertension, chronic smoking, and prior stroke with residual left hemiparesis and aphasia. His medication included losartan, furosemide, simvastatin, and metformin.He was admitted to the emergency room in cardiac arrest and was quickly resuscitated with cardiopulmonary resuscitation and external electric cardioversion maneuvers. Immediately after return of spontaneous circulation, a 12-lead ECG was performed (Figure 1).Based on the ECG, what is the most likely etiology of his cardiac arrest? Please turn the page to read the diagnosis.

show abstract

“…In both ERS and BrS, the manifestation of the J wave or ER is dynamic 14,29,30 , with the most prominent ECG changes appearing just before the onset of VT/VF [7][8][9][10][11][12][13][14][29][30][31] . Other ECG characteristics of ERS also closely match those of BrS, including the presence of accentuated J waves, pause and bradycardia-dependence, short coupled extrasystole-induced polymorphic VT/VF.…”

Section: Clinical Manifestations Of J Wave Syndromesmentioning

confidence: 99%

“…Our observation in 2000 that an ER pattern in the canine coronary-perfused wedge preparation can easily convert to one in which phase 2 reentry gives rise to polymorphic ventricular tachycardia/ ventricular fibrillation (VT/VF), prompted the suggestion that ER may in some cases predispose to malignant arrhythmias in the clinic 1,4,5 . A number of case reports and experimental studies have suggested a critical role for the J wave in the pathogenesis of idiopathic ventricular fibrillation (IVF) [6][7][8][9][10][11][12][13][14] . A definitive association between ER and IVF was presented in the form of two studies published in the New England Journal of Medicine in 2008 15,16 These were followed by another study from Viskin and co-workers 17 that same year and two large population association studies in 2009 and 2010.…”

mentioning

confidence: 99%

J Wave Syndromes: From Cell to Bedside

Antzelevitch¹

2011

J Arrhythmia

View full text Add to dashboard Cite

The J wave, a deflection that follows the QRS complex of the surface ECG, is usually partially buried in the R wave in humans, appearing as a J point elevation. An early repolarization (ER) pattern characterized by J point elevation, slurring of the terminal part of the QRS and ST segment elevation has long been recognized and considered to be totally benign. Recent studies have presented evidence demonstrating that an ER pattern in inferior leads or infero-lateral leads is associated with increased risk for life-threatening arrhythmias, named early repolarization syndrome (ERS). ERS and Brugada syndrome (BrS) share similar ECG characteristics, clinical outcomes, risk factors as well as a common arrhythmic platform related to amplification of I tomediated J waves. Although BrS and early repolarization syndrome (ERS) differ with respect to the magnitude and lead location of abnormal J wave manifestation, they can be considered to represent a continuous spectrum of phenotypic expression, termed J wave syndromes. ERS has been proposed to be divided into three subtypes: Type 1, displaying an ER pattern predominantly in the lateral precordial leads, is prevalent among healthy male athletes and rarely seen in VF survivors; Type 2, displaying an ER pattern predominantly in the inferior or infero-lateral leads, is associated with a higher level of risk; whereas Type 3, displaying an ER pattern globally in the inferior, lateral and right precordial leads, is associated with the highest level of risk for development of malignant arrhythmias and is often associated with VF storms. KeywordsCardiac arrhythmias; Sudden cardiac death; Sudden cardiac arrest; Transient outward current; J point elevation; Early repolarization syndrome; Brugada syndrome; Idiopathic Ventricular fibrillation; Hypothermia; STEMI The J wave is a deflection that follows the QRS complex on the surface electrocardiogram (ECG). When partially buried in the R wave, the J wave appears as a J point elevation and may be accompanied by an ST segment elevation, an ECG feature referred to as an early repolarization (ER) pattern. Recent studies have provided evidence in support of an © 2011 Elsevier Inc. All rights reserved. Publisher's Disclaimer: This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. Conflicts of Interest:There are no conflicts of interest to disclose. The early repolarization pattern, consisting of a distinct J wave or J point elevation, a notch or slur of the terminal part of the QRS and an ST segment elevation, is predominantly found in healthy young males and has traditionally been viewed as benign 2, 3 . Our observation i...

show abstract

Brugada syndrome with atypical ECG: downsloping ST-segment elevation in inferior leads

Cited by 70 publications

References 19 publications

Dynamic Change in ST-Segment and Spontaneous Occurrence of Ventricular Fibrillation in Brugada Syndrome With a Novel Nonsense Mutation in the SCN5A Gene During Long-Term Follow-up

Dynamic Change in ST-Segment and Spontaneous Occurrence of Ventricular Fibrillation in Brugada Syndrome With a Novel Nonsense Mutation in the SCN5A Gene During Long-Term Follow-up

Unusual ST-Segment Elevation in the Anterolateral Precordial Leads

J Wave Syndromes: From Cell to Bedside

Contact Info

Product

Resources

About