Positive Brugada Challenge Test in V1R–V3R as a Predictor of Malignant Prognosis in Brugada Patients

Hashemi, Arash; Shahrzad, Shahab; Shahrzad, Sorayya; Saber, Siamak; Taban, Samira; Aslani, Amir; Emkanjoo, Zahra

doi:10.1111/anec.12055

Cited by 3 publications

(3 citation statements)

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“…In the BrS family, eight patients were detected with p.Arg222Ter mutation; nevertheless, six of them had normal ECG in serial monitoring situations. The prevalence of the mutation in our limited study population emerged to be 50%, which is more than previous studies ( 12 - 14 ). The detected mutation in SSS patients was located in the cytoplasmic region of Nav1.5 with a loss of function effect, i.e., attenuation of cardiac Na current ( 15 ).…”

Section: Discussioncontrasting

confidence: 59%

Genetic analysis of cardiac SCN5A Gene in Iranian patients with hereditary cardiac arrhythmias

Asadi¹,

Foo²,

Bhuiyan³

et al. 2015

Anatol J Cardiol

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Objective:SCN5A encodes alpha subunit of the major sodium channel (Nav1.5) in human cardiac tissue. Malfunction of this cardiac sodium channel is associated with a variety of cardiac arrhythmias and myocardial inherited diseases.Methods:Fifty-three members from three families each diagnosed with long-QT syndrome type 3 (LQTS3), Brugada syndrome (BrS), or sick sinus syndrome (SSS) were included in this observational, cross-sectional study. In this study, we analyzed the sequences of coding region of the SCN5A gene.Results:Eleven members of the LQTS family (39%) showed p.Gln1507-Lys1508-Pro1509del mutation, 8 of BrS family (50%) showed p.Arg222Ter nonsense mutation, and 5 of 9 SSS family members (55%) showed a novel p.Met1498Arg mutation in the SCN5A gene.Conclusion:p.Gln1507-Lys1508-Pro1509del mutation, p.Arg222Ter nonsense mutation, and p.Met1498Arg in LQTS, BrS, and SSS, respectively, are reported for the first time in the Iranian population. Information regarding underlying genetic defects would be necessary for verifying certain clinically diagnosed arrhythmia types, carrier screening in affected families, and more precise therapy of the patients are required. (Anatol J Cardiol 2016; 16: 170-4)

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Section: Discussioncontrasting

confidence: 59%

Genetic analysis of cardiac SCN5A Gene in Iranian patients with hereditary cardiac arrhythmias

Asadi¹,

Foo²,

Bhuiyan³

et al. 2015

Anatol J Cardiol

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“…13 The prognostic accuracy of these medications is variable, with studies reporting ajmaline to be more sensitive than flecainide, 22 and IV procainamide more sensitive than oral flecainide. 23 A positive drug challenge where a type 1 ECG is provoked in standard or high lead ECGs is diagnostic for BrS. 13 Unfortunately without a gold standard for diagnosis, the sensitivity and specificity of drug testing is unknown.…”

Section: Drug Challengementioning

confidence: 99%

Brugada syndrome: updated perspectives

Walia¹,

Steinberg²,

Laksman³

2019

RRCC

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Since the first reported descriptions of Brugada syndrome, there has been a growing awareness and appreciation of the disease and its implications. From the diagnostic criteria, to risk stratification and management, there is an ongoing evolution, reclassification and re-thinking of Brugada syndrome as basic science, registry and clinical trial data shape our understanding of the pathophysiology and its clinical implications. This in-depth review sheds light on the most important literature to date, highlighting insights that shifted the global perspective on the disease. Current clinical paradigms and guidelines are presented, along with their justification, and possible opportunities for future research are explored.

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“…Use of right-sided precordial lead placement V1 R –V3 R has also been used to detect the pattern. [ 39 ] In cases where it may be hard to differentiate the Type 2 Brugada pattern from r’ in V1 in healthy athletes, the duration of the base of the triangle of r’ in V1/V2 taken at 5 mm from the peak of r’ >160 ms is specific and sensitive for BrS [ Figure 2 ]. [ 40 ] Computer algorithms for recognizing Type 1, Type 2/3, and suggestive Brugada patterns have been developed with reasonable accuracy.…”

Section: Electrocardiographic Findingsmentioning

confidence: 99%

Brugada syndrome in children - Stepping into unchartered territory

Behere

Weindling

2017

Ann Pediatr Card

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Brugada syndrome (BrS) is an autosomal dominant inherited channelopathy. It is associated with a typical pattern of ST-segment elevation in the precordial leads V1–V3 and potentially lethal ventricular arrhythmias in otherwise healthy patients. It is frequently seen in young Asian males, in whom it has previously been described as sudden unexplained nocturnal death syndrome. Although it typically presents in young adults, it is also known to present in children and infants, especially in the presence of fever. Our understanding of the genetic pathogenesis and management of BrS has grown substantially considering that it has only been 24 years since its first description as a unique clinical entity. However, there remains much to be learned, especially in the pediatric population. This review aims to discuss the epidemiology, genetics, and pathogenesis of BrS. We will also discuss established standards and new innovations in the diagnosis, prognostication, risk stratification, and management of BrS. Literature search was run on the National Center for Biotechnology Information's website, using the Medical Subject Headings (MeSH) database with the search term “Brugada Syndrome” (MeSH), and was run on the PubMed database using the age filter (birth–18 years), yielding 334 results. The abstracts of all these articles were studied, and the articles were categorized and organized. Articles of relevance were read in full. As and where applicable, relevant references and citations from the primary articles were further explored and read in full.

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Positive Brugada Challenge Test in V₁R–V₃R as a Predictor of Malignant Prognosis in Brugada Patients

Cited by 3 publications

References 20 publications

Genetic analysis of cardiac SCN5A Gene in Iranian patients with hereditary cardiac arrhythmias

Genetic analysis of cardiac SCN5A Gene in Iranian patients with hereditary cardiac arrhythmias

<p>Brugada syndrome: updated perspectives</p>

Brugada syndrome in children - Stepping into unchartered territory

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