Prolonged right ventricular ejection delay identifies high risk patients and gender differences in Brugada syndrome

Malderen, Sophie C H Van; Kerkhove, Dirk; Theuns, Dominic; Weytjens, Caroline; Droogmans, Steven; Tanaka, Kaoru; Daneels, Dorien; Dooren, Sonia Van; Meuwissen, Marije; Bonduelle, M.; Brugada, Pedro; Camp, Guy Van

doi:10.1016/j.ijcard.2015.04.243

Cited by 15 publications

(14 citation statements)

References 47 publications

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“…In addition, testosterone may increase outward repolarizing currents, leading to loss of the AP dome ( 48 ). In line with this hypothesis, the delayed right ventricular ejection, more frequently observed in males than in females, could contribute to an increase risk of malignant events in BrS ( 49 ). In concordance to this fact, in 2019 a case report of a female living as a transgender male was reported, in which testosterone supplementation unmasked the BrS ECG pattern ( 50 ).…”

Section: Cellular Basismentioning

confidence: 65%

“…Pathogenic alterations in this gene leads to loss of function in the α subunit of the cardiac sodium channel protein (Nav1.5). To date, more than 150 deleterious alterations in SCN5A have been associated with BrS and underlie nearly 30% of all BrS cases ( 49 , 51 ). Although several genetic alterations located in more than 20 genes have been reported as potentially cause of BrS ( 52 ) recent evidence-based reappraisal of gene-disease validity disputed the causality of main part of these genes, leaving SCN5A as the only gene with definite causality in BrS ( 53 ).…”

Section: Geneticsmentioning

confidence: 99%

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Brugada Syndrome in Women: What Do We Know After 30 Years?

Martínez-Barrios

Arbelo

César

et al. 2022

Front. Cardiovasc. Med.

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Brugada syndrome (BrS) was initially described in 1992 by Josep and Pedro Brugada as an arrhythmogenic disease characterized by ST segment elevation in the right precordial leads and increased risk of sudden cardiac death (SCD). Alterations in the SCN5A gene are responsible for approximately 30% of cases of BrS, following an autosomal dominant pattern of inheritance. However, despite its autosomal transmission, sex-related differences are widely accepted. BrS is more prevalent in males than in females (8–10 times), with males having a 5.5-fold higher risk of SCD. There are also differences in clinical presentation, with females being more frequently asymptomatic and older than males at the time of diagnosis. Some factors have been identified that could explain these differences, among which testosterone seems to play an important role. However, only 30% of the available publications on the syndrome include sex-related information. Therefore, current findings on BrS are based on studies conducted mainly in male population, despite the wide acceptance of gender differences. The inclusion of complete clinical and demographic information in future publications would allow a better understanding of the phenotypic variability of BrS in different age and sex groups helping to improve the diagnosis, management and risk management of SCD.

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Section: Cellular Basismentioning

confidence: 65%

Section: Geneticsmentioning

confidence: 99%

Brugada Syndrome in Women: What Do We Know After 30 Years?

Martínez-Barrios

Arbelo

César

et al. 2022

Front. Cardiovasc. Med.

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“…14 Surprisingly, the mean RVED of BrS patients with any SCN5A variant in that cohort was not significantly prolonged compared with non-carriers. 14 We hypothesized that this lack of difference between SCN5A variant carriers and non-carriers could be attributed to the fact that different subtypes of SCN5A variants resulting in different degrees of INa reduction 12,15,16 may have different effects on conduction delay and RVED measurements. To date, this detailed .…”

Section: Methodsmentioning

confidence: 99%

Prolonged Right Ventricular Ejection Delay in Brugada Syndrome Depends on the Type of <i>SCN5A</i> Variant　― Electromechanical Coupling Through Tissue Velocity Imaging as a Bridge Between Genotyping and Phenotyping ―

Malderen

Daneels

Kerkhove

et al. 2018

Circ J

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“…Initially considered as a purely electrical disease, the use of cardiac imaging in Brugada syndrome only gained interest recently. Mild morphological and functional abnormalities affecting the right ventricular outflow tract [3], [4], [5], [6], [7], [8] - and to a lesser extent the right ventricle (RV) and the left ventricle (LV) [9], [10], [11] – were observed. Echocardiographic speckle tracking can reveal subtle alterations in myocardial contraction patterns [12].…”

Section: Datamentioning

confidence: 99%

Speckle tracking echocardiography data in Brugada syndrome patients

et al. 2019

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Brugada syndrome is characterized by typical electrocardiogram changes and a high risk for sudden cardiac death (Priori et al., 2013). In addition to the well known electrical substrate, morphological and functional alterations appeared to be present in a subset of the Brugada syndrome patients (Catalano et al., 2009). Echocardiographic speckle tracking enables us to detect subtle contraction alterations (Smiseth et al.,2016). We performed transthoracic echocardiography with speckle tracking analysis in 82 healthy controls and 175 Brugada syndrome patients. Main findings are presented and discussed in the article “Contraction alterations in Brugada syndrome; association with life-threatening ventricular arrhythmias” (Scheirlynck et al., 2019). This related Data article contains segmental longitudinal strain values for RV and LV, and the comparison of echocardiographic parameters between Brugada syndrome patients with spontaneous and drug-induced type 1 pattern and between patients with and without ventricular arrhythmia inducibility during electrophysiological study.

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Prolonged right ventricular ejection delay identifies high risk patients and gender differences in Brugada syndrome

Cited by 15 publications

References 47 publications

Brugada Syndrome in Women: What Do We Know After 30 Years?

Brugada Syndrome in Women: What Do We Know After 30 Years?

Prolonged Right Ventricular Ejection Delay in Brugada Syndrome Depends on the Type of <i>SCN5A</i> Variant　― Electromechanical Coupling Through Tissue Velocity Imaging as a Bridge Between Genotyping and Phenotyping ―

Speckle tracking echocardiography data in Brugada syndrome patients

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Prolonged right ventricular ejection delay identifies high risk patients and gender differences in Brugada syndrome

Cited by 15 publications

References 47 publications

Brugada Syndrome in Women: What Do We Know After 30 Years?

Brugada Syndrome in Women: What Do We Know After 30 Years?

Prolonged Right Ventricular Ejection Delay in Brugada Syndrome Depends on the Type of <i>SCN5A</i> Variant ― Electromechanical Coupling Through Tissue Velocity Imaging as a Bridge Between Genotyping and Phenotyping ―

Speckle tracking echocardiography data in Brugada syndrome patients

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Resources

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Prolonged Right Ventricular Ejection Delay in Brugada Syndrome Depends on the Type of <i>SCN5A</i> Variant　― Electromechanical Coupling Through Tissue Velocity Imaging as a Bridge Between Genotyping and Phenotyping ―