Electroanatomic and Pathologic Right Ventricular Outflow Tract Abnormalities in Patients With Brugada Syndrome

Pieroni, Maurizio; Notarstefano, Pasquale; Oliva, Antonio; Campuzano, Òscar; Santangeli, Pasquale; Coll, Mónica; Nesti, Martina; Carnevali, Andrea; Fraticelli, Aureliano; Iglesias, Anna; Grassi, Simone; Brugada, Ramón; Bolognese, Leonardo

doi:10.1016/j.jacc.2018.09.037

Cited by 78 publications

(63 citation statements)

References 28 publications

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“…[ 17 ] However, evidence demonstrating normalization of the pathognomonic ECG pattern and apparent significant reduction of the tendency to arrhythmia in most patients following radiofrequency ablation (RFA) of the RVOT epicardium strengthens the theory that structural abnormalities have a critical role in arrhythmogenesis in BrS. [ 23 ]…”

Section: Pathophysiology – Channelopathy or Cardiomyopathy?mentioning

confidence: 99%

Brugada syndrome: Clinical features, risk stratification, and management

et al. 2020

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In 1992, the Brugada brothers published a patient series of aborted sudden death, who were successfully resuscitated from ventricular fibrillation (VF). These patients had a characteristic coved ST-segment elevation in the right precordial leads on their 12-lead electrocardiogram with no apparent structural heart abnormality. This disease was referred to as “right bundle branch block, persistent ST-segment elevation, and sudden death syndrome.” The term Brugada syndrome (BrS) was first coined for this new arrhythmogenic entity in 1996. BrS is more prevalent in Southeast Asian ethnic groups and was considered a familial disease due to the presence of syncope and/or sudden deaths in several members of the same family, however, the genetic alteration was only noted in 1998. The genetic characterization of BrS has proven to be challenging. The most common and well-established BrS genotype involves loss-of-function mutations in the SCN5A gene, but only represents between 15% and 30% of the diagnosed patients. Patients with BrS can present with a range of symptoms which can include syncope, seizures, and nocturnal agonal breathing due to polymorphic ventricular tachycardia or VF. If these arrhythmias are sustained, sudden cardiac death may result. Despite the significant progress on the understanding of BrS over the last two decades, there remain a number of uncertainties and challenges; we present an update review on the subject.

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Section: Pathophysiology – Channelopathy or Cardiomyopathy?mentioning

confidence: 99%

Brugada syndrome: Clinical features, risk stratification, and management

et al. 2020

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“…2 However, evidence demonstrating normalisation of the pathognomonic ECG pattern and elimination of the arrhythmic disposition in most patients following radiofrequency ablation of the RVOT epicardium supports the theory that structural abnormalities play an important role in the pathophysiology of Brugada syndrome. 68 Indeed, recent results suggest that microanatomical changes such as increased collagen, fibrosis and reduced gap junction expression, which may be mediated by underlying pan-myocardial inflammation, are responsible for the characteristic electrographic pattern and arrhythmic susceptibility. 68,69 As a result of these findings, Brugada syndrome and arrhythmogenic cardiomyopathy have been proposed to be part of the same disease spectrum.…”

Section: Is Brugada Syndrome a Channelopathy Or Cardiomyopathy?mentioning

confidence: 99%

“…68 Indeed, recent results suggest that microanatomical changes such as increased collagen, fibrosis and reduced gap junction expression, which may be mediated by underlying pan-myocardial inflammation, are responsible for the characteristic electrographic pattern and arrhythmic susceptibility. 68,69 As a result of these findings, Brugada syndrome and arrhythmogenic cardiomyopathy have been proposed to be part of the same disease spectrum. 70 Although these two conditions have distinct macropathological appearance, known genetic predisposition and clinical features, several overlapping manifestations can be seen.…”

Section: Is Brugada Syndrome a Channelopathy Or Cardiomyopathy?mentioning

confidence: 99%

Current Controversies and Challenges in Brugada Syndrome

Snir

Raju

2019

Eur Cardiol

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“…[19] A recent study has further shown that RVOT electroanatomical alterations (a low-voltage area) correlate with myocardial inflammation and arrhythmia vulnerability, supporting the hypothesis that BrS is a combination of electrical and structural disease. [20]…”

Section: Models For Pathophysiologymentioning

confidence: 99%

Brugada Syndrome: Progress in Genetics, Risk Stratification and Management

Romero

Avendaño

et al. 2019

Arrhythm Electrophysiol Rev

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Brugada syndrome (BrS) is one of the most common causes of sudden cardiac death in normal structural heart individuals. First characterised in 1992, the global prevalence of BrS is unclear, with estimates placing it at around 0.05% and presenting most frequently in southeast Asian countries. This review aims to summarise the development in the understanding of BrS and, importantly, progress in its management, underpinned by knowledge regarding its genetics and molecular mechanisms. It also provides update on risk stratification and promising new therapies for BrS, including epicardial ablation. Future studies are required to increase understanding of the pathogenesis of this disease and to guide clinical practice.

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Electroanatomic and Pathologic Right Ventricular Outflow Tract Abnormalities in Patients With Brugada Syndrome

Cited by 78 publications

References 28 publications

Brugada syndrome: Clinical features, risk stratification, and management

Brugada syndrome: Clinical features, risk stratification, and management

Current Controversies and Challenges in Brugada Syndrome

Brugada Syndrome: Progress in Genetics, Risk Stratification and Management

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