scholarly journals 217 Why it is important to recognize Brugada syndrome in athletes: a case report

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Lidia Colangelo ◽  
Maria Colangelo ◽  
Luca Stefanini
Keyword(s):  
High Risk ◽  
Sudden Cardiac Death ◽  
Effective Treatment ◽  
Brugada Syndrome ◽  
Cardiac Death ◽  
Recovery Period ◽  
Young Athletes ◽  
Case Presentation ◽  
Timely Diagnosis ◽  
Increased Risk

Abstract Aims The Brugada syndrome (Brs) is an inherited disorder associated with risk of ventricular fibrillation and sudden cardiac death in a structurally normal heart. The purpose of this case presentation was to spread awareness about this condition, highlight the importance of timely diagnosis and effective treatment of this channelopathy especially in asymptomatic young athletes at high risk of sudden cardiac death. Methods and results In this report, we discuss the case of a 47-year-old male. He was a tennis player who performed a visit to the sports doctor to have issued a certificate for competitive fitness. He had no familiar history of sudden death or syncope. The patient’s electrocardiogram (ECG) revealed J-point elevation and ST-segment elevation in the right precordial leads V1 and V2 positioned in the second, third, or fourth intercostal space, showing classic type II ‘saddleback’ morphology in V2 and BrS was suspected. Hence, the patient underwent Holter ECG monitoring with evidence of spontaneous type 1 Brugada pattern (‘coved’ morphology), as well as frequent ventricular ectopic beats with left branch block morphology. Indeed, a diagnosis of BrS was made. Antiarrhythmic prophylaxis therapy with hydroquinidine was initiated and the patient was suspended from competitive activity with a 3-month follow-up. Conclusions The BrS is a hereditary disease characterized by a typical ECG pattern potentially predisposing active individuals with no patent structural heart disease to ventricular arrhythmias (VA) and sudden cardiac death (SCD). Nowadays, it is difficult to discern the true burden of BrS due to the unknown real prevalence of asymptomatic patients and the dynamic variability of the ECG pattern in individuals. The purpose of this case presentation was to spread awareness about this condition, highlight the importance of timely diagnosis, and effective treatment of this channelopathy especially in asymptomatic young athletes at high risk of SCD. Indeed, exercise may potentially worsen the ECG abnormalities in BrS patients, resulting in higher peak J-point amplitudes during the vasovagal reaction of the recovery period, possibly leading to an increased risk of cardiac events. Moreover, the enhanced vagal tone in athletes could be both a BrS risk factor and an exercise effect. For this reason, athletic pre-participation screening is essential for minimizing the risk for SCD in athletes participating in either competitive or leisure sporting activities.

scholarly journals Risk Stratification in Brugada Syndrome: Current Status and Emerging Approaches

2018 ◽  
Vol 7 (2) ◽  
pp. 79 ◽  
Author(s):  
Shohreh Honarbakhsh ◽  
Rui Providencia ◽  
Pier D Lambiase ◽  
◽  
◽  
...  
Keyword(s):  
High Risk ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Brugada Syndrome ◽  
Cardiac Death ◽  
Right Ventricular Outflow Tract ◽  
Current Status ◽  
Drug Induced ◽  
Increased Risk

Brugada syndrome (BrS) is one of the most common inherited channelopathies associated with an increased risk of sudden cardiac death. Appropriate use of an ICD in high-risk patients is life-saving. However, there remains a lack of consensus on risk stratification, and even on the diagnosis of BrS itself. Some argue that people with a type 1 Brugada ECG pattern but no symptoms should not be diagnosed with BrS, and guidelines recommend observation without therapy in these patients. Others argue that the presence of a spontaneous (rather than drug-induced) type 1 ECG pattern alone is enough to label them as high-risk for arrhythmic events, particularly if syncope is also present. Syncope and a spontaneous type 1 ECG pattern are the only factors that have consistently been shown to predict ventricular arrhythmic events and sudden cardiac death. Other markers have yielded conflicting data. However, in combination they may have roles in risk scoring models. Epicardial catheter ablation in the right ventricular outflow tract has shown promise in studies as an alternative management option to an ICD, but longer follow-up is required to ensure that the ablation effect is permanent.

scholarly journals Common variants at SCN5A-SCN10A and HEY2 are associated with Brugada syndrome, a rare disease with high risk of sudden cardiac death

2013 ◽  
Vol 45 (9) ◽  
pp. 1044-1049 ◽  
Author(s):  
Connie R Bezzina ◽  
Julien Barc ◽  
Yuka Mizusawa ◽  
Carol Ann Remme ◽  
Jean-Baptiste Gourraud ◽  
...  
Keyword(s):  
High Risk ◽  
Sudden Cardiac Death ◽  
Rare Disease ◽  
Brugada Syndrome ◽  
Cardiac Death ◽  
Common Variants

scholarly journals Erratum: Common variants at SCN5A-SCN10A and HEY2 are associated with Brugada syndrome, a rare disease with high risk of sudden cardiac death

2013 ◽  
Vol 45 (11) ◽  
pp. 1409-1409 ◽  
Author(s):  
Connie R Bezzina ◽  
Julien Barc ◽  
Yuka Mizusawa ◽  
Carol Ann Remme ◽  
Jean-Baptiste Gourraud ◽  
...  
Keyword(s):  
High Risk ◽  
Sudden Cardiac Death ◽  
Rare Disease ◽  
Brugada Syndrome ◽  
Cardiac Death ◽  
Common Variants

scholarly journals Sudden Cardiac Death in Chagas Disease

2016 ◽  
Vol 7 ◽  
Author(s):  
Dario Di Toro ◽  
Adrian Baranchuk
Keyword(s):  
High Risk ◽  
Sudden Cardiac Death ◽  
Effective Treatment ◽  
Chagas Disease ◽  
Cardiac Death ◽  
Implantable Cardioverter Defibrillators ◽  
Endemic Region ◽  
Arrhythmic Death ◽  
Risk Patients ◽  
Cardioverter Defibrillators

<p>Chagas’ disease remains a major epidemiologic problem in endemic countries. The most dramatic course of the disease is represented by sudden cardiac death. Some of these deaths could be preventable, if efforts are directed towards identifying high-risk patients. Implantable cardioverter-defibrillators are an effective treatment for the prevention of sudden cardiac arrhythmic death, however; its costs are the major limitation for their widespread use in the endemic region.</p><p>This review will cover some of the most relevant aspects of sudden cardiac death in Chagas’ disease. </p>

scholarly journals Does the Age of Sudden Cardiac Death in Family Members Matter in Brugada Syndrome?

2021 ◽  
Author(s):  
Pattara Rattanawong ◽  
Jakrin Kewcharoen ◽  
Chanavuth Kanitsoraphan ◽  
Timothy Barry ◽  
Anusha Shanbhag ◽  
...  
Keyword(s):  
Family History ◽  
Sudden Cardiac Death ◽  
Brugada Syndrome ◽  
Cardiac Death ◽  
Meta Analysis ◽  
Positive Family History ◽  
Family Members ◽  
Increased Risk ◽  
History Of ◽  
Risk Predictor

Background Brugada syndrome is an inherited cardiac channelopathy associated with major arrhythmic events (MAEs). The presence of a positive family history of sudden cardiac death (SCD) as a risk predictor of MAE remains controversial. We aimed to examine the association between family history of SCD and MAEs stratified by age of SCD with a systematic review and meta‐analysis. Methods and Results We searched the databases of MEDLINE and EMBASE from January 1992 to January 2020. Data from each study were combined using the random‐effects model. Fitted metaregression was performed to evaluate the association between the age of SCD in families and the risk of MAE. Twenty‐two studies from 2004 to 2019 were included in this meta‐analysis involving 3386 patients with Brugada syndrome. The overall family history of SCD was not associated with increased risk of MAE in Brugada syndrome (pooled odds ratio [OR], 1.11; 95% CI, 0.82–1.51; P =0.489, I 2 =45.0%). However, a history of SCD in family members of age younger than 40 years of age did increase the risk of MAE by ≈2‐fold (pooled OR, 2.03; 95% CI, 1.11–3.73; P =0.022, I 2 =0.0%). When stratified by the age of cut point at 50, 45, 40, and 35 years old, a history of SCD in younger family member was significantly associated with a higher risk of MAE (pooled OR, 0.49, 1.30, 1.51, and 2.97, respectively; P =0.046). Conclusions A history of SCD among family members of age younger than 40 years was associated with a higher risk of MAE.

scholarly journals Diagnosis of Brugada Syndrome, a Rare Inherited Arrhythmogenic Disorder

2020 ◽  
Vol 3 (2) ◽  
pp. 70-73
Author(s):  
Juwairiya Syed Iqbaluddin ◽  
Fathima Murthuza ◽  
Sumaiya Iqbal
Keyword(s):  
Family History ◽  
Sudden Cardiac Death ◽  
Brugada Syndrome ◽  
Cardiac Death ◽  
Genetic Disorder ◽  
Positive Family History ◽  
Provocation Test ◽  
Increased Risk ◽  
History Of ◽  
Electrocardiogram Ecg

Brugada syndrome (BrS) is a rare, autosomal dominant genetic disorder with mutation in the SCN5A gene. It is associated with an increased risk of arrhythmias and sudden cardiac death. BrS can be diagnosed by characteristic electrocardiogram (ECG) findings and significant events, such as syncope, palpitations, nocturnal respiratory agonia, and family history of sudden cardiac death below the age of 45 years. Special investigations, such as electrophysiology study, ajmaline provocation test, and genetic testing, play an important role in its diagnosis. This case report describes a patient who presented with chest pain and dizziness along with a positive family history of sudden cardiac deaths below the age of 45 years. He was discovered to have type 2 Brugada pattern on ECG, and by ajmaline provocation test, the type 1 pattern was unmasked, which established a definitive diagnosis of BrS. The patient was then advised for an implantable cardioverter-defibrillator. This case highlights the need for physicians to be competent in identifying patients with BrS in order to provide the necessary management and prevent fatal outcomes.

scholarly journals Brugada syndrome and arrhythmogenic cardiomyopathy: overlapping disorders of the connexome?

EP Europace ◽  
2020 ◽  
Author(s):  
Yael Ben-Haim ◽  
Angeliki Asimaki ◽  
Elijah R Behr
Keyword(s):  
Sudden Cardiac Death ◽  
Brugada Syndrome ◽  
Cardiac Death ◽  
Cell Junctions ◽  
Cardiac Cell ◽  
Arrhythmogenic Cardiomyopathy ◽  
Inherited Diseases ◽  
Disease Spectrum ◽  
Increased Risk ◽  
Cell Cell

Abstract Arrhythmogenic cardiomyopathy (ACM) and Brugada syndrome (BrS) are inherited diseases characterized by an increased risk for arrhythmias and sudden cardiac death. Possible overlap between the two was suggested soon after the description of BrS. Since then, various studies focusing on different aspects have been published pointing to similar findings in the two diseases. More recent findings on the structure of the cardiac cell–cell junctions may unite the pathophysiology of both diseases and give further evidence to the theory that they may in part be variants of the same disease spectrum. In this review, we aim to summarize the studies indicating the pathophysiological, genetic, structural, and electrophysiological overlap between ACM and BrS.

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