Materiały źródłowe

• #1

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5968343/

  Brugada syndrome (BrS) is considered to be an inherited arrhythmic disease associated with fatal complications and premature sudden unexpected death. The worldwide pooled prevalence of BrS was 0.5 per 1,000 [95% confidence interval (CI): 0.3-0.7]. The highest prevalence was reported in Southeast Asia (3.7 per 1,000, 95% CI: 0.7-6.7); the lowest was found in North Africa (0 per 1,000). BrS in Asians was nine times more common than in Caucasians and 36 times more common than in Hispanics. The worldwide pooled prevalence of Type-2/3 BrP was 6.1 per 1,000 (95% CI: 5.0-7.1). The highest prevalence was also reported in Southeast Asia (35.5 per 1,000, 95% CI: 17.1-53.9). […] This study revealed a significant difference in the worldwide prevalence of BrS and Type-2/3 BrP. Brugada electrocardiogram patterns are highly prevalent in Southeast Asia.

• #2 Orphanet: Brugada syndrome

  https://www.orpha.net/en/disease/detail/130

  Given that the ECG pattern diagnostic for Brugada Syndrome is fluctuant, unlike other inherited arrhythmogenic syndromes, the data regarding the prevalence of the disease are controversial. According to a recent metanalysis, the worldwide prevalence of Brugada syndrome is estimated at 1/2,000, but it varies according to region and ethnicity. Brugada syndrome is rare in Hispanic and Caucasian populations and non-rare in Asian populations. By region, the prevalence is estimated at 1/20,000 in North America and 1/10,000 in Europe. Prevalence is higher in Asia and Middle East where estimates range between 1/270-625. The disease is observed more frequently in men than in women (8:1) and it is extremely rare in children. […] The majority of patients remain asymptomatic, 20-30% experience syncope and 8-12% experience at least one cardiac arrest (potentially leading to sudden death). Risk factors for cardiac arrest and sudden death are a spontaneously diagnostic ECG pattern and a history of syncope.

• #3 Brugada Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK519568/

  Brugada syndrome is a rare but potentially life-threatening inherited disease that predisposes patients to fatal cardiac arrhythmias. […] The prevalence of Brugada Syndrome is approximately 3 to 5 per 10,000 people. Brugada syndrome is approximately 8 to 10 times more common in males than females. […] Brugada syndrome is also more prevalent in those who are of Southeast Asian descent. The mean affected age is 41 years old. Brugada syndrome accounts for 4% of all sudden cardiac deaths.

• #4 9. Brugada syndrome – ReCent Medical News: Special Edition – Adult-onset monogenic diseases

  https://life-and-health.hannover-re.com/recent-medical-news-special-edition-adult-onset-monogenic-diseases/9-brugada-syndrome

  Brugada syndrome is an inherited cardiac disorder associated with an increased risk of development of abnormal heart rhythms, such as ventricular fibrillation or ventricular tachycardia, and consequently, may result in syncope, cardiac arrest, and sudden cardiac death. […] The prevalence of Brugada syndrome varies from 1:5000 to 1:2000, depending on geographic location. […] In Southeast Asian countries, the disease is considered endemic. […] While Brugada syndrome can be diagnosed at early or old age, the syndrome typically manifests in adulthood, with the mean age of presentation around 41. At this age, Brugada syndrome is 8-10 times more prevalent in males than females. […] The diagnosis is equally distributed in genders before puberty. […] The incidence of Brugada syndrome in pediatric patients is low, but patients have an unfavorable prognosis demonstrating high recurrence of arrhythmic events.

• #5 Brugada Syndrome | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions/b/brugada-syndrome-1.html

  Brugada syndrome is rare. It affects about 5 of every 10,000 people worldwide. […] First-degree relatives of someone with Brugada syndrome should be screened. This may include at least a health history, physical exam, and an ECG. Genetic screening may also be used.

• #6 Brugada Syndrome – Melbourne Heart Rhythm

  https://staging.melbourneheartrhythm.com.au/learn/conditions/52-brugada-syndrome

  Brugada Syndrome is reported to be responsible for 4% of all sudden deaths and 20% of sudden deaths in those without structural heart disease. […] The ECG changes of Brugada Syndrome are dynamic and can vary spontaneously making it difficult to assess its exact incidence in the general population but its prevalence is reported to be about 1 in 2000. […] The diagnosis of Brugada Syndrome may also be made on family screening of patients with Brugada Syndrome or from a routine ECG. […] In cases of sudden cardiac deaths Brugada Syndrome may be under-diagnosed as ECG prior to death is generally not available and even if an ECG has been recorded ECG changes, being variable, may not be diagnostic. […] The diagnosis of Brugada Syndrome can only be considered when a diagnostic Type-1 Brugada pattern ECG occurs spontaneously or following drug provocation. The diagnosis is further confirmed when the ECG pattern occurs in conjunction with one of the following: Documented Ventricular Fibrillation (VF), polymorphic VT, a family history of sudden cardiac death at <45 years, a diagnostic Type-1 Brugada pattern ECG in family members, inducibility of VT during an electrical study, syncope or nocturnal agonal respiration (attributed to self-terminating polymorphic VT or VF).

• #7 Brugada Syndrome: Progress in Diagnosis and Management | AER Journal

  https://www.aerjournal.com/articles/brugada-syndrome-progress-diagnosis-and-management?language_content_entity=en

  Brugada syndrome (BrS) represents an inherited disorder associated with risk of sudden cardiac death due to VF in patients without structural heart disease. […] At present, it is challenging to establish the actual burden of the syndrome, mainly because we do not know the real number of asymptomatic people due to the high variability and fluctuations of the typical ECG pattern. The incidence appears to be low (1%), but the condition is responsible for 10% of all sudden deaths and up to 20% of sudden deaths in structurally normal hearts. The prevalence is 810 times higher in men than women. […] Asymptomatic people are the majority (about 63%) of newly diagnosed Brugada patients. Although the reported annual rate of asymptomatic BrS events has decreased over time, this is not negligible (0.5%1.2% annual incidence), leading to a malignant arrhythmic events rate of 12% at 10-year follow-up in a population with a mean age of 40 years.

• #8 Brugada Syndrome within Asian Populations: State-of-the-Art Review

  https://www.mdpi.com/2035-8148/13/2/7

  Brugada syndrome (BrS) is an inherited cardiac channelopathy with variable expressivity that can lead to sudden cardiac arrest (SCA). Studies worldwide suggest that BrS and Brugada pattern (BrP) have low prevalences in general. However, studies also note that BrS is most prevalent among certain Asian populations. Among the different global regions, the highest prevalence is believed to be in Southeast Asia, followed by the Middle East, South Asia, East Asia, Europe, and North America. […] The importance of identifying such differences lies in the necessity to develop improved risk assessment strategies to guide secondary prevention and treatment for these patients. Specifically, the decision to pursue placement of an implantable cardiac defibrillator (ICD) can be lifesaving for high-risk BrS patients. However, there remains a significant lack of consensus on how to best risk stratify BrS patients.

• #9

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5968343/

  The worldwide prevalence of BrS was estimated at 0.5 per 1,000. BrS was highly prevalent in Southeast Asia as was Type-2/3 BrP. Thailand had the highest prevalence of BrS in the world with a prevalence of 6.8 per 1,000, which is approximately 14 times higher than the worldwide prevalence. […] Our results revealed that gender, ethnicity, and geographic region affected the prevalence of BrS. The heterogeneity of the results may be secondary to gene distribution variance in different regions. We estimated that the prevalence of BrS in Thailand was over 146 times more common than in North America and 37 times more common than in Europe.

• #10 Brugada Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/163751-overview

  Brugada syndrome is genetically determined and has an autosomal dominant pattern of transmission in about 50% of familial cases. The typical patient with Brugada syndrome is young, male, and otherwise healthy, with normal general medical and cardiovascular physical examinations. […] Because of its recent identification, the prevalence of Brugada syndrome is not well established. In a large university hospital on the West Coast of the United States, the prevalence of a Brugada ECG pattern among unselected, mainly white and Hispanic adults was 2 of 1348 patients (0.14%); in both cases, the ECG patterns were type 2. The prevalence in Asian and other ethnic populations may be higher. […] The highest prevalence of Brugada syndrome is in Southeast Asia; the lowest is in North Africa. In parts of Asia (eg, the Philippines, Thailand, Japan), Brugada syndrome seems to be the most common cause of natural death in men younger than 50 years. It is known as Lai Tai (Thailand), Bangungot (Philippines), and Pokkuri (Japan). In Northeast Thailand, the mortality rate from Lai Tai is approximately 30 cases per 100,000 population per year.

• #11 Brugada Syndrome – Revista Española de Cardiología (English Edition)

  https://www.revespcardiol.org/en-brugada-syndrome-articulo-13145482

  First described in 1992, Brugada syndrome is characterized by a specific electrocardiographic pattern in the right precordial leads and susceptibility to ventricular arrhythmias and sudden death. […] The prevalence of Brugada syndrome has been estimated at 5/10 000, although this figure possibly underestimates the actual prevalence, since many patients can present silent forms of the disease. Great geographical variability has been reported, such that the syndrome seems to be much more frequent in Asia than in western Europe or North America. […] In fact, the syndrome is thought to be endemic to certain regions of southeast Asia, where it is usually known as sudden unexplained death syndrome, also called bangungot (in Philippines), pokkuri (in Japan), or lai tai (in Thailand).

• #12 Brugada Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/163751-overview

  Brugada syndrome is most common in people from Asia. The reason for this observation is not yet fully understood but may be due to an Asian-specific sequence in the promoter region of SCN5A. Brugada syndrome is 8-10 times more prevalent in men than in women, although the probability of having a mutated gene does not differ by sex. The penetrance of the mutation therefore appears to be much higher in men than in women. Brugada syndrome most commonly affects otherwise healthy men aged 30-50 years, but affected patients aged 0-84 years have been reported. The mean age of patients who die suddenly is 41 years.

• #13 Current Controversies and Challenges in Brugada Syndrome | ECR Journal

  https://www.ecrjournal.com/articles/current-controversies-and-challenges-brugada-syndrome?language_content_entity=en

  Despite previous reports describing a strong negative predictive value of EPS in patients without cardiac arrest, a recent systematic review concluded that a negative EPS does not reliably indicate low risk in asymptomatic patients in the presence of other high-risk features. […] Brugada syndrome is about 7 to 10 times more prevalent in men, and male gender is associated with a higher incidence of ventricular arrhythmias and SCD at diagnosis and follow-up. […] Risk stratification in women with Brugada syndrome has been more challenging as most studies have mainly consisted of male participants. […] The mainstay of treatment in high-risk patients remains ICD implantation. […] Important risk-reducing strategies for all patients include avoiding excessive alcohol intake, immediate treatment of fever with antipyretics as well as avoidance of potentially aggravating medications.

• #14 Brugada syndrome – WikiAnesthesia

  https://wikianesthesia.org/wiki/Brugada_syndrome

  The overall prevalence of the Brugada ECG pattern ranges between 0.1 and 1 percent. There is a higher prevalence in the Philippines, Thailand and Japan. The mean age of sudden cardiac death is 41. The youngest patient was diagnosed 2 days of age, the oldest at 84 years; 20% of Brugada syndrome patients may develop supraventricular arrythmias (Antzelevitch). Brugada syndrome has an incidence of 1/5000 to 1/2000, Brugada pattern on ECG 0.12 0.8% (Ranucci).

• #15 Insights for Stratification of Risk in Brugada Syndrome | ECR Journal

  https://www.ecrjournal.com/articles/insights-stratification-risk-brugada-syndrome?language_content_entity=en

  Brugada syndrome (BrS) is an inherited disease with an increased risk of sudden cardiac death (SCD). However, testing identifies genetic disorders in only 20-30% of patients analysed, indicating a gap in knowledge of its genetic aetiology. […] Risk stratification in BrS patients is challenging, primarily because of the complexity of the issue. As a result, clinicians fail to provide the appropriate strategy for SCD prevention for many patients. […] Several variables and interventions are being studied to improve diagnostics and maximise patient protection. In addition, the scientific community must increase efforts to provide patient care according to knowledge and research for improving stratification of risk. […] The incidence of SCD peaks in young men, with the highest risk in those under 40 years old. Women and elderly patients are usually considered to be at lower risk. Overall, women have a lower incidence of BrS and lower risk of SCD than men.

• #16 Brugada Syndrome: Defining the Risk in Asymptomatic Patients | AER Journal

  https://www.aerjournal.com/articles/brugada-syndrome-defining-risk-asymptomatic-patients?language_content_entity=en

  Since the first description of the Brugada syndrome (BS) in 1992, scientific progress in the understanding of this disease has been enormous; at the same time more and more individuals with the disease have been diagnosed. […] The profile of patients with BS has changed with more asymptomatic individuals and less expressive clinical features. Asymptomatic BS individuals are at lower arrhythmic risk than those presenting with syncope or sudden cardiac death (SCD). […] The event incidence rate is around 0.5 % per year; this figure is relevant due to the fact that individuals have a long life expectancy and are otherwise healthy. […] As a result of the risk of SCD, risk stratification is of utmost importance. […] In our experience, 67 % of patients with BS are asymptomatic. Similar figures are found in other registries: 64 % in the France, Italy, Netherlands, Germany (FINGER) registry and 79 % in the Programmed Electrical Stimulation Predictive Value (PRELUDE) registry.

• #17 Frontiers | The Brugada Syndrome: A Rare Arrhythmia Disorder with Complex Inheritance

  https://www.frontiersin.org/journals/cardiovascular-medicine/articles/10.3389/fcvm.2016.00009/full

  The Brugada syndrome (BrS) is a rare inherited arrhythmia disease associated with high risk of sudden cardiac death in the young adult. […] Epidemiological studies have produced heterogeneous results regarding BrS incidence across the World. While estimated at 5 for 10,000 in western Europe and the USA, the prevalence of BrS seems higher in Southeast Asia, reaching 20 for 10,000. […] A third of the affected patients are identified after syncope, frequently preceded by vagal symptoms. […] The majority of patients are asymptomatic at time of diagnosis. More than one-third of cases are identified during familial screening. […] Only few clinical parameters allow risk stratification in BrS. […] There is still need for medical therapies that could reduce arrhythmia occurrence and prevent SCD.

• #18 Territory-wide cohort study of Brugada syndrome in Hong Kong: predictors of long-term outcomes using random survival forests and non-negative matrix factorisation | Open Heart

  https://openheart.bmj.com/content/8/1/e001505

  BrS has a high prevalence in Asia with a five-fold difference compared with western populations. A recently published systematic review and meta-analysis found that the prevalence is the highest in Southeast Asia, followed by North Africa, Middle East, East Asia, South Asia, North America, Europe and Hawaii. However, epidemiological and particularly outcomes-based data from Asia on BrS are lacking. […] The event rates of spontaneous VT/VF differ depending on initial symptoms, with prior VT/VF/SCD, syncope and asymptomatic subgroups. Results from the multi-centre FINGER registry reported annual event rates of 7.7%, 1.9% and 0.5%, respectively. […] The present study also found that P wave duration SD and the presence of other arrhythmias such as AF, mean QRS duration and QTc intervals were predictors of spontaneous VT/VF. Our findings, therefore implicate an additional role for atrial arrhythmias and abnormalities in ventricular repolarisation as important determinants of ventricular arrhythmogenesis in BrS and ECG biomarkers reflecting such processes provide incremental value for risk stratification. […] Machine learning techniques significantly improves overall risk stratification performance.

• #19 Brugada Syndrome: Defining the Risk in Asymptomatic Patients | AER Journal

  https://www.aerjournal.com/articles/brugada-syndrome-defining-risk-asymptomatic-patients?language_content_entity=en

  Quantification of arrhythmic events is crucial to offer solid management recommendations. Initial reports showed an event rate of 2.7 % per year in asymptomatic patients. […] Recent registries show an annual incidence of 0.5 % during a mean follow-up of 3273 months. […] This might seem relatively low, but when considering the long life expectancy of these patients and the lack of other conditions, this figure becomes very relevant. […] It should also be noted that the risk of SCD in the general population without BS and at 40 years of age is, at the most, 1:10.000 per year, which is about 100 times less than that for asymptomatic BS patients. […] More than 20 years after the first description of BS, risk stratification remains challenging and controversial. […] Current practical guidelines and consensus recommend implantation of an ICD in patients surviving a SCD (class I) and those with syncope and spontaneous type 1 electrocardiogram (ECG) class IIa.

• #20 Genetics of Brugada syndrome | Journal of Human Genetics

  https://www.nature.com/articles/jhg201597

  The Brugada syndrome is responsible for 4 to 12% of sudden cardiac death in the general population. The frequency is higher in Southeast Asia including Japan compared with Western countries. Brugada syndrome is more common in Southeast Asia including Japan, with the estimated prevalence of 12/10000 individuals, compared with western countries, with the estimated prevalence of 1 to 5/10000 individuals. […] A family history of unexplained sudden death is present in 20 to 30% of patients with Brugada syndrome. […] The annual incidence of arrhythmia recurrences in survivors of ventricular fibrillation is 7 to 8%. […] A genome-wide association study is used to examine the association of many common genetic variants selected from the whole genome with a trait. […] However, a recent genome-wide association study for Brugada syndrome has succeeded to identify two common genetic variants in SCN5A-SCN10A and HEY2 associated with the rare disease.

• #21 Current Controversies and Challenges in Brugada Syndrome | ECR Journal

  https://www.ecrjournal.com/articles/current-controversies-and-challenges-brugada-syndrome?language_content_entity=en

  A spontaneous type 1 Brugada ECG pattern is an important established risk factor for cardiac events, which has been reported to confer a three- to fourfold higher risk in asymptomatic patients. […] Despite this, only conservative surveillance and risk modification of lifestyle is universally recommended in asymptomatic patients. […] While a multitude of ECG parameters have been associated with increased risk of cardiac arrhythmias, two that have been consistently reported as independent risk factors are abnormal QRS fragmentation and early repolarisation pattern in inferior and/or lateral leads. […] There is ongoing debate regarding the prognostic value of conducting electrophysiological study (EPS) with programmed electrical stimulation for risk stratification in patients without a history of cardiac arrest.

• #21 Current Controversies and Challenges in Brugada Syndrome | ECR Journal

  https://www.ecrjournal.com/articles/current-controversies-and-challenges-brugada-syndrome?language_content_entity=en

  Brugada syndrome remains engulfed in controversy. This review aims to shed light on the main challenges surrounding the diagnostic pathway and criteria, risk stratification of asymptomatic patients, pharmacological and interventional risk modification strategies as well as our current pathophysiological understanding of the disease. […] Recent data support the premise that Brugada syndrome is the most common single underlying aetiology for sudden unexplained death with negative autopsy, representing 28% of cases in the UK. […] Yet more than 30 years since its recognition, there are many questions and uncertainties surrounding the pathophysiology, diagnosis, risk assessment and management of Brugada syndrome. […] Most patients diagnosed with Brugada syndrome are asymptomatic and present a greater challenge for risk stratification and management decisions. While the established risk of arrhythmic events is much lower for asymptomatic patients at about 1% annually overall, many cases of SCD still occur in this population.

• #22 Brugada Syndrome: Defining the Risk in Asymptomatic Patients | AER Journal

  https://www.aerjournal.com/articles/brugada-syndrome-defining-risk-asymptomatic-patients?language_content_entity=en

  No specific statement is made for asymptomatic patients. […] Risk stratification in BS remains under active investigation. […] In our experience, three variables should be taken into special consideration in asymptomatic patients: a spontaneous type 1 ECG, the presence of SND and inducible VAs during programmed stimulation of the heart. […] A negative predictive value of the test is 98 %, making a patient very unlikely to present future events.

• #23 Brugada Syndrome – Brigham and Women’s Hospital

  https://www.brighamandwomens.org/heart-and-vascular-center/diseases-and-conditions/brugada-syndrome

  Brugada syndrome is a newly discovered, newly defined disease. The condition occurs much more frequently in people of Asian ancestry, particularly in Southeast Asian populations. The exact prevalence of Brugada syndrome is unknown, although it is estimated to affect five in 10,000 people worldwide, according to the National Institutes of Health. […] Although the average age of a patient with sudden cardiac death is 35 to 40 years old, patients with inherited arrhythmias may range from 6 months to 75 years old. […] If you have Brugada syndrome, it is important that your siblings and children be screened for the condition.

• #24 Où dois-je aller pour me faire dépister pour le syndrome de Brugada ?

  https://www.vietnam.vn/fr/hoi-chung-brugada-nen-di-kham-o-dau

  Le syndrome de Brugada est un trouble de l’activit lectrique du cur qui entrane des troubles du rythme cardiaque potentiellement mortels. […] Le syndrome de Brugada est lune des causes de mort subite chez les jeunes (lge moyen de la mort subite est de 40 ans). […] De nombreux patients atteints du syndrome de Brugada ne savent pas quils sont atteints de la maladie et ne sont diagnostiqus que par hasard. […] Lorsque les symptmes et facteurs de risque ci-dessus sont prsents, le patient doit tre examin par un cardiologue – spcialiste de l’arythmie pour un examen clinique et un lectrocardiogramme (ECG), ventuellement un test gntique (dtecte environ 30 35 % des cas), un examen lectrophysiologique… […] Il nexiste actuellement aucun remde contre le syndrome de Brugada, seulement des traitements pour protger les patients des consquences possibles darythmies ventriculaires dangereuses.

• #25 Top Published Expert Doctors for Brugada Syndrome

  https://findexpertmd.com/d/Brugada_Syndrome

  731 top medical experts on Brugada Syndrome across 45 countries and 31 U.S. states, including 592 MDs (Physicians). This is based on an objective analysis of their Scientific Publications, Clinical Trials, Medicare, and NIH Grants. […] Clinical Trials ClinicalTrials.gov: at least 40 including 1 Active, 10 Completed, 14 Recruiting.

• #26 Brugada syndrome | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/brugada-syndrome?lang=us

  Age of diagnosis ranges from 2 days to 84 years old. It is estimated to cause 4% of all sudden deaths and in excess of 20% of sudden deaths that occurs in patients without structural cardiac abnormalities. It is the second leading cause of death in males under 40 years old in the countries in which it is endemic (primarily located in Southeast Asia) 1.

• #27 Long QT/Brugada Syndrome NGS Panel | Fulgent Genetics

  https://www.fulgentgenetics.com/long-qt-brugada-syndrome-focus

  The Long QT/Brugada Syndrome Panel examines 34 genes associated with hereditary Long QT syndrome (LQTS) and Brugada syndrome. […] Patients identified with LQTS or Brugada syndrome can benefit from increased surveillance and preventative steps to better manage their risks.

• #28 Brugada syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Brugada_syndrome

  Between 1 and 30 per 10,000 people are affected by Brugada syndrome. […] Brugada syndrome is more common in people of Asian descent and is the most common cause of sudden death in young males without known underlying cardiac disease in Thailand and Laos. […] Type 1 Brugada ECG patterns are seen more frequently in Asian populations (00.36%) than those in Europe (00.25%) and the United States (0.03%). Similarly, Type 2 and Type 3 ECG patterns are more prevalent in Asia (0.122.23%) than in Europe (0.00.6%) or the United States (0.02%).

• #29 Brugada syndrome: a review and the role of epicardial ablation in management | The Egyptian Heart Journal | Full Text

  https://tehj.springeropen.com/articles/10.1186/s43044-024-00485-3

  Brugada syndrome (BrS) exhibits a relatively low incidence, estimated to be less than 1% of the population. However, despite its infrequency, this syndrome accounts for more than 10% of all cases of sudden death and can be responsible for up to 20% of sudden deaths in individuals with structurally normal hearts. Notably, BrS demonstrates a clear male predominance, with an approximate male-to-female ratio of 8:1. The typical age of presentation for BrS is around 40 years, although it is important to recognize that it can manifest at a younger age too. […] Stratifying the risk of sudden cardiac death (SCD) in patients with BrS is a challenging yet essential task, considering that the first manifestation of the syndrome for most patients is cardiac arrest or sudden cardiac death. Consequently, the identification and management of asymptomatic individuals at high risk of sudden death pose significant challenges within the realm of BrS management.

• #30 Brugada Syndrome: Progress in Diagnosis and Management | AER Journal

  https://www.aerjournal.com/articles/brugada-syndrome-progress-diagnosis-and-management?language_content_entity=en

  Identification and management of asymptomatic subjects at high risk of sudden death represent the major challenges in BrS. […] The recent guidelines neither encourage nor discourage electrophysiological study and VT/VF inducibility patterns for BrS stratification in patients with BrS. […] Management of patients with BrS continues to be challenging. There are limited therapeutic options, essentially ICD implantation and quinidine. […] An ICD is always indicated in symptomatic BrS, i.e. resuscitated cardiac arrest and/or non-vagal syncope, or nocturnal agonal respiration. […] Although effective for preventing sudden cardiac death, ICD also carries a relevant risk of complications over the patients lifetime, particularly if the patient is younger at the time of device implantation. […] ESC guidelines strongly recommend that all BrS patients should be educated about modulating or precipitating factors and taught to avoid these.

• #31 Brugada syndrome: clinical and genetic findings | Genetics in Medicine

  https://www.nature.com/articles/gim201535

  Taking all data into account, to date, genetic analysis has not been helpful in determining clinical risk stratification. […] In summary, few things are clear from the risk stratification data: symptomatic patients are at higher risk than asymptomatic ones; sudden death survivors are at higher risk than patients with syncope; males are at higher risk than females; patients with type I ECG at baseline have a higher risk than those who require class I antiarrhythmics; and asymptomatic patients may also die suddenly. […] Currently, the ICD is the only effective therapeutic strategy for the prevention of SCD in BrS patients. […] Despite its effectiveness, the ICD has several disadvantages, including high cost, potential procedural complications at implantation, multiple device replacements, and psychological effects due to high rates of complications, mainly inappropriate shocks. […] Finally, several medications, antidepressants, antiarrhythmics, and anesthetics have been associated with an increased risk of development of ST elevation and even arrhythmias in genetically predisposed individuals.

• #32 Brugada syndrome: a review and the role of epicardial ablation in management | The Egyptian Heart Journal | Full Text

  https://tehj.springeropen.com/articles/10.1186/s43044-024-00485-3

  Given the potentially catastrophic consequences associated with SCD in BrS, efforts to refine risk stratification strategies and identify novel predictive markers are ongoing. Future research endeavors aimed at establishing standardized risk stratification approaches and incorporating both invasive and noninvasive methods hold promise in improving the management and outcomes of individuals with BrS.

• #33 Insights for Stratification of Risk in Brugada Syndrome | ECR Journal

  https://www.ecrjournal.com/articles/insights-stratification-risk-brugada-syndrome?language_content_entity=en

  Risk stratification in BrS patients is challenging. Implantation of an ICD is generally accepted if the estimated 5-year risk is higher than about 6.0% or the annual risk is higher than about 1.2%. […] Many different variables and interventions are being studied to increase the diagnostic yield and to maximise patient protection. Meanwhile, the scientific community has to increase efforts in providing patient care according to knowledge and research for improving stratification of risk.

• #34 Brugada Syndrome within Asian Populations: State-of-the-Art Review

  https://www.mdpi.com/2035-8148/13/2/7

  Despite multiple revisions and improvements to diagnostic criteria, risk stratification remains a significant challenge for BrS patients. […] The estimated prevalence of asymptomatic BrS (ECG Type 1) and Type 2/3 BrP in Asia ranges from 0.00% to 1.77% and 0.014% to 15.96%, respectively. However, it is worth noting that most of these studies were conducted in adult males, and the Shanghai score may underestimate the true prevalence of Brugada syndrome. […] Overall, these results demonstrate a significantly higher prevalence of all three Brugada phenotypes (symptomatic and asymptomatic) in Thailand within South Asia, and even more so than in any other Asian country. […] The most accepted theory behind such higher prevalence in Asia centers around ethnic-specific polymorphisms that may modulate the activity of the primary disease-causing variant. While there are higher rates of prevalence within Asia, studies assessing clinical outcomes note similar risk factors for non-Asian patients when evaluating the risk for SCD or malignant arrhythmias.

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