Exercise Stress Test in The Assessment of Brugada Syndrome Patients

Abstract Brugada syndrome (BS) is a genetic channelopathy, clinically characterized by an increased risk of sudden cardiac death. The diagnosis requires a typical electrocardiographic pattern, and data on risk stratification are limited in the literature. The aim of this study was to conduct a review on the importance of exercise stress test (EST) in risk stratification in BS. Articles were searched in the PubMed, Scielo and Google Scholar databases. From the 200 articles retrieved, eight were included, with a total of 712 patients (95% men) aged between 35 and 60 years. Severe symptoms and ventricular arrhythmias were reported by 256 patients before the EST, with syncope being reported in 70% of cases. The reviewed articles suggest that the EST is a safe method that can help in the diagnosis and risk stratification for malignant arrhythmias in patients with BS. Potential predictors of poor prognosis were: augmentation in ST-segment elevation > 0.5 mV in V1, V2 or V3 in early recovery; J-point elevation in lead > 2mm in aVR in late recovery; heart rate reduction < 40% from maximum heart rate in late recovery and occurrence of ventricular extrasystoles in early recovery.

Síndrome de Brugada: 30 Anos de Aventura Científica / Brugada Syndrome: 30 Years of Scientific Ventures

Resumo Trinta anos atrás, uma nova síndrome clínico-eletrocardiográfica distinta foi descrita, agora conhecida como síndrome de Brugada (SBr). Típico para essa síndrome é o eletrocardiograma com supradesnivelamento do segmento ST nas derivações precordiais direitas. A apresentação clínica da doença é altamente variável: os pacientes podem permanecer completamente assintomáticos, mas também podem desenvolver episódios de síncope, fibrilação atrial (FA), síndrome do nódulo sinusal (SNS), distúrbios de condução, assistolia e fibrilação ventricular (FV). A doença é causada por mutações nos genes responsáveis pelo potencial de ação das células do coração. O gene mais frequentemente envolvido é o SCN5A, que controla a estrutura e função do canal de sódio cardíaco. A descrição dessa nova síndrome teve implicações muito positivas em todos os campos da medicina.

Abstract Thirty years ago, a distinctly new clinical-electrocardiographic syndrome was described, today known as Brugada Syndrome (BrS). Typical treatment for this type of syndrome is electrocardiography with ST-segment elevation in the direct precordial derivations. The clinical presentation of the disease is highly variable: the patients can remain completely asymptomatic, but they can also develop episodes of syncope, atrial fibrillation (AF), sinus node dysfunction (SNF), conduction disorders, asystole, and ventricular fibrillation (VF). This disease is caused by mutations in the genes responsible for the potential action of cardiac cells. The most commonly involved gene is SCN5A, which controls the structure and function of the heart's sodium channel. The description of this new syndrome has shown highly positive implications in all fields of medicine.

Brugada syndrome unmasked by fever and paradoxical lower degree of dromotropic disturbance in the right ventricular outflow tract

Brugada syndrome (BrS) is an inherited clinical-electrocardiographic arrhythmic entity with an autosomal dominant genetic pattern of inheritance or de novo variant. The syndrome has low worldwide prevalence, but is endemic in Southeast Asian countries (Thailand, Philippines and Japan). The BrS is a subtle structural heart disease (SHD), and the diagnosis is only possible when the so-called type 1 Brugada ECG pattern is spontaneously present or induced for example with fever. Repolarization-depolarization disturbances in BrS patients can be caused by genetic mutations, abnormal neural crest cell migration, low expression of connexin-43 gap junction protein, or connexome disturbances. A recent autopsy study revealed increase in biventricular collagen with myocardial fibrosis when compared with control subjects although the main affected cardiac territory is the right ventricular outflow tract (RVOT). In this location, there is abnormally low expression of significant connexin-43 gap junction responsible for the electro-vectorcardiographic manifestations of terminal QRS conduction delay in the right standard precordial leads (V1-V2), high right precordial leads (V1H-V2H), as well as in the unipolar aVR lead ("the forgotten lead"). Based on their location, these leads reflect the electrical activity of the RVOT.

A síndrome de Brugada (SBr) é uma entidade arrítmica clínico-eletrocardiográfica hereditária com padrão genético autossômico dominante de herança ou variante de novo. A síndrome tem baixa prevalência mundial, porém sendo endêmica no Sudeste Asiático (Tailândia, Filipinas e Japão). A SBr é uma doença cardíaca minimamente estrutural, sendo o diagnóstico só possível na presença do chamado padrão ECG de Brugada tipo 1 espontâneo ou induzido, por exemplo, a febre. Os distúrbios de repolarização-despolarização em pacientes com SBr podem ser causados por mutações genéticas responsáveis pela migração anormal de células da crista neural, baixa expressão "gap junctions" conexina-43 ou distúrbios do conexoma. Um estudo recente de autópsia revelou aumento do colágeno biventricular com fibrose miocárdica quando comparado aos controles, embora o principal território cardíaco afetado seja a via de saída do ventrículo direito (VSVD). Nessa área, há menor expressão da conexina-43, o que se traduz no ECG-VCG por atraso final de condução do QRS nas derivações precordiais direitas (V1-V2), precordiais direitas altas (V1H-V2H), bem como na derivação unipolar aVR ("a derivação esquecida"). Com base em sua localização, esses eletrodos refletem a atividade elétrica da VSVD

Bloqueo del plano transverso abdominal para control del dolor intra y posoperatorio en herniorrafia inguinal en paciente con síndrome de Brugada / Block of transverse abdominal pain for intra-and posoperative pain control in inguinal herniorrhaphy in patient with Brugada syndrome

Los pacientes con síndrome de Brugada suponen un reto desde el punto de vista anestésico ya que son muchas las circunstancias perioperatorias y los fármacos que pueden desencadenar una crisis arritmogénica, los cuales pueden llegar a tener un fatal desenlace. Las implicaciones anestésicas en estos pacientes son múltiples por lo que se debe ser muy cuidadoso y tener en cuenta muchos factores. Se exponen las consideraciones y el manejo anestésico que se realizó en un paciente con síndrome de Brugada sometido a una herniorrafía inguinal izquierda en la que se opta por la realización de una anestesia general junto con un bloqueo ecoguiado del plano transverso abdominal para control de dolor(AU)

Patients with Brugada syndrome are a challenge from the anesthetic point of view, since there are many perioperative circumstances and drugs that can trigger an arrhythmogenic crisis, which can have a fatal outcome. Anesthetic implications in these patients are multiple, a reason why one must be very careful and take into account many factors. The considerations and anesthetic management are described for the case of a patient with Brugada syndrome who underwent left inguinal herniorrhaphy, in which general anesthesia was chosen together with ultrasound-guided block of transverse abdominal plane for pain control(AU)

Prevalência e características relacionadas de pacientes com eletrocardiograma com padrão de brugada em Santa Catarina, Brasil / Prevalence and related characteristics of patients with brugada pattern electrocardiogram in Santa Catarina, Brazil

Resumo Fundamento: A síndrome de Brugada é um distúrbio arritmogênico hereditário caracterizado pela presença de características eletrocardiográficas específicas com ou sem sintomas. Os pacientes apresentam risco aumentado de morte súbita por fibrilação ventricular. A prevalência desse padrão eletrocardiográfico difere de acordo com a região estudada. Porém, informações epidemiológicas, incluindo a população brasileira, são escassas. Objetivo: Avaliar a prevalência do padrão eletrocardiográfico da síndrome de Brugada e o perfil epidemiológico associado a ela. Métodos: Estudo transversal que incluiu 846.533 registros ECG de 716.973 pacientes do banco de dados de eletrocardiograma (ECG) da Rede de Telemedicina de Santa Catarina por um período de quatro anos. Todos os exames foram ECG de 12 derivações convencionais (sem V1 e V2 em posições altas). Os exames identificados com o diagnóstico de "Síndrome de Brugada" (tipos 1 e 2) foram revisados por um eletrofisiologista. Foram considerados significativos valores de p<0,05. Resultados: Apresentavam padrão potencialmente consistente com ECG do tipo Brugada 83 pacientes. Destes, 33 foram confirmados com padrão de Brugada tipo 1, e 22 com tipo 2, após reavaliação. A prevalência de ECG do tipo 1 de Brugada foi de 4,6 por 100.000 pacientes. O ECG do tipo Brugada 1 foi associado ao sexo masculino (81,8% vs. 41,5%, p<0,001) e menor prevalência de obesidade (9,1% vs. 26,4%, p=0,028). Conclusões: Este estudo mostrou baixa prevalência de ECG do tipo Brugada no sul do Brasil. A presença de ECG com padrão Brugada tipo 1 esteve associada ao sexo masculino e menor prevalência de obesidade que a população geral.

Abstract Background: Brugada Syndrome is an inherited arrhythmogenic disorder characterized by the presence of specific electrocardiographic features with or without clinical symptoms. The patients present increased risk of sudden death due to ventricular fibrillation. The prevalence of this electrocardiographic pattern differs according to the studied region. However, epidemiological information including the Brazilian population is scarce. Objectives: To assess the prevalence of the electrocardiographic pattern of Brugada syndrome and the epidemiological profile associated with it. Methods: Cross-sectional study that included 846,533 ECG records of 716,973 patients from the electrocardiogram (ECG) database from the Santa Catarina Telemedicine Network over a 4-year period. All tests were 12-lead conventional ECG (without V1 and V2 in high positions). The tests revealing "Brugada Syndrome" diagnosis (Types 1 and 2) were reviewed by a cardiac electrophysiologist. The level of significance was set at p<0.05. Results: In total, 83 patients had a pattern potentially consistent with Brugada-type pattern ECG. Of these, 33 were confirmed having Brugada-type 1, and 22 with type 2 ECG after reevaluation. The prevalence of Brugada-type 1 ECG was 4.6 per 100,000 patients. Brugada-type 1 ECG was associated with the male gender (81.8% vs. 41.5%, p<0.001) and a lower prevalence of obesity diagnosis (9.1% vs. 26.4%, p=0.028). Conclusions: This study showed low prevalence of Brugada-type ECG in Southern Brazil. The presence of Brugada-type 1 ECG was associated with the male gender and lower prevalence of obesity diagnosis comparing to the general population.

Peculiaridade dos pacientes com arritmias hereditárias na pandemia pela COVID-19 / Peculiar aspects of patients with Inherited arrhythmias during the COVID-19 pandemic

Resumo Desde dezembro de 2019, observamos o rápido avanço da síndrome respiratória aguda grave causada pelo coronavírus 2019 (SARS-CoV-2). O impacto da evolução clínica de uma infecção respiratória é pouco conhecido em pacientes portadores de arritmias hereditárias, devido à baixa prevalência dessas doenças. Os pacientes que apresentam quadros infecciosos podem exacerbar arritmias primárias ocultas ou bem controladas, por diversos fatores, tais como febre, distúrbios eletrolíticos, interações medicamentosas, estresse adrenérgico e, eventualmente, o próprio dano miocárdico do paciente séptico. O objetivo desta revisão é destacar os principais desafios que podemos encontrar durante a pandemia pela Covid 19, especificamente nos pacientes com arritmias hereditárias, com destaque para a síndrome do QT longo congênito (SQTL), a síndrome de Brugada (SBr), a taquicardia ventricular polimórfica catecolaminérgica (TVPC) e a cardiomiopatia arritmogênica do ventrículo direito.

Abstract Since December 2019 we have observed the rapid advance of the severe acute respiratory syndrome caused by the new coronavirus (SARS-CoV-2). The impact of the clinical course of a respiratory infection is little known in patients with hereditary arrhythmias, due to the low prevalence of these diseases. Patients who present with infectious conditions may exacerbate hidden or well-controlled primary arrhythmias, due to several factors, such as fever, electrolyte disturbances, drug interactions, adrenergic stress and, eventually, the septic patient's own myocardial damage. The aim of this review is to highlight the main challenges we may encounter during the Covid 19 pandemic, specifically in patients with hereditary arrhythmias, with emphasis on the congenital long QT syndrome (LQTS), Brugada syndrome (SBr), ventricular tachycardia polymorphic catecholaminergic (CPVT) and arrhythmogenic right ventricular cardiomyopathy.

Muerte súbita por fibrilación ventricular en deportista: una presentación inusual del Síndrome de Brugada. Caso Clínico / Sudden cardiac death during exercise due to Brugada syndrome. report of one case

We report a 20-year-old male athlete who while running, presented a sudden death due to ventricular fibrillation. He was successfully rescued by cardiopulmonary resuscitation maneuvers and an automatic external defibrillator. Without evidence of structural heart disease, Brugada syndrome was diagnosed as the cause, after which a subcutaneous implantable cardioverter defibrillator was indicated. We discuss the subject of sudden cardiac death in athletes and its unusual relationship with exercise in this channelopathy.

Brugada syndrome: current concepts and genetic background / Síndrome de Brugada: conceitos atuais e antecedentes genéticos

BACKGROUNG: Brugada syndrome (BrS) is a hereditary clinical-electrocardiographic arrhythmic entity with low worldwide prevalence. The syndrome is caused by changes in the structure and function of certain cardiac ion channels and reduced expression of Connexin 43 (Cx43) in the Right Ventricle (RV), predominantly in the Right Ventricular Outflow Tract (VSVD), causing electromechanical abnormalities. The diagnosis is based on the presence of spontaneous or medicated ST elevation, characterized by boost of the J point and the ST segment ≥2 mm, of superior convexity "hollow type" (subtype 1A) or descending rectilinear model (subtype 1B). BrS is associated with an increased risk of syncope, palpitations, chest pain, convulsions, difficulty in breathing (nocturnal agonal breathing) and/or Sudden Cardiac Death (SCD) secondary to PVT/VF, unexplained cardiac arrest or documented PVT/VF or Paroxysmal atrial fibrillation (AF) in the absence of apparent macroscopic or structural heart disease, electrolyte disturbance, use of certain medications or coronary heart disease and fever. In less than three decades since the discovery of Brugada syndrome, the concept of Mendelian heredity has come undone. The enormous variants and mutations found mean that we are still far from being able to concretely clarify a genotype-phenotype relationship. There is no doubt that the entity is oligogenetic, associated with environmental factors, and that there are variants of uncertain significance, especially the rare variants of the SCN5A mutation, with European or Japanese ancestors, as well as a spontaneous type 1 or induced pattern, thanks to gnomAD (coalition) researchers who seek to aggregate and harmonize exome and genome sequencing data from a variety of large-scale sequencing projects and make summary data available to the scientific community at large). Thus, we believe that this in-depth analytical study of the countless mutations attributed to BrS may constitute a real cornerstone that will help to better understand this intriguing syndrome.

INTRODUÇÃO: A Síndrome de Brugada (SBr) é uma entidade arrítmica clínico-eletrocardiográfica hereditária com baixa prevalência mundial. A síndrome é causada por alterações na estrutura e função de certos canais iônicos cardíacos e redução da expressão da Connexina 43 (Cx43) no Ventrículo Direito (VD), predominantemente no Trato de Saída do Ventricular Direito (VSVD), causando anormalidades eletromecânicas. O diagnóstico é baseado na presença de supradesnivelamento de ST espontâneo ou medicamentoso caracterizado por supradesnivelamento do ponto J e do segmento ST ≥2 mm, de convexidade superior "tipo covado" (subtipo 1A) ou modelo retilíneo descendente (subtipo 1B). A SBr está associado a um risco aumentado de síncope, palpitações, dor precordial, convulsões, dificuldade em respirar (respiração agonal noturna) e/ou Morte Cardíaca Súbita (MSC) secundária a PVT/VF, parada cardíaca inexplicada ou PVT/VF documentado ou Fibrilação atrial paroxística (FA) na ausência de doença cardíaca macroscópica ou estrutural aparente, distúrbio eletrolítico, uso de certos medicamentos ou coração coronário e febre. Em menos de três décadas desde a descoberta da síndrome de Brugada, o conceito de hereditariedade mendeliana se desfez. As enormes variantes e mutações encontradas significam que ainda estamos longe de sermos capazes de esclarecer concretamente uma relação genótipo-fenótipo. Não há dúvida de que a entidade é oligogenética associada a fatores ambientais, e que há variantes de significado incerto, principalmente as raras variantes da mutação SCN5A, com ancestrais europeus ou japoneses, bem como padrão espontâneo tipo 1 ou induzido, graças ao gnomAD (coalizão de pesquisadores que buscam agregar e harmonizar dados de sequenciamento de exoma e genoma de uma variedade de projetos de sequenciamento em grande escala e disponibilizar dados resumidos para a comunidade científica em geral). As enormes variantes e mutações encontradas significam que ainda estamos longe de sermos capazes de esclarecer concretamente uma relação genótipo-fenótipo. Assim, acreditamos que este estudo analítico em profundidade das inúmeras mutações atribuídas à BrS pode constituir uma verdadeira pedra angular que ajudará a compreender melhor esta síndrome intrigante.

Síndrome de brugada en el paciente quirúrgico de urgencia. Reporte de caso / Brugada syndrome in the emergency surgical patient. Case report.

Introducción: El síndrome de Brugada es una condición genética rara, el diagnóstico se establece por un patrón electrocardiográfico en particular que se asocia a un riesgo de fibrilación ventricular y muerte súbita; Objetivo: Presentar un caso interesante de un paciente joven quién ingreso a quirófano con la impresión clínica de apendicitis aguda para realizar apendicetomía video laparoscópica. Durante el procedimiento presento múltiples episodios de taquiarritmias; desencadenando Fibrilación Ventricular de difícil manejo trans y post operatorio, debido a los medicamentos peri-operatorios, agresión quirúrgica y fiebre. Material y Métodos: Se documento y presentó un caso interesante; Presentación de Caso: Paciente masculino de 26ª, sin antecedentes médicos, con impresión clínica de apendicitis aguda ingresa a quirófano, durante el acto quirúrgico presenta arritmias, documentándose Fibrilación Ventricular con inestabilidad hemodinámica que amerito desfibrilación externa, revirtió a ritmo sinusal. Al concluir el acto quirúrgico, se realiza EKG, evidenciando supradesnivel del segmento ST en V1 y V2 e inversión de la onda T por lo que pasa a unidad de cuidados intensivos, sin reversión anestésica y con tubo orotraqueal. Paciente se monitoriza en UTI, se realiza EKG evidencia ritmo nodal. Se extuba a las 12h post operatorias y se traslada a cardiología en 48h donde establecen que paciente cursa con patrón de Brugada. Conclusiones: Con el creciente número de pacientes con trastornos de conducción heredadas que se presentan para cirugía no cardiaca que están en riesgo de muerte súbita; el éxito en el manejo peri, trans y post operatorio depende de un conocimiento detallado de estas condiciones. (AU)

Introduction: Brugada syndrome is a rare genetic condition, the diagnosis is established by a particular electrocardiographic pattern and is associated with a risk of ventricular fibrillation and sudden death; Objective: To present an interesting case of a young patient who enters the operating room with the clinical impression of acute appendicitis to perform laparoscopic appendectomy and that during the procedure present multiple episodes of tachyarrhythmias; triggering Ventricular Fibrillation, what causes him difficult trans and postoperative management, due to peri-operative medications, surgical aggression and fever. Material and Methods: An interesting case was documented and presented; Case Presentation: Male patient of 26 years old, without medical history, with clinical impression of acute appendicitis was admitted to the operating room, during the surgical act it presents arrhythmias, showing Ventricular Fibrillation with hemodynamic instability that warrants external defibrillation, reversed at sinus pace, EKG is performed showing elevation gain of the ST segment in V1 and V2 and inversion of the T wave at the end of the surgical act, so it goes to intensive care unit, without anesthetic reversal and with orotracheal tube. Patient is monitored in ICU, EKG shows nodal rhythm, extubates at 12h post op and at 48h was transferred to cardiology where they establish that patient studies suggest Brugada pattern; Conclusions: With the increasing number of patients with inherited driving disorders who present the thee for non-cardiac surgery who are at risk of sudden death; success in peri, trans and postoperative management depends on a detailed knowledge of these conditions. (AU)

Síndrome de Brugada asociado a pericarditis aguda / Brugada syndrome associated to acute pericarditis

Resumen Comunicamos el caso de un hombre de 56 años que ingresó por pericarditis aguda febril, probablemente de causa viral, que durante su evolución presentó una fibrilación auricular paroxística y taquicardia ventricular polimorfa recurrente. El análisis de la historia clínica y serie electrocardiográfica permitió el diagnóstico retrospectivo asociado de un síndrome de Brugada. Hubo buena respuesta inicial al tratamiento antiinflamatorio asociado a colchicina y se implantó un desfibrilador automático intracavitario para prevención de muerte súbita. Se discuten aspectos clínicos del síndrome de Brugada, la importancia de la fiebre y de la pericarditis como cuadro clínico asociado.

Abstract A 56-year-old patient was admitted for acute febrile pericarditis, probably viral, who presented with paroxysmal atrial fibrillation and recurrent polymorphic ventricular tachycardia during his clinical course. Analysis of the clinical and electrocardiographic findings allowed the retrospective diagnosis of Brugada syndrome in the context of pericarditis. An initial response to anti-inflammatory treatment associated with colchicine was good. An internal cardioverter defibrillator was implanted to prevent sudden death. Clinical aspects of the Brugada syndrome, the importance of fever and pericarditis as an associated clinical condition are discussed.

The numerous denominations of the Brugada syndrome and proposal about how to put an end to an old controversy - a historical-critical perspective

BACKGROUNG: The eponymous Brugada Syndrome (BrS) in honor of its discovery as an independent entity by the Spanish/Catalan Brugada brothers, Pedro and Josep, has deserved numerous denominations derived mainly from the clinical genotype/phenotype correlation. The purpose of this manuscript is to present and analyze the nomenclatures that this intriguing and challenging syndrome has received over the past 28 years. We also compared the main features between cases from the first report of the Brugada brothers and an article by Martini et al. The nomenclatures used by these authors are closely linked to the BrS, but the cases (except one) presented in the article by Martini et al do not present the type 1 Brugada ECG pattern, which is mandatory for the diagnosis of BrS

INTRODUÇÃO: A Síndrome de Brugada (SB), em homenagem à sua descoberta como entidade independente pelos irmãos espanhóis / catalães Pedro e Josep Brugada, tem merecido inúmeras denominações derivadas principalmente da correlação genótipo /fenótipo clínico. O objetivo deste manuscrito é apresentar e analisar as nomenclaturas que esta intrigante e desafiadora síndrome recebeu nos últimos 28 anos. Também comparamos as principais características entre os casos do primeiro relato dos irmãos Brugada e um artigo de Martini e col. As nomenclaturas utilizadas por esses autores estão intimamente ligadas à SB, mas os casos (exceto um) apresentados no artigo de Martini e cols. não apresentam o padrão eletrocardiográfico de Brugada tipo 1, obrigatório para o diagnóstico da SB

Patrón electrocardiográfico Brugada tipo I asociado a un cuadro febril por Influenza B. A propósito de la pandemia de COVID-19. Caso clínico

We report a 44-year-old male who was admitted for Influenza B and fever, presenting a type I Brugada pattern on the electrocardiogram. He evolved without cardiovascular symptoms. The pharmacological test with intravenous Procainamide reproduced type I Brugada pattern and the programmed electrical stimulation was negative for ventricular arrhythmias. He was discharged without incidents. Clinical aspects of Brugada syndrome and the importance of fever are discussed in the current context of the COVID-19 pandemic.

A high number of ‘natural’ mitochondrial DNA polymorphisms in a symptomatic Brugada syndrome type 1 patient

Brugada syndrome (BrS) is a rare genetic arrhythmic disorder with a complex model of transmission. At least 20 different genes have been identified as BrS-causal or susceptibility genes. Of these, SCN5A is the most frequently mutated. Coregulation of different mutations or genetic variants, including mitochondrial DNA (mtDNA), may contribute to the clinical phenotype of the disease. In the present study, we analysed the mitochondrial genome of a symptomatic BrS type 1 patient to investigate a possible mitochondrial involvement recently found in the arrhytmogenic diseases. No pathogenic mutation was identified; however, a high number of singlenucleotide polymorphisms were found (n=21) and some of them were already been reported in molecular autopsy case for sudden death. The results reported here further support our hypothesis on the potential role of mtDNA polymorphisms in mitochondrial dysfunction, which may represent a risk factor for arrhythmogenic disease.

Paro cardiorrespiratorio intraoperatorio en un paciente con síndrome de Brugada / Intraoperative cardiac arrest in a patient with Brugada síndrome

A 61-year-old female patient with history of hipertension is scheduled to undergo a minor ginecological procedure (endoscopic endometrial polipus resection) with general anesthesia. She received standard monitorization, induction with midazolam, propofol and fentanyl. Ventilated with laringeal mask. Anesthesia was maintained with sevoflurane, nitrous oxide and oxygen. During surgical procedure, the patient received atropine and ephedrine associated with two episodes of bradycardia without hemodinamic disturbances. The surgery ended without problems. During the weaking up process she presented characteristical waves of ventricular fibrillation, recuperating sinusal rhythm secondary to defibrillation with 360 J. There was no clear cause for cardiac arrest at that moment so patient was translated to the ICU for observation, monitoring and study. Postoperative EKG presented an ascending ST segment in V to V derivations without hemodynamic alterations associated. The possible diagnosis of Brugada's Syndrome was proposed. The patient received an implantable defibrillator. The mechanisms and anesthetic implications are discussed and reviewed.

Paciente de 61 años, hipertensa, fue sometida a un procedimiento endoscópico menor (histeroscopía) bajo anestesia general balanceada. Recibió monitorización estándar, inducción con midazolam, propofol y fentanilo. Se ventiló con máscara laríngea y la mantención anestésica fue con sevoflurano asociado a NO en O. En dos oportunidades recibió atropina y efedrina para el manejo de bradicardias sin compromiso hemodinámico. Se completó el procedimiento ginecológico sin complicaciones. Durante el despertar anestésico, presentó una fibrilación ventricular que cedió con desfibrilación. Se estabilizó y trasladó a UCI donde se estudió las posibles etiologías. Se obtiene ECG postoperatorio con elevación del segmento ST en derivaciones Va V sin alteraciones hemodinámicas asociadas, postulándose un síndrome de Brugada. Se le instaló desfibrilador implantable. Se revisa y discuten los diversos mecanismos e implicancias anestésicas asociadas.

Catheter Ablation for Brugada Syndrome

Brugada syndrome (BrS) is an arrhythmogenic disease associated with an increased risk of ventricular fibrillation (VF) and sudden cardiac death (SCD). To date, the standard therapy for the prevention of SCD in BrS is the use of an implantable cardioverter-defibrillator (ICD) especially in patients who have experienced a prior cardiac arrest or syncopal events secondary to VF. However, ICDs do not prevent the occurrence of VF but react to defibrillate the VF episode, thereby preventing SCD. Often patients with recurrent VF have to be maintained on antiarrhythmic drugs that are effective but have remarkable adverse effects. An alternative therapy for BrS with recurrent VF is catheter ablation which emerged as an effective therapy in eliminating VF-triggering premature ventricular complexes in limited case series; however, there has been a remarkable progress in effectiveness of catheter ablation since epicardial substrate ablation was first applied in 2011 and such approach is now widely applicable.

Syncope in a 3-year-old male: A case report / 世界急诊医学杂志（英文）

@#Syncope is a common presenting complaint in pediatric emergency departments. The differential diagnosis of syncope is vast, including various neurological and cardiac conditions. Brugada syndrome (BrS) is a primary inherited arrhythmia which can present with syncope but can lead to sudden cardiac deaths due to ventricular fi brillation. BrS is a rare genetic condition, often diagnosed in the adulthood. BrS presenting in childhood, especially symptomatic BrS, is extremely rare. We present a case of a previously healthy 3-year-old male who presented to the emergency department with syncope and was later diagnosed with BrS.

Terapia ablativa en un caso de síndrome de Brugada con tormenta eléctrica / Ablative therapy in a case of Brugada syndrome with an electrical storm / Terapia ablativa em um caso de síndrome de Brugada com tempestade elétrica

Resumen: Los pacientes con síndrome de Brugada pueden presentar arritmias ventriculares malignas y muerte súbita, por lo que frecuentemente tienen indicación de implante de cardiodesfibrilador. Ocasionalmente, estos pacientes pueden tener choques recurrentes difíciles de manejar en la práctica clínica. Estudios recientes han mostrado que la ablación por radiofrecuencia en los pacientes con síndrome de Brugada es posible y efectiva.A una mujer de 55 años con síndrome de Brugada afectada por una tormenta eléctrica, se le efectuó mapeo cardíaco electroanatómico tridimensional y ablación por radiofrecuencia. Durante el estudio electrofisiológico no se observaron arritmias espontáneas o inducidas. Un mapeo de voltaje cardíaco en ritmo sinusal, precedió al uso de ablación por radiofrecuencia para modificación del sustrato arritmogénico en endocardio y epicardio del tracto de salida del ventrículo derecho. El patrón de Brugada desapareció de su electrocardiograma en el período posablativo inmediato. En cuatro meses de seguimiento la paciente no ha tenido arritmias ventriculares ni evidencia electrocardiográfica de patrón de Brugada.La modificación del sustrato arritmogénico por medio de ablación por radiofrecuencia en pacientes con síndrome de Brugada y tormenta eléctrica, puede ser de utilidad en el manejo crítico.

Summary: Patients with Brugada syndrome may have malignant ventricular arrhythmias and sudden cardiac death. Thus, these patients frequently have indication of implantable cardioverter defibrillator. Occasionally, these patients may experience recurrent appropriate cardioverter defibrillator shocks refractory to medical treatment. Recent reports indicate that radiofrequency ablation in Brugada syndrome is feasible, and effective.A 55-year-old woman with Brugada syndrome and an electrical storm, underwent three-dimensional electroanatomic mapping and radiofrequency ablation. During electrophysiological study there were no spontaneous or inducible arrhythmias. Voltage mapping of the right ventricle was created in sinus rhythm and both, endocardial and epicardial substrate modification of right ventricular outflow tract was performed. Brugada ECG pattern resolved post procedure. Follow up at 4 months revealed no recurrence of ventricular arrhythmias or Brugada electrocardiographic pattern.Radiofrequency mediated therapy for substrate modification in patients with Brugada syndrome and an electrical storm, may be useful in their critical care management.

Resumo: Os pacientes com síndrome de Brugada podem apresentar arritmias ventriculares malignas e morte súbita, de modo que frequentemente têm indicação de implante de cardiodefibrilador. Ocasionalmente, esses pacientes podem ter choques recorrentes difíceis de lidar na prática clínica. Estudos recentes mostraram que a ablação por radiofrequência em pacientes com síndrome de Brugada é possível e eficaz.Uma mulher de 55 anos com síndrome de Brugada afetada por uma tempestade elétrica apresentava mapeamento cardíaco eletroanatômico tridimensional e ablação por radiofrequência. Durante o estudo eletrofisiológico não foram observadas arritmias espontâneas ou induzidas. O mapeamento da tensão cardíaca em ritmo sinusal antecedeu o uso da ablação por radiofrequência para modificação do substrato arritmogênico no endocárdio e no epicárdio da via de saída do ventrículo direito. O padrão de Brugada desapareceu de seu eletrocardiograma no período pós-ablativo imediato. Em 4 meses de acompanhamento, o paciente não apresentou arritmias ventriculares ou evidências eletrocardiográficas do padrão de Brugada.A modificação do substrato arritmogênico por meio da ablação por radiofrequência em pacientes com síndrome de Brugada e tempestade elétrica pode ser muito útil no manejo crítico.