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)

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.

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.

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.

Cohort of Patients Referred for Brugada Syndrome Investigation in an Electrophysiology Service - 19-Year Registry / Coorte de Pacientes Encaminhados para Investigação de Síndrome de Brugada em um Serviço Terciário de Eletrofisiologia - Registro de 19 Anos

Abstract Background: Brugada syndrome (SBr) is an arrhythmic condition characterized by ST-T segment abnormalities in the right precordial leads associated with a high risk of ventricular arrhythmias and sudden death. Local data regarding the clinical characteristics of patients with a typical electrocardiographic (ECG) pattern undergoing electrophysiological study are scarce. Objective: To evaluate patients with an ECG pattern suggestive of SBr referred for electrophysiological evaluation in a specialized center. Methods: Cohort study of patients referred for electrophysiological study because of an ECG pattern compatible with SBr between January 1998 and March 2017. Results: Of the 5506 procedures, 35 (0.64%) were for SBr investigation, 25 of which (71.42%) were performed in men. The mean age was 43.89 ± 13.1 years. The ECG patterns were as follows: type I, 22 (62.85%); type II, 12 (34.30%); and type III, 1 (2.85%). Twenty-three patients (65.7%) were asymptomatic, 6 (17.14%) had palpitations, 5 (14.3%) had syncope, and 3 (8.6%) had a family history of sudden death. Electrophysiological study induced ventricular tachyarrhythmias in 16 cases (45.7%), the mean ventricular refractory period being 228 ± 36 ms. Ajmaline / procainamide was used in 11 cases (31.4%), changing the ECG pattern to type I in 7 (63.6%). Sixteen cases (45.7%) received an implantable cardioverter defibrillator (ICD). In a mean 5-year follow-up, 1 of the 16 patients (6.25%) with ICD had appropriate therapy for ventricular fibrillation. There was no death. Other arrhythmias occurred in 4 (11.4%) cases. Conclusions: Most patients are men, and a type I ECG pattern is the main indication for electrophysiological study. Class IA drugs have a high ECG conversion rate. The ICD event rate was 6%. (Arq Bras Cardiol. 2018; [online].ahead print, PP.0-0)

Resumo Fundamento: Síndrome de Brugada (SBr) é uma condição arrítmica definida por anormalidades do segmento ST-T em derivações V1-V3 associada a risco elevado de arritmias ventriculares e morte súbita. Dados locais quanto às características clínicas dos pacientes com padrão eletrocardiográfico (ECG) típico avaliados por estudo eletrofisiológico (EEF) são escassos. Objetivo: Avaliar pacientes com padrão ECG sugestivo de SBr encaminhados para EEF em um centro especializado. Métodos: Estudo de coorte de casos encaminhados para EEF por padrão ECG compatível com SBr, entre janeiro de 1998 e março de 2017. Resultados: Dos 5506 procedimentos, 35 (0,64%) foram para investigação de SBr. Vinte e cinco (71,42%) eram homens. Idade média 43,89 ± 13,1 anos. Apresentação ECG foi tipo I em 22 casos (62,85%), tipo II em 12 (34,30%) e tipo III em 1 (2,85%). Vinte e três (65,7%) eram assintomáticos, 6 (17,14%) apresentavam palpitações, 5 (14,3%) síncope, 3 (8,6%) história familiar de morte súbita. Estudo eletrofisiológico induziu taquiarritmias ventriculares em 16 casos (45,7%), sendo o período refratário ventricular médio de 228 ± 36 ms. Utilizou-se ajmalina/procainamida em 11 casos (31,4%), sendo o padrão ECG transformado em tipo I em 7 (63,6%). Dezesseis casos (45,7%) receberam cardiodes fibrilador (CDI). Em seguimento médio de 5 anos, 1 dos 16 pacientes (6,25%) com CDI teve terapia apropriada para fibrilação ventricular. Nenhuma morte foi registrada. Outras arritmias ocorreram em 4 (11,4%) casos. Conclusões: Homens são maioria, sendo o padrão ECG tipo I a principal indicação de EEF. Droga classe IA possui alta taxa de conversão do padrão ECG. A taxa de eventos no CDI foi de 6%. (Arq Bras Cardiol. 2018; [online].ahead print, PP.0-0)

Brugada Syndrome: A Major Cause of Sudden Cardiac Death.

In 1992 a new syndrome was described consisting of syncopal episodes or sudden death in patients with a structurally normal heart and an electrocardiogram characteristic of right bundle branch block with ST segment elevation in leads V1 to V3. Brugada syndrome is an autosomal dominant disorder. It has been shown to be associated with mutations in the gene (SCN5A) that encodes for the sodium ion channel in cardiac myocyte. Over 160 mutations of gene SCN5A have been identifi ed. Th e incidence of the disease is diffi cult to estimate, but it causes sudden deaths of 5 per 10,000 inhabitants per year and involved much more frequently in people of Asian ancestry. Diagnosis can be easily made by means of genetic analysis and ECG. Recent data suggest that loss of the action potential dome in the right ventricular epicardium underlies ST segment elevation seen in this syndrome. Right ventricular epicardium is preferentially aff ected because of the predominance of transient outward current in this tissue. Antiarrhythmic drugs like amiodarone and beta-blockers do not prevent death in symptomatic or asymptomatic individuals. Th ough Implantation of an automatic cardioverter–defi brillator is the only recently proven eff ective therapy; Quinidine has been found to decrease Ventricular fi brillation and could prove to be a secured option of implantable cardioverter–defi brillator. However, researcher set focus on gene therapy that may off er an enduring cure in future years. Th e purpose of this brief review is to record the past highlights that have brought us to our present understanding of Brugada syndrome.

Muerte súbita cardiaca en el corazón estructuralmente normal: una actualización / Sudden cardiac death in individuals with normal hearts: an update

La muerte súbita (MS) es un evento trágico que representa un grave problema de salud. Se estima que causa cerca de 4-5 millones de decesos por año en todo el mundo. La MS se define como la muerte ocurrida en el lapso de 1 h en una persona sin signos previos de fatalidad; puede denominarse «recuperada¼, cuando el paciente afectado sobrevive al episodio potencialmente fatal ya sea por reanimación cardiopulmonar o desfibrilación efectiva. Las canalopatías arritmogénicas son alteraciones funcionales de los canales iónicos del corazón, generalmente condicionados por mutaciones en los genes que los codifican y dan lugar a diversos tipos de arritmias que pueden culminar en MS, el deceso ocurre normalmente antes de los 40 años y el corazón en estudio de autopsia suele ser estructuralmente normal. En la presente revisión presentamos las principales causas de MS en el contexto del corazón estructuralmente normal y discutimos el abordaje que se debe dar a los pacientes y familiares de víctimas que han experimentado éste trágico evento.

Sudden death (SD) is a tragic event and a world-wide health problem. Every year, near 4-5 million people experience SD. SD is defined as the death occurred in 1 h after the onset of symptoms in a person without previous signs of fatality. It can be named «recovered SD¼ when the case received medical attention, cardiac reanimation effective defibrillation or both, surviving the fatal arrhythmia. Cardiac channelopathies are a group of diseases characterized by abnormal ion channel function due to genetic mutations in ion channel genes, providing increased susceptibility to develop cardiac arrhythmias and SD. Usually the death occurs before 40 years of age and in the autopsy the heart is normal. In this review we discuss the main cardiac channelopathies involved in sudden cardiac death along with current management of cases and family members that have experienced such tragic event.

Posible valor pronóstico de la fibrilación y el flutter auricular en el síndrome de Brugada / Possible prognostic value of atrial fibrillation and atrial flutter in Brugada syndrome

Objetivo: Analizar el riesgo específico para las variables: patrón tipo 1 espontáneo, patrón tipo 1 inducido, patrón tipo 1 con variabilidad espontánea, síncope, antecedente de muerte súbita familiar, fibrilación auricular y flutter auricular, con el desarrollo subsecuente de eventos arrítmicos malignos. Métodos: Se analizaron retrospectivamente 43 pacientes con síndrome de Brugada (90% hombres; edad media 40.4 años), con patrón tipo 1 espontáneo (74.4%) o inducido por ajmalina (25.6%). El 58.1% presentó variabilidad espontánea. El 18.6% tenía antecedente de muerte súbita familiar. El 39.5% era sintomático. El 18.6% presentó fibrilación auricular o flutter auricular. El antecedente de un paro cardiaco resucitado, muerte súbita o choque apropiado del desfibrilador automático implantable se consideró evento arrítmico maligno. Durante un seguimiento promedio de 51 meses no se registraron muertes, el 6.9% de los pacientes presentó un evento arrítmico maligno, y en su totalidad fueron choques apropiados. La tasa anual de eventos arrítmicos malignos en los pacientes con síncope fue del 1.7%, con un patrón tipo 1 espontáneo, del 2.79%, y con variabilidad espontánea, del 2.87%. No se observaron eventos arrítmicos malignos en los asintomáticos y en aquellos con un patrón persistente o tipo 1 inducido. La tasa anual de eventos con antecedente positivo o negativo de muerte súbita familiar fue del 2.94 y 1.7%, respectivamente. En presencia de fibrilación auricular, flutter auricular y fibrilación auricular/flutter auricular fue del 7.3, 15.69 y 10%, respectivamente. En ausencia de fibrilación/flutter no se observaron eventos arrítmicos malignos. Conclusión: De las variables analizadas, las que tuvieron mayor relación con la presencia de un evento arrítmico maligno fueron la presencia de fibrilación auricular (p = 0,046) y de flutter auricular (p = 0,03).

Objective: To analyze the specific risk for the variables: type 1 spontaneous pattern, type 1 induced pattern, type 1 pattern with spontaneous variability, syncope, family history of sudden death, atrial fibrillation and atrial flutter with the subsequent development of malignant arrhythmic events. Methods: Forty-three Brugada patients (90% males; mean age 40.4 years), with a type 1 spontaneous pattern (74.4%) or induced by ajmaline (25.6%) were retrospectively analyzed. Of these, 58.1% presented spontaneous variability, 18.6% had family history of sudden death, 39.5% were symptomatic, and 18.6% presented atrial fibrillation or atrial flutter. The antecedents of resuscitated cardiac arrest, sudden death or appropriate shock from implantable automatic defibrillator were considered malignant arrhythmic events. During a mean follow up of 51 months, no deaths were recorded, 6.9% of the patients presented a malignant arrhythmic event, and all of them were appropriate shocks. The annual rate of events in patients with syncope was 1.7%, with a spontaneous type 1 pattern was 2.79%, and spontaneous variability was 2.87%. No malignant arrhythmic event was observed in asymptomatic patients or in those with a persistent pattern or induced type 1 pattern. The annual rate of events with positive or negative history of family sudden death was 2.94 and 1.7%, respectively. In the presence of atrial fibrillation, atrial flutter and atrial fibrillation/atrial flutter were 7.3, 15.69 and 10%, respectively. In the absence of atrial fibrillation/atrial flutter no malignant arrhythmic events were observed. Conclusion: Of the variables analyzed, the one that was most related to a malignant arrhythmic event was the presence of atrial fibrillation (P= .046) and atrial flutter (P= .03).

Exarcerbação clínica da Síndrome de Brugada em pacientes com febre / Clinical exacerbation of Brugada Syndrome in a patient with fever

A síndrome de Brugada (BRS) é uma alteração elétrica na ausência de cardiopatia estrutural subjacente aparente, herdada por um gene autossômico dominante. As mutações foram identificadas no gene que codifica a subunidade SCN5A do canal de sódio voltagem dependente Nav1.5. Relata-se o caso de paciente de 56 anos, sexo masculino, diagnosticado com BRS comcontaminação de uma endoprótese vascular migrada, levando à disfunção do desfibrilador implantávelautomático (CDI) e dois tipos de complicações: episódiosarritmogênicos associados à febre que levou a acionamentos apropriados do CDI e episódios caracterizados por descargas inadequadas do CDI.

Supraventricular tachyarrhythmia and Brugada syndrome: a case of Atrial Fibrillation and Brugada syndrome in a young patient without structural heart disease / Taquiarritmia supraventricular y síndrome de Brugada: un caso de fibrilación atrial y síndrome de Brugada en un paciente joven sin cardiopatía estructural

We report a case of a 29-year old man who initially presented with a single episode of syncope. The initial electrocardiogram (ECG) showed atrial fibrillation and an ST segment elevation on lead V1. A flecainide test unmasked the Brugada syndrome. The pathophysiology of Brugada syndrome and atrial fibrillation in this patient could be connected by sodium channel dysfunction throughout the heart. In addition, we reviewed the possible connection between Brugada syndrome and atrial fibrillation.

Reportamos el caso de un hombre de 29 años de edad que se presentó inicialmente con un solo episodio de síncope. El electrocardiograma inicial (ECG) mostró fibrilación atrial y una elevación del segmento ST en la derivación V1. Una prueba de flecainida reveló la presencia del síndrome de Brugada. La patofisiología del síndrome de Brugada y la fibrilación atrial en este paciente podrían estar conectados por una disfunción del canal de sodio a través del corazón. Además, examinamos la posible conexión entre el síndrome de Brugada y la fibrilación atrial.