RESUMO
BACKGROUND: Brugada syndrome (BrS) is somewhat a challenging diagnosis, due to its dynamic pattern. One of the aspects of this disease is a significant conduction disorder located in the right ventricular outflow tract (RVOT), which can be explained as a consequence of low expression of Connexin-43. This decreased conduction speed is responsible for the typical electrocardiographic pattern. Opposite leads located preferably in inferior leads of the electrocardiogram may show a deep and widened S wave associated with ascending ST segment depression. Holter monitoring electrocardiographic (ECG) aspects is still a new frontier of knowledge in BrS, especially in intermittent clinical presentations. METHODS: We describe, as an exploratory analysis, five case series of intermittent type 1 BrS to demonstrate the appearance of ascending ST segment depression and widening of the S wave, during 3-channel 24h-Holter monitoring (C1, C2 and C3) with bipolar leads. RESULTS: In the five cases described, the ST segment depression was observed mainly in C2, but in some cases also in C1 and C3. Only case 1 presented concomitant intermittent elevation of the ST segment in C1. All cases were intermittent. CONCLUSION: The recognition of an ECG pattern with ascending ST-segment depression and widening of the S wave in 3-channel Holter described in this case series should raise a suspicion of the BrS and suggests the counterpart of a dromotropic disturbance registered in the RVOT and/or reciprocal changes.
Assuntos
Síndrome de Brugada , Arritmias Cardíacas , Depressão , Eletrocardiografia , Eletrocardiografia Ambulatorial , HumanosRESUMO
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.
Assuntos
Fenótipo , Fibrilação Atrial , Morte Súbita Cardíaca , Doença das Coronárias , Hereditariedade , Eletrólitos , Síndrome de Brugada , Exoma , Genótipo , Coração , CardiopatiasRESUMO
Conduction delay in the right ventricular outflow tract as manifested in the electrocardiogram constitutes a high-risk predictor of ventricular arrhythmias in patients with Brugada syndrome. We present a case with a right QRS axis between -90° and ±180°. This feature has never been reported in the context of Brugada syndrome. (Level of Difficulty: Advanced.).
RESUMO
In patients experiencing an ST-elevation myocardial infarction (STEMI), rapid diagnosis and immediate access to reperfusion therapy leads to optimal clinical outcomes. The rate-limiting step in STEMI diagnosis is the availability and performance of a 12-lead ECG. Recent technology has provided access to a reliable means of obtaining an ECG reading through a smartphone application (app) that works with an attachment providing all 12-leads of a standard ECG system. The ST LEUIS study was designed to validate the smartphone ECG app and its ability to accurately assess the presence or absence of STEMI in patients presenting with chest pain compared with the gold standard 12-lead ECG. We aimed to support the diagnostic utility of smartphone technology to provide a timely diagnosis and treatment of STEMI. The study will take place over 12months at five institutions. Approximately 60 patients will be enrolled per institution, for a total recruitment of 300 patients.
Assuntos
Eletrocardiografia , Aplicativos Móveis , Projetos de Pesquisa , Infarto do Miocárdio com Supradesnível do Segmento ST/diagnóstico , Smartphone , Adulto , Idoso , Dor no Peito/diagnóstico , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos Prospectivos , Sensibilidade e EspecificidadeRESUMO
The electrocardiogram is an important tool for the initial diagnostic suspicion of hypertrophic cardiomyopathy in any of its forms, both in symptomatic and in asymptomatic patients because it is altered in more than 90 percent of the cases. Electrocardiographic anomalies are more common in patients carriers of manifest hypertrophic cardiomyopathy and the electrocardiogram alterations are earlier and more sensitive than the increase in left ventricular wall thickness detected by the echocardiogram. Nevertheless, despite being the leading cause of sudden death among young competitive athletes there is no consensus over the need to include the method in the pre-participation screening. In apical hypertrophic cardiomyopathy the electrocardiographic hallmarks are the giant negative T waves in anterior precordial leads. In the vectorcardiogram, the QRS loop is located predominantly in the left anterior quadrant and T loop in the opposite right posterior quadrant, which justifies the deeply negative T waves recorded. The method allows estimating the left ventricular mass because it relates to the maximal spatial vector voltage of the left ventricle in the QRS loop. The recording on electrocardiogram or Holter monitoring of nonsustained monomorphic ventricular tachycardia in patients with syncope, recurrent syncope in young patient, hypotension induced by strain, bradyarrhythmia, or concealed conduction are markers of poor prognosis. The presence of rare sustained ventricular tachycardia is observed in mid-septal obstructive HCM with apical aneurysm. The presence of complete right bundle branch block pattern is frequent after the percutaneous treatment and complete left bundle branch block is the rule after myectomy.
Assuntos
Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/epidemiologia , Vetorcardiografia/métodos , Adolescente , Adulto , Distribuição por Idade , Idoso , Criança , Pré-Escolar , Eletrocardiografia/métodos , Feminino , Humanos , Lactente , Masculino , Prevalência , Reprodutibilidade dos Testes , Medição de Risco , Sensibilidade e Especificidade , Índice de Gravidade de Doença , Adulto JovemRESUMO
Congenital or familial short QT syndrome is a genetically heterogeneous cardiac channelopathy without structural heart disease that has a dominant autosomal or sporadic pattern of transmission affecting the electric system of the heart. Patients present clinically with a spectrum of signs and symptoms including irregular palpitations due to episodes of paroxysmal atrialfibrillation, dizziness and fainting (syncope) and/or sudden cardiac death due to polymorphic ventricular tachycardia and ventricular fibrillation. Electrocardiographic (ECG) findings include extremely short QTc intervals (QTc interval ≤330 ms) not significantly modified with heart rate changes and T waves of great voltage witha narrow base. Electrophysiologic studies are characterized by significant shortening of atrial and ventricular refractory periods and arrhythmias induced by programmed stimulation. A few families have been identified with specific genotypes: 3 with mutations in potassium channels called SQT1 (Iks), SQT2 (Ikr) and SQT3 (Ik1). These 3 potassium channel variants are the "genetic mirror image" of long QT syndrome type 2, type 1 and Andersen-Tawil syndrome respectively because they exert opposite gain-of-function effects on the potassium channels in contrast to the loss-of-function of the potassium channels in the long QT syndromes. Three new variants with overlapping phenotypes affecting the slow inward calcium channels havealso been described. Finally, another variant with mixed phenotype affecting the sodium channel was reported. This review focuses the landmarks of this newest arrhythmogenic cardiac channelopathy on the main clinical, genetic, and proposed ECG mechanisms. In addition therapeutic options and the molecular autopsy of this fascinating primary electrical heart disease are discussed.
Assuntos
Canalopatias , Potenciais de Ação , Animais , Arritmias Cardíacas/congênito , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/metabolismo , Arritmias Cardíacas/mortalidade , Arritmias Cardíacas/fisiopatologia , Arritmias Cardíacas/terapia , Canais de Cálcio/genética , Canais de Cálcio/metabolismo , Canalopatias/congênito , Canalopatias/diagnóstico , Canalopatias/metabolismo , Canalopatias/mortalidade , Canalopatias/fisiopatologia , Canalopatias/terapia , Eletrocardiografia , Técnicas Eletrofisiológicas Cardíacas , Predisposição Genética para Doença , Sistema de Condução Cardíaco/metabolismo , Sistema de Condução Cardíaco/fisiopatologia , Humanos , Mutação , Fenótipo , Canais de Potássio/genética , Canais de Potássio/metabolismo , Valor Preditivo dos TestesRESUMO
In the great majority of cases the ECG pattern of early repolarization (ERP) is a benign phenomenon observed predominantly in teenagers, young adults, male athletes and the black race. The universally accepted criterion for its diagnosis is the presence, in at least two adjoining leads, of ≥ 1 mm or 0.1 mV ST segment elevation. In benign ERP reciprocal ST segment changes are possible only in lead aVR. In contrast, reciprocal ST segment changes can be observed in several leads in idiopathic ventricular fibrillation (IVF) and acute coronary syndrome. In benign ERP the ST segment and T wave patterns have a relative temporal stability. IVF is an entity with low prevalence, possibly familiar, and characterized by the occurrence of VF events in a young person. More frequently this occurs in male subjects without structural heart disease and with otherwise with normal ECG even using high right accessory leads and/or after ajmaline injection. Several clinical entities cause ST segment elevation include asthenic habitus, acute pericarditis, ST segment elevation myocardial infarction, Brugada syndrome, congenital short QT syndrome, and idiopathic VF. In these circumstances clinical and ECG data are most important for differential diagnosis. In IVF the modifications could be dramatic and predominantly at night during vagotonic predominance when J waves > 2 mm in amplitude. The ST/T abnormalities are dynamic, inconstant, and reversed with isoproterenol. Convex upward J waves, with horizontal/descending ST segments or "lambda-wave" ST shape are suggestive of IVF with early repolarization abnormalities. Premature ventricular contractions with very short coupling and "R on T" phenomenon are characteristics with two pattern: When originate from right ventricular outflow tract left bundle branch block morphology and from peripheral Purkinje network, left bundle branch block pattern. The inherited-familial forms are not frequent in IVF; however mutations were identified in the genes KCNJ8, DPP6, SCN5A, SCN3B, CACNA1C, CACNB2, and CACNA2D1. The management of IVF has class I indication for ICD implantation. Ablation therapy is considered additional to ICD implantation in those patients with repetitive ventricular arrhythmia. Quinidine is a highly efficient drug that prevents recurrence.
Assuntos
Arritmias Cardíacas/diagnóstico , Eletrocardiografia , Sistema de Condução Cardíaco/fisiopatologia , Fibrilação Ventricular/diagnóstico , Potenciais de Ação , Adulto , Arritmias Cardíacas/genética , Arritmias Cardíacas/fisiopatologia , Arritmias Cardíacas/terapia , Diagnóstico Diferencial , Feminino , Predisposição Genética para Doença , Humanos , Masculino , Valor Preditivo dos Testes , Prognóstico , Medição de Risco , Fatores de Risco , Fatores de Tempo , Fibrilação Ventricular/genética , Fibrilação Ventricular/fisiopatologia , Fibrilação Ventricular/terapia , Adulto JovemRESUMO
AIMS: Previous studies have reported right bundle branch block in Brugada syndrome. Subsequent analysis of electrocardiograms (ECGs) found one-third of cases classified as right bundle branch block did not meet criteria of a wide final S wave in the left leads. We aimed to study the role of the vectorcardiogram to characterize Brugada type 1 ECG pattern. METHODS AND RESULTS: Compare Frank-method vectorcardiogram in 11 patients with Brugada type 1 ECG pattern (BrS group) with vectorcardiogram of 20 healthy individuals with ECGs depicting incomplete right bundle branch block (IRBBB group) and 12 patients with complete right bundle branch block (CRBBB group). Initial 10-20 ms vector of the QRS loop in the horizontal plane (HP): BrS and IRBBB groups: Vector heading anterior and leftward. CRBBB group: Vector directed anterior and rightward. Right end conduction delay of the QRS loop: BrS group: Upper right quadrant of the frontal plane, right posterior quadrant of the HP. IRBBB group: Upper right quadrant of the frontal plane (30%) and right anterior quadrant of the HP (90%). CRBBB group: Upper right quadrant on the frontal plane (30%); all cases in the right anterior quadrant of the HP. 0 point (onset of QRS loop) and J point (end of QRS loop) relationship: BrS group: Not coincidental. IRBBB and CRBBB groups: Coincidental. T loop morphology, size, and appearance: BrS group: Circular, with symmetrical afferent and efferent limbs in 10 cases (90%). IRBBB and CRBBB groups: Elliptical or linear with slow inscription of efferent limb and rapid inscription of afferent limb. CONCLUSIONS: Vectorcardiograms in patients with Brugada type 1 ECG pattern have distinctive characteristics compared with healthy individuals with incomplete and CRBBB. These differences relate to the spatial location of the end conduction delay (right superior and posterior quadrant in the BrS group) and the morphology, size, and velocity of inscription of afferent and efferent limbs of the T loop (circular, small, of symmetrical limbs) and with a 1:1 length/width ratio.