Familial cardiomyopathy underlies syndrome of right bundle branch block, ST segment elevation and sudden death

Corrado, Domenico; Nava, Andrea; Buja, Gianfranco; Martini, Bortolo; Fasoli, G; Oselladore, Luca; Turrini, Pietro; Thiene, Gaetano

doi:10.1016/0735-1097(95)00485-8

Cited by 218 publications

(103 citation statements)

References 20 publications

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“…Discrimination between Brugada syndrome and arrhythmogenic right ventricular cardiomyopathy (ARVC) may be particularly difficult because ARVC may at times mimic Brugada syndrome and structural abnormalities may only be found at time of autopsy. 24,25 Before the diagnosis Brugada syndrome is made, a serious attempt should be taken to exclude ARVC. Drug challenge with sodium channel blockers may be useful in discriminating between these 2 diseases.…”

Section: Differential Diagnosismentioning

confidence: 99%

“…Right or left bundle branch block, left ventricular hypertrophy 28 Acute myocardial ischemia or infarction 29 Acute myocarditis 30 Right ventricular ischemia or infarction 31 Dissecting aortic aneurysm 32 Acute pulmonary thromboemboli 33 Various central and autonomic nervous system abnormalities 34,35 Heterocyclic antidepressant overdose 36 Duchenne muscular dystrophy 37 Friedreich's ataxia 38 Thiamine deficiency 39,40 Hypercalcemia 41 Hyperkalemia 42 Cocaine intoxication 43,44 Mediastinal tumor compressing RVOT 45 Arrhythmogenic right ventricular dysplasia/cardiomyopathy 24,25 Long-QT syndrome, type 3 11,12 Other conditions that can lead to ST-segment elevation in the right precordial leads…”

Section: Table 2 Abnormalities That Can Lead To St-segment Elevationmentioning

confidence: 99%

See 1 more Smart Citation

Proposed Diagnostic Criteria for the Brugada Syndrome

Wilde

Antzelevitch

Borggrefe

et al. 2002

European Heart Journal

308

124

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Section: Differential Diagnosismentioning

confidence: 99%

Section: Table 2 Abnormalities That Can Lead To St-segment Elevationmentioning

confidence: 99%

Proposed Diagnostic Criteria for the Brugada Syndrome

Wilde

Antzelevitch

Borggrefe

et al. 2002

European Heart Journal

308

124

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“…10,11 Subsequently, a group of Italian researchers considered it as a form of arrhythmogenic right ventricular cardiomyopathy. 12 The identification of the first putative casual gene mutation in 1998 clarified the controversy confirming the genetic nature of the disease. 13 Over the past two decades a considerable number of studies, including reports of two consensus conferences, contributed to definition of the clinical characteristics, and of cellular and molecular features associated with the disease.…”

Section: Brugada Syndrome and Early Repolarisationmentioning

confidence: 99%

Brugada Syndrome and Early Repolarisation: Distinct Clinical Entities or Different Phenotypes of the Same Genetic Disease?

Conte

Caputo

Regoli

et al. 2016

Arrhythmia &Amp; Electrophysiology Review

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General understanding of early repolarisation (ER) has dramatically changed in the last decade. For several years, ER has been considered a benign electrocardiographic (ECG) finding with high prevalence in the general population. Recently different studies have challenged this view and showed a significant association with life-threatening arrhythmias. [1][2][3][4][5] In 2008 Haïssaguerre et al. first reported an increased prevalence of a particular pattern of ER on the resting 12-lead ECG of patients with history of idiopathic ventricular fibrillation (VF) (see Figure 1). 2 In these patients, ER was characterised by elevation of the QRS-ST segment junction of at least 0.1 mV above the baseline level, manifesting as QRS slurring (a smooth transition from the QRS complex to the ST segment) or notching (a positive J deflection of at least 1 mm inscribed on the S wave) in two adjacent inferior (II, III and aVF), lateral (I, aVL, and V4-V6), or infero-lateral leads. The ER pattern was observed in 31 % of patients with idiopathic VF and in 5 % of healthy control subjects.Moreover, patients with idiopathic VF and ER pattern presented a higher risk of experiencing an arrhythmic recurrence during a 5-year follow-up.2 This observation was confirmed by a case-control study showing that J-point elevation in the inferior and lateral leads is more frequent in patients with idiopathic VF than in matched control subjects (27 % versus 8 % in inferior leads; 13 % versus 1 % in lateral leads).3 Conversely, ER localised exclusively in V4 to V6 occurs with similar frequency among patients and healthy subjects.

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“…8,9 A plausible explanation for the initially conflicting interpretation of the etiopathogenesis of the Brugada syndrome has been in part provided by a clinicopathologic study that addressed prevalence, substrates, and clinical profile of young sudden death victims with the ECG pattern of right precordial ST-segment elevation. 17 Among a series of 273 young victims of cardiovascular sudden death who were prospectively studied from 1979 to 1998 in the Veneto Region of Italy, 12-lead ECG was available in 96 cases (36%). Right precordial ST-segment elevation was found in 14% of young sudden death victims with available ECG, and this ECG pattern characterized a subgroup of patients with an underlying RV cardiomyopathy who showed a propensity for SCD from nonexercise-related cardiac arrest and to exhibit dynamic ECG changes and polymorphic ventricular tachycardia, all clinical and ECG features typically observed in Brugada syndrome.…”

Section: Is the Brugada Syndrome A Cardiomyopathy? (D Corrado)mentioning

confidence: 99%

Diagnostic and genetic aspects of the Brugada and other inherited arrhythmias syndromes

Antzelevitch

Wilde

Eckardt

et al. 2007

Journal of Electrocardiology

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Doctor Wilde, presenting on behalf of himself and Dr Eckardt, discussed the role of invasive and noninvasive tests for risk stratification of Brugada syndrome. Doctor Hiraoka, presenting on behalf of Y. Yokoyama, M. Takagi, N. Aihara, K. Aonuma, and the Japan Idiopathic Ventricular Fibrillation Study Investigators, further discussed the diagnostic criteria for the Brugada syndrome. Doctor Antzelevitch examined the hypothesis that amplification of spatial dispersion of repolarization in the form of transmural dispersion of repolarization underlies the development of life-threatening ventricular arrhythmias associated with inherited ion channelopathies including the long QT, short QT, and Brugada syndromes. Doctor Corrado discussed the relationship between channelopathies and heart muscle diseases. KeywordsArrhythmia; Sudden cardiac death; Ventricular tachycardia; Fibrillation; Electrophysiology; Channelopathies Role of invasive and noninvasive tests for stratification (A. Wilde and L. Eckardt)Brugada syndrome is increasingly recognized as a disease entity associated with sudden cardiac death (SCD), most often, in relatively young individuals without structural heart disease. The typical ECG is characterized by right precordial ST-segment elevation and discrete prolongation of diverse conduction parameters. Three types of ST-segment elevation are recognized, but only one, type 1 (ie, the 'coved-type' ST segment), is considered to be diagnostic of Brugada syndrome. When a type 1 ECG is associated with documented (or inducible) ventricular arrhythmias, premature SCD or similar ECGs in family members, or nocturnal agonal respiration, Brugada syndrome is diagnosed. 1,2 When the ECG is absent at baseline, drug challenge (iv flecainide, ajmaline procainamide, pilsicainide, or other sodium channel blockers) can unmask the ECG features. A genetic diagnosis can identify 18% to 30% of patients with SCN5A mutations. It is not essential for definitive diagnosis of the syndrome.

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Familial cardiomyopathy underlies syndrome of right bundle branch block, ST segment elevation and sudden death

Abstract: An autosomal dominant familial cardiomyopathy, mainly involving the right ventricle and the conduction system, accounted for the ECG changes and the electrical instability of the syndrome.

Cited by 218 publications

References 20 publications

Proposed Diagnostic Criteria for the Brugada Syndrome

Proposed Diagnostic Criteria for the Brugada Syndrome

Brugada Syndrome and Early Repolarisation: Distinct Clinical Entities or Different Phenotypes of the Same Genetic Disease?

Diagnostic and genetic aspects of the Brugada and other inherited arrhythmias syndromes

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