Diagnosis and long-term follow-up of the Brugada syndrome in patients with idiopathic ventricular fibrillation

Remme, CA

doi:10.1053/euhj.2000.2366

Cited by 100 publications

(59 citation statements)

References 14 publications

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“…10 This consensus is supported by our observation that a previous cardiac arrest is a strong indicator of adverse outcome, yet treatment of most asymptomatic individuals with a Brugada ECG or patients with syncope remains less clear. Following the recommendations of Brugada et al, 2 implantation of a defibrillator in patients with a previous syncope and a type 1 ECG "seems to be as mandatory as it is in patients with resuscitated sudden arrhythmic death."…”

Section: Treatment Strategy In Individuals With a Brugada Ecgsupporting

confidence: 58%

Long-Term Prognosis of Individuals With Right Precordial ST-Segment–Elevation Brugada Syndrome

et al. 2005

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Background-Brugada syndrome is an arrhythmogenic disease characterized by an ECG pattern of ST-segment elevation in the right precordial leads and an increased risk of sudden cardiac death as a result of ventricular fibrillation. Controversy exists with regard to risk stratification and therapeutic management, particularly in asymptomatic individuals. Methods and Results-A total of 212 individuals (mean age, 45Ϯ6 years) with a type 1 Brugada ECG pattern were studied.Of these, 123 (58%) were asymptomatic, 65 (31%) had Ն1 syncope of unknown origin, and 24 (11%) had to be resuscitated because of ventricular fibrillation. In 125 individuals (59%), a spontaneous type 1 ECG was recorded. In the remaining, drug challenge with a class I antiarrhythmic agent unmasked a Brugada ECG. The mean ST elevation was 2.3Ϯ1.2 mm in symptomatic patients and 1.9Ϯ1.5 mm in asymptomatic individuals (Pϭ0.04). During a mean follow-up of 40Ϯ50 months, 4 of the 24 patients (17%) with aborted sudden cardiac death and 4 of 65 (6%) with a prior syncope had a recurrent arrhythmic event, whereas only 1 of 123 asymptomatic individuals (0.8%) had a first arrhythmic event. Four of 9 patients with arrhythmic events during follow-up were not inducible during programmed electrical stimulation. A previous history of aborted sudden death or syncope and the presence of a spontaneous type 1 ECG were predictors of adverse outcome. Conclusions-The present study reports data on a large population of individuals with a type 1 Brugada ECG pattern with the longest follow-up reported so far. A very low incidence of severe arrhythmic events, particularly in asymptomatic individuals, was found during follow-up. In the presence of very few arrhythmic events on follow-up, programmed electrical stimulation showed very little accuracy in predicting outcome. (Circulation. 2005;111:257-263.)

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Section: Treatment Strategy In Individuals With a Brugada Ecgsupporting

confidence: 58%

Long-Term Prognosis of Individuals With Right Precordial ST-Segment–Elevation Brugada Syndrome

et al. 2005

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“…The electrocardiographic signature of the syndrome is dynamic and often concealed, but can be unmasked by potent sodium channel blockers such as flecainide, ajmaline, and procainamide, 7 although the specificity of this effect for uncovering patients at risk for sudden death has been an issue of concern. A recent report by Remme et al 8 has shown that the number of idiopathic ventricular fibrillation patients diagnosed as having Brugada syndrome is a sensitive function of the diagnostic criteria applied. What are the proper diagnostic criteria for identifying Brugada syndrome?…”

mentioning

confidence: 99%

Proposed Diagnostic Criteria for the Brugada Syndrome

Wilde

Antzelevitch

Borggrefe

et al. 2002

European Heart Journal

308

124

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“…This condition, known as Brugada syndrome, accounts for Ϸ20% to 30% of cases previously diagnosed as idiopathic ventricular fibrillation. 2 The pathophysiology of this syndrome remains poorly understood. In 1998, Chen et al 3 discovered the first genetic defect, a mutation in the cardiac sodium channel gene SCN5A.…”

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confidence: 99%

Abnormal Myocardial Presynaptic Norepinephrine Recycling in Patients With Brugada Syndrome

et al. 2004

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Background— Life-threatening ventricular tachyarrhythmias can occur in young patients without structural heart disease (idiopathic forms). In many patients, these are typically triggered by an increased sympathetic tone, eg, by physical or mental stress. In contrast, in Brugada syndrome, ventricular tachyarrhythmias more often occur during rest or sleep when the vagal tone is predominant. Furthermore, adrenergic agonists can reduce the level of ST-segment elevation, whereas it is increased by parasympathetic agonists or adrenergic antagonists. The aim of this study was to investigate presynaptic and postsynaptic myocardial sympathetic function in patients with Brugada syndrome. Methods and Results— Nine patients with Brugada syndrome (6 male, 3 female; age, 41±13 years) were enrolled in this study. The cardiac autonomic nervous system was assessed noninvasively, quantifying myocardial presynaptic and postsynaptic sympathetic function by means of positron emission tomography with the norepinephrine analogue 11 C-Hydroxyephedrine ( 11 C-HED) and the nonselective β-blocker 11 C-CGP 12177 ( 11 C-CGP). Presynaptic sympathetic norepinephrine recycling, assessed by 11 C-HED, was globally increased in patients with Brugada syndrome compared with a group of age-matched healthy control subjects (92.9±16.2 mL/g versus 69.1±14.2 mL/g; P <0.05), whereas postsynaptic β-adrenoceptor density, assessed by 11 C-CGP, was similar in patients and control subjects (10.4±6.7 pmol/g versus 10.2±2.9 pmol/g; P =NS). Conclusions— The present study on autonomic innervation in Brugada syndrome describes an enhanced presynaptic norepinephrine recycling with preserved β-adrenoceptor density, further supporting the hypothesis of an autonomic dysfunction in Brugada syndrome. This is a further step toward the understanding of the pathophysiology of the disease with potential future impact on therapeutic strategies.

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Diagnosis and long-term follow-up of the Brugada syndrome in patients with idiopathic ventricular fibrillation

Cited by 100 publications

References 14 publications

Long-Term Prognosis of Individuals With Right Precordial ST-Segment–Elevation Brugada Syndrome

Long-Term Prognosis of Individuals With Right Precordial ST-Segment–Elevation Brugada Syndrome

Proposed Diagnostic Criteria for the Brugada Syndrome

Abnormal Myocardial Presynaptic Norepinephrine Recycling in Patients With Brugada Syndrome

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