Brugada syndrome: From cell to bedside
Section snippets
Clinical characteristics
The Brugada syndrome is characterized by ST segment elevation in the right precordial ECG leads and a high incidence of sudden death in patients with structurally normal hearts. The syndrome manifests primarily during adulthood, with a mean age of sudden death of approximately 40 years. The youngest patient diagnosed with the syndrome was 2 days of age, and the oldest was 84. The syndrome is thought to be responsible for 4–12% of all sudden deaths and at least 20% of deaths in patients with
Relationship with structural heart disease
A subpopulation of arrhythmogenic right ventricular cardiomyopathy (ARVC) patients have been found to display an ST-segment elevation and polymorphic VT characteristic of the Brugada syndrome.25 In addition one case has been reported in which a patient with a Brugada phenotype required heart transplantation due to untreatable arrhythmias26 and in whom severe fibrosis of the right ventricle was subsequently reported.
These facts notwithstanding, the vast majority of Brugada patients possess a
Factors that precipitate and modulate the ECG and arrhythmic manifestations of the Brugada syndrome
ST segment elevation in the Brugada syndrome can be very dynamic. The Brugada ECG is often concealed, but may be unmasked or modulated by sodium channel blockers, a febrile state, vagotonic agents, α-adrenergic agonists, β-adrenergic blockers, tricyclic or tetracyclic antidepressants, first-generation antihistaminics (dimenhydrinate), a combination of glucose and insulin, hyperkalemia, hypokalemia, hypercalcemia, and alcohol and cocaine toxicity (Fig 16).6, 7, 8, 83, 84, 85, 86, 87, 88, 89
Therapy of congenital Brugada syndrome
Despite impressive strides in the identification and characterization of Brugada syndrome over the past decade, progress relative to therapy has been less noteworthy. The various device and pharmacologic therapies tested clinically or suggested based on experimental evidence are listed in Table 7. Currently, an implantable cardioverter defibrillator (ICD) is the only proven effective treatment for the disease.99, 100 In a multicenter trial of 690 patients with Brugada syndrome, in which 258
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Cited by (104)
Telemedicine for detecting Brugada Syndrome in eastern Indonesia: A multi-center prospective observational study
2021, Annals of Medicine and SurgeryCitation Excerpt :This may be related to the clinical phenotype that was found more frequent in male subjects. Moreover, different pattern of outward wave is more prominent in the male subject, which results in a more negative end of phase I in the right ventricular epicardium that increase the risk of ventricular tachycardia [31]. Previous studies also reported that BrS patients with conduction abnormalities such as longer PR interval, longer QRS duration, and longer QT interval on surface ECG are more likely to develop syncope [32].
Meta-Analysis of Clinical Outcome After Implantable Cardioverter-Defibrillator Implantation in Patients With Brugada Syndrome
2019, JACC: Clinical ElectrophysiologyAgeing, the autonomic nervous system and arrhythmia: From brain to heart
2018, Ageing Research ReviewsCitation Excerpt :It is characterised by a triad of ST-segment elevation in the right precordial leads V1–V3, prolonged conduction intervals and right bundle branch block. It is implicated in 4–12% of sudden cardiac deaths (Antzelevitch et al., 2005; Jeevaratnam et al., 2016). Typically, arrhythmias in BrS occur in the third or fourth decade in life (Postema et al., 2013) under conditions of increased vagal tone (Nakazawa et al., 2003).
Lifestyle impact and genotype-phenotype correlations in brugada syndrome
2018, Lifestyle in Heart Health and DiseaseEvaluation of baseline ECG in patients undergoing Oral Flecainide Challenge test for suspected Brugada Syndrome: An analysis of lead II
2017, Indian Pacing and Electrophysiology Journal
Supported by Grants HL47678 and HL066169 from NHLBI (C.A., R.B.), American Heart Association (R.B., C.A.), and NYS and Florida Grand Lodges F.&A.M.