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Treatment of Electrical Storm with Amiodarone in Brugada Syndrome- an Unexpected Protective Effect
No full textWe are reporting on a 53 year old man with proven Brugada syndrome and ICD implantation for resuscitation in context of polymorphic VT. After recurrent arrhythmia he was treated with Amiodarone. This showed to have a protective effect despite various reports suggesting avoiding Amiodarone in Brugada syndrome
Featuring: Josep Brugada
Get PDFIn the Cardiology Masters section of European Cardiology Review, we bring you an insight into the career of a key contributor to the field of cardiology. In this edition, we feature Josep Brugada
Proposed diagnostic criteria for the Brugada syndrome - Consensus report
No full textAsyndrome characterized by ST-segment elevation in right precordial leads (V1 to V3) that is unrelated to ischemia, electrolyte disturbances, or obvious structural heart disease was reported as early as 1953,1 but was first described as a distinct clinical entity associated with a high risk of sudden cardiac death in 1992.The Brugada syndrome is a familial disease that displays an autosomal dominant mode of transmission, with incomplete penetrance and an incidence ranging between 5 and 66 per 10 000. In regions of Southeast Asia where it is endemic, the clinical presentation of Brugada syndrome is distinguished by a male predominance (8:1 ratio of male:female) and the appearance of arrhythmic events at an average age of 40 years (range: 1 to 77 years).Although a number of candidate genes are considered plausible, thus far the syndrome has been linked only to mutations in SCN5A, the gene encoding for the α subunit of the sodium channel
Brugada syndrome - Report of the second consensus conference
No full textSince its introduction as a clinical entity in 1992, the Brugada syndrome has progressed from being a rare disease to one that is second only to automobile accidents as a cause of death among young adults in some countries. Electrocardiographically characterized by a distinct ST-segment elevation in the right precordial leads, the syndrome is associated with a high risk for sudden cardiac death in young and otherwise healthy adults, and less frequently in infants and children. Patients with a spontaneously appearing Brugada ECG have a high risk for sudden arrhythmic death secondary to ventricular tachycardia/fibrillation. The ECG manifestations of Brugada syndrome are often dynamic or concealed and may be unmasked or modulated by sodium channel blockers, a febrile state, vagotonic agents, alpha-adrenergic agonists, beta-adrenergic blockers, tricyclic or tetracyclic antidepressants, a combination of glucose and insulin, hypo- and hyperkalemia, hypercalcemia, and alcohol and cocaine toxicity. In recent years, an exponential rise in the number of reported cases and a striking proliferation of articles defining the clinical, genetic, cellular, ionic, and molecular aspects of the disease have occurred. The report of the first consensus conference, published in 2002, focused on diagnostic criteria. The present report, which emanated from the second consensus conference held in September 2003, elaborates further on the diagnostic criteria and examines risk stratification schemes and device and pharmacological approaches to therapy on the basis of the available clinical and basic science data
Genètica i caos elèctric : el canal de sodi en la Síndrome de Brugada
Get PDFTítol obtingut de la portada digitalitzadaaConsultable des del TDXIn this work we have investigated the Brugada syndrome caused by mutations in the sodium channel. We have performed clinical and genetic investigations to identify the genetic carriers. We have also investigated how the genetic background and the external modifiers modulate the disease phenotype. We have been able to prove that in Brugada syndrome the genetic testing is very valuable for two main reasons: first, because in a family with an identified mutation we are able to identify the non-carriers, and therefore those that are free of the disease. Secondly genetic testing allows the adoption of preventive measures in asymptomatic carriers, like the rapid treatment of fever or the avoidance of drugs that may block the sodium channel. Only for those two reasons it is justifiable the genetic analysis. Finally we have shown that neither the type of mutation, nor its localization in the protein allows us to estimate the risk of sudden death. In the asymptomatic individual with a normal ECG, the presence of a mutation is only indicative of a risk of developing the disease, but it is not a given that he will suffer from it. Despite the increase in genetic testing, at present its practice does not seem justified if only to define the therapeutic aggressiveness. The asymptomatic individuals who develop symptoms at follow-up present always clear clinical alterations, like a prolongation of the QT interval or elevation of the ST segment. These ecg parameters already define the need to perform additional tests or the adoption of preventive measures. In conclusion, being only a genetic carrier does not justify a more aggressive treatment at present. The treatment has to be determined by the result of the clinical tests
Proposed diagnostic criteria for the Brugada syndrome: consensus report
No full textWhat are the proper diagnostic criteria for identifying Brugada syndrome? A definitive answer to this question has been out of reach and is the reason for the establishment of a special task force of Arrhythmia Working Group of the European Society of Cardiology that met from August 31 to September 1, 2000. This report is the consensus statement from that meeting. The diagnostic criteria described herein are based on the currently available clinical data and state-of-the-art understanding of the molecular and cellular mechanisms underlying Brugada syndrome
Proposed diagnostic criteria for the Brugada syndrome
Get PDFWlat are the proper diagnostic cliteria to be used in
identifying Brugada syndrome? A definitive answer to
this question has been out ofreach and is the reason for
the establishment of a special Arrhythrn:a Working
Group of the European Society of Cardiology that met
on 31 August-l September 2000. This report is the
consensus document emanating from that meeting. The
diagnostic criteria described hereiu are based on the
currentiy available ciinical data and state-of-the-art
understanding of the molecuiar and cellular mechanisms
underlying Brugada syndrome. The proposed criteria
must be considered a work-in-progress that will be
fine-tuned as confirmatoqr data from future molecular
studies and prospective trials become available
Simultaneous ST-segment elevation in the right precordial and inferior leads in Brugada syndrome
No full textWe report the case of a patient with genetically confirmed Brugada syndrome who presented with ST-segment elevation in the right precordial and inferior leads. The presenting arrhythmia was atrial fibrillation, which degenerated into ventricular fibrillation during intravenous amiodarone. A flecainide test was markedly positive. Four appropriate cardioverter-defibrillator discharges occurred during a two-year follow-up period after implantation of the device
Role of pharmacotherapy in Brugada syndrome
No full textIn patients who undergo aborted sudden cardiac death or syncope of unknown origin (symptomatic Brugada syndrome), no one argues that the implantation of an ICD is the first-line therapy regardless of the findings of the EP study. For those patients, drug therapy plays not a contradictory but a complimentary role to the ICD by reducing the number of ICD shock deliveries. Prevention of VF contributes to the improvement in the quality of life of the patients by avoiding uncomfortable ICD shock deliveries.
As mentioned above, Belhassen et al. 11, performed EP-guided medical therapy in 34 patients with idiopathic VF, in 5 of whom the criterion of Brugada syndrome were fulfilled, and reported excellent long-term results. Although medical therapy requires markers that can accurately predict the preventive effect of VF over a long-term period, there, however, seems to be no reliable marker. An EP study is usually used to examine the preventive effects of antiarrhythmic agents on sustained ventricular tachycardia in patients with structural heart disease, whereas the prognostic value of the EP study in predicting life-threatening events in Brugada syndrome is still controversial. Brugada et al., 3 suggested that among the asymptomatic patients, the inducibility of VT during the EP study might be a prognostic marker of risk. Studies by Priori et al., 22 and Kanda et al., 23 failed to find an association between the inducibility and recurrence of VT/VF in patients with Brugada syndrome regardless of whether it was symptomatic or asymptomatic. As Belhassen suggested 13, the difference might be due to the VF induction protocol in the EP study, in which Belhassen et al., used a stimulus current intensity of five times the diastolic threshold along with the use of repetition of double and triple extrastimulation at the shortest coupling intervals that resulted in ventricular capture. Further appropriate clinical trials are needed to clarify this issue.
In patients with asymptomatic Brugada syndrome who are family members of symptomatic Brugada syndrome patients, the same strategy as that for the symptomatic Brugada syndrome patients should be considered 2,3,4. In another asymptomatic-patient group in whom an ECG that discloses the Brugada sign is performed for routine reasons such as a workup prior to surgery or sport license or screening for insurance, risk stratification to find the patients at high risk is needed because in most cases these patients have a benign prognosis3,22,23. Antzelevitch et al. 4, recommended that all asymptomatic patients with the Brugada sign should undergo an EP study for risk stratification, and, if inducible, an ICD should be implanted since Brugada et al.3, reported that an overall 8% life-threatening event rate was found in initially asymptomatic patients. Belhassen et al.,13 suggested that EP study-guided quinidine therapy might become an alternative to ICD therapy for prophylaxis of arrhythmic events in these patients. Further appropriate clinical trials are needed
Brugada-type Electrocardiographic Pattern Induced by Fever
No full textST-segment elevation in Brugada syndrome is caused by a shift in the ionic current balance and the creation of a voltage gradient between the epicardium and the endocardium. This ionic mechanism have been shown to be temperature dependent. We describe a 33-year-old man who presented with fever with the dynamic electrocardiographic changes similar to the Brugada syndrome. These electrocardiographic anomalies disappeared when the temperature returned to normal
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