Proposed Diagnostic Criteria for the Brugada Syndrome

Wilde, Arthur A.M.; Antzelevitch, Charles; Borggrefe, Martin; Brugada, Josép; Brugada, Ramón; Brugada, Pedro; Corrado, Domenico; Hauer, R.N.W.; Kass, Robert S.; Nademanee, Koonlawee; Priori, Silvia G.; Towbin, Jeffrey A.

doi:10.1053/euhj.2002.3382

Cited by 308 publications

(139 citation statements)

References 43 publications

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“…Continuous cardiac monitoring is advised owing to the pro-arrhythmic effects of these drugs. Ajmaline is preferred, if available, as it is inactivated within a few minutes; the half-life of flecainide is 20 h, while the sensitivity of procainamide in the diagnosis of Brugada syndrome has been disputed [44, 55]. The protocol for drug administration and interpretation of ECG changes is described in the recent consensus report on Brugada syndrome [44].…”

Section: Supplementary Investigationsmentioning

confidence: 99%

Sudden Cardiac Death in the Young: A Strategy for Prevention by Targeted Evaluation

Sen‐Chowdhry

McKenna

2006

Cardiology

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The annual incidence of sudden cardiac death (SCD) in the general population is estimated as 1 in a 1,000. Since survival rates from out-of-hospital cardiac arrests are poor, primary prevention is key to reducing the burden of SCD in the community. Prominent causes of SCD include ischaemic heart disease, anomalous coronary arteries, and the primary myocardial diseases: hypertrophic cardiomyopathy, dilated cardiomyopathy, and ar rhythmogenic right ventricular cardiomyopathy (ARVC). In 4% of sudden deaths in the 16–64 age group, post-mortem examination fails to identify a cause, yielding a default diagnosis of sudden arrhythmic death syndrome (SADS). The inherited arrhythmia syndromes (long QT, short QT, and Brugada syndromes, and familial catecholaminergic polymorphic ventricular tachycardia) may be implicated in SADS, owing to their propensity for producing ventricular tachyarrhythmia in the structurally normal heart. Monogenic disorders therefore predominate as causes of SCD in the young. The advent of effective therapies for these diseases, particularly implantable cardioverter defibrillators, has prompted calls for universal screening to enable timely diagnosis of occult cardiac disease. Since prospective cardiac assessment of the general population is not feasible, the solution may be to target high-risk subgroups, namely, patients with cardiac symptoms, relatives of SCD victims, and competitive athletes. The recommended preliminary work-up includes a 12-lead ECG, signal-averaged ECG, transthoracic echocardiogram, exercise test, and ambulatory ECG monitoring. Cardiovascular magnetic resonance is a useful adjunct in patients with suspected ARVC or anomalous coronary arteries. Provocative challenge with a sodium challenge blocker may be of value in unmasking the Brugada syndrome. Identification of disease-causing mutations in affected individuals facilitates cascade screening of families.

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Section: Supplementary Investigationsmentioning

confidence: 99%

Sudden Cardiac Death in the Young: A Strategy for Prevention by Targeted Evaluation

Sen‐Chowdhry

McKenna

2006

Cardiology

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“…Other cardiac channelopathies, such as short-QT [10,12] and Brugada syndrome [2,46], might be studied in a similar fashion using patient-specific iPSC and patch clamp electrophysiology. Catecholaminergic polymorphic ventricular tachycardia (CPVT; recently reviewed in [30]), another disease characterized by the occurrence of ventricular arrhythmias in patients with structurally normal hearts, has already been modeled using iPSC technology [9,15,27].…”

Section: Future Targets Of Cardiovascular Research Based On Patient-smentioning

confidence: 99%

Recapitulating Long-QT Syndrome Using Induced Pluripotent Stem Cell Technology

et al. 2012

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The generation of patient-specific stem cells by reprogramming somatic cells to induced pluripotent stem cells (iPSC) provides the basis for a promising new type of in vitro disease models. Patient-specific iPSC derived from individuals with hereditary disorders can be differentiated into somatic cells in vitro, thus allowing the pathophysiology of the diseases to be studied on a cellular level. Different types of long-QT syndrome have been successfully modeled using this approach, demonstrating that the iPSC-derived patient-specific cardiomyocytes recapitulated key features of the disease in vitro. This approach will likely serve to model other monogenetic or polygenetic cardiovascular disorders in the future. Moreover, test platforms based on patient-specific iPSC could be used to test the potential of drug candidates to induce QT-interval prolongation or other unwanted side effects, screen for novel cardiovascular drugs, or to tailor medical therapy to the specific needs of a single patient.

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“…Nur selten kommt es zu Synkopen, plötzliche Herztodesfälle wurden nur ganz vereinzelt berichtet. [20]. Wegweisender Befund ist die rechtspräkordiale Repolarisationsstörung mit schulter-oder sattelförmiger ST-Elevation mit negativer oder biphasischer TWelle (.…”

Section: Therapie Und Prognoseunclassified

“…), vagotone Stimulation oder Fieber demaskieren oder aggravieren. Neben dem Nachweis des typischen EKG ist zur Diagnosestellung vor allem der Ausschluss von Elektrolytstörungen, extrakardialen Ursachen und strukturellen Herzerkrankungen als Grundlage von ventrikulä-ren Tachyarrhythmien und Synkopen erforderlich [20].…”

Section: Therapie Und Prognoseunclassified

Arrhythmien aus dem rechten Ventrikel

et al. 2004

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Right ventricular arrhythmias predominantly occur in young patients with rare cardiac diseases. Underlying cardiac conditions include idiopathic right ventricular outflow-tract tachycardia (RVOT-VT), arrhythmogenic right ventricular cardiomyopathy (ARVC), Brugada syndrome, and postoperative congenital heart disease (i.e. tetralogy of Fallot). According to the underlying cardiac disease, there are significant differences in the diagnostic and therapeutic management and prognosis which is mainly determined by life-threatening ventricular arrhythmia recurrences and sudden cardiac death. To provide optimal treatment for affected patients, a detailed diagnostic evaluation and risk stratification is mandatory. Tailored treatment strategies aim at the suppression or effective termination of recurrent ventricular tachyarrhythmias and prevention of sudden death by antiarrhythmic drug therapy, catheter ablation, and the implantation of cardioverter-defibrillators. This review summarizes the current knowledge on pathogenesis, diagnosis, treatment and prognosis of those conditions that are associated with arrhythmias originating from the right ventricle.

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Proposed Diagnostic Criteria for the Brugada Syndrome

Cited by 308 publications

References 43 publications

Sudden Cardiac Death in the Young: A Strategy for Prevention by Targeted Evaluation

Sudden Cardiac Death in the Young: A Strategy for Prevention by Targeted Evaluation

Recapitulating Long-QT Syndrome Using Induced Pluripotent Stem Cell Technology

Arrhythmien aus dem rechten Ventrikel

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