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Brugada-Syndrom

Brugada syndrome

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Herzschrittmachertherapie + Elektrophysiologie Aims and scope Submit manuscript

Zusammenfassung

Das Brugada-Syndrom ist eine Ionenkanalerkrankung, die mit einem erhöhten Risiko eines plötzlichen Herztodes einhergeht. Wahrscheinlich liegt hier eine Kombination aus einer genetisch bedingten Ionenstromdysbalance sowie einer lokalen Leitungsverzögerung im rechten Ventrikel im Epikardium vor, die zu einem Phase-2-Reentry führt. Das höchste Risiko eines plötzlichen Herztodes besteht bei Patienten, die bereits eine Synkope erlitten haben, unter Ruhebedingungen typische ST-Hebungen am J-Punkt aufweisen und bei denen während der programmierten Stimulation Kammerflimmern auslösbar ist. Asymptomatische Patienten, bei denen die EKG-Veränderungen lediglich nach Gabe eines Natriumkanalblockers nachzuweisen sind, haben eine bessere Prognose. Erst kürzlich sind die ersten Konsensusempfehlungen der großen Fachgesellschaften für die Diagnosekriterien und die Therapie von Patienten erschienen.

Summary

Brugada syndrome is an ion channel disease which is associated with an increased risk of sudden cardiac death. Most probably the pathogenesis of ventricular fibrillation in these patients is a combination of both genetically determined repolarisation abnormalities and conduction delay in the right ventricular epicardium. The highest risk of sudden cardiac death is present in patients who have experienced syncope before, who reveal the pathognomic electrocardiographic changes already at rest and who have inducible ventricular fibrillation. Asymptomatic patients who have the J point elevations only after administration of a sodium channel blocker seem to be at lower risk. Most recently the latest joint consensus recommendations of the largest societies for diagnostic criteria, indications for genetic testing and therapy have been published.

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Authors and Affiliations

  1. Klinik für Innere Medizin, Kardiologie, Nephrologie und internistische Intensivmedizin, Klinikum Ludwigsburg, Posilipostraße 4, 71640, Ludwigsburg, Deutschland

    Christian Wolpert, Claudia Herrera-Siklody, Ulli Parade, Christian Strotmann & Norman Rüb

  2. Medizinische Klinik, Universitätsmedizin Mannheim, Mannheim, Deutschland

    Christian Wolpert

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  1. Christian Wolpert
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  2. Claudia Herrera-Siklody
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  3. Ulli Parade
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  5. Norman Rüb
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Correspondence to Christian Wolpert.

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Wolpert, C., Herrera-Siklody, C., Parade, U. et al. Brugada-Syndrom. Herzschr Elektrophys 24, 209–216 (2013). https://doi.org/10.1007/s00399-013-0294-2

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