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Das Brugada-Syndrom ist eine genetisch bedingte Erkrankung die mit einem autosomal dominanten Erbgang verbunden ist. Das Oberflächen-EKG charakterisiert sich durch intermittierende ST-Strecken-Hebungen in V1–V3 und ein Rechtsschenkelblock. Manchmal gibt es auch verlängerte Leitungszeiten in andere Herzstrukturen. Das Brugada-Syndrom manifestiert sich manchmal mit Episoden schneller Kammertachykardien oder Kammerflimmern. In etwa 15% der Patienten würden Mutationen im Bereich des SCN5A Gen nachgewiesen, das den kardialen Natriumstrom beeinflusst. Genetische Verschiedenartigkeit ist demonstriert aber weitere ursächlich verwickelte Erbfaktoren erwarten Identifizierung. Ein „Genotype-Phenotype” Verhältnis, wie in das Langes-QT-Syndrom, war bis vor kurzem unbekannt. Kürzlich aber haben wir beobachtet das die Brugada-Syndrom Patienten mit eine Mutation im SCN5A-Gen verlängerte kardialen Leitungszeiten haben. Weitere Identifizierung „Genotype-Phenotype”-Verhältnissen in das Brugada-Syndrom wird vielleicht weitere ursächliche Erbfaktoren identifizieren. Jetzt werden wir eventuelle Möglichkeiten weitere „Genotype-Phenotype”-Verhältnissen besprechen.
Herzschrittmachertherapie + Elektrophysiologie – Springer Journals
Published: Feb 12, 2014
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