Fokus: Mitral Annulus Disjunction Arrhythmic Syndrome

Zusammenfassung

Als „Mitral annular Disjunction“ (MAD) bezeichnet man eine pathologische, umschriebene, bindegewebige Dehiszenz zwischen dem kompakten Myokard des linken Ventrikels und dem Mitralklappenanulus. Das Bindegewebe ersetzt anulusnah das Myokard und strahlt in unregelmäßiger Form in die benachbarte Muskulatur des linken Ventrikels ein. Die MAD tritt oft in Verbindung mit einem Mitralklappenprolaps (MVP) auf. Die inhomogene Fibrosierung des klappennahen Myokards sowie eingelagerte und versprengte Purkinje-Fasern bilden ein arrhythmogenes Substrat. Die MAD wird deshalb gehäuft bei Patienten mit ventrikulären Herzrhythmusstörungen bzw. bei plötzlichem Herztod mit und ohne MVP gefunden. Die Diagnose wird mittels Echokardiografie oder kardialer Magnetresonanztomografie bzw. Computertomografie gestellt. Da die prognostische Bedeutung der MAD unklar ist, gibt es bisher keine Therapieempfehlung.

Abstract

“Mitral annular disjunction” (MAD) describes a tissue gap between the left ventricular myocardium and the mitral valve ring. It consists of fiber tissue that spreads in an unsharp manner into the ventricular myocardium and the papillary muscles. MAD is closely linked to mitral valve prolapse (MVP). It is said to have arrhythmogenic characteristiscs. MAD is found in MVP patients with ventricular arrhythmias and sudden cardiac death. But it may also be seen in patients with arrhythmias and no MVP or patients with no arrhythmias but MVP. MAD can be diagnosed with echocardiography or cardiac magnetic resonance imaging or CT scan. Since the prognosis of MAD is unknown, no specific treatments are established.

Schlüsselwörter

Mitralklappenprolaps - ventrikuläre Arrhythmien - Bildgebung - Mitral Annulus Disjunction

Key words

mitral valve prolapse - ventricular arrhythmias - imaging - mitral annulus disjunction

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