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Arrhythmien bei Amyloidose

Arrhythmias and amyloidosis

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Herzschrittmachertherapie + Elektrophysiologie Aims and scope Submit manuscript

Zusammenfassung

Die kardiale Amyloidose ist eine infiltrative Kardiomyopathie, die durch die extrazelluläre Ablagerung von Amyloidfibrillen im Myokard verursacht wird. Obwohl Patienten mit kardialer Amyloidose hauptsächlich Symptome von Herzinsuffizienz zeigen, sind Arrhythmien und Erkrankungen des Reizleitungssystems häufige Krankheitsmanifestationen. Vorhofflimmern (VHF) wird bei bis zu 80 % der Patienten zum Zeitpunkt der Diagnosestellung beobachtet. Typischerweise haben die Patienten aufgrund gleichzeitig auftretender Erkrankungen des Reizleitungssystems eine normale Herzfrequenz. Das thrombembolische Risiko ist bei Patienten mit kardialer Amyloidose besonders hoch, linksatriale Thromben wurden sogar ohne Vorhandensein von VHF beobachtet. AV-Knoten- und infrahisäre Erkrankungen sind hochprävalent, und permanente Schrittmacher werden häufig benötigt. Die Verwendung implantierbarer Defibrillatoren in dieser Population ist umstritten. Diese Übersicht fasst die veröffentlichten Daten und therapeutischen Strategien im Zusammenhang mit Arrhythmien und Erkrankungen des Reizleitungssystems zusammen, mit dem Ziel, den Lesern bei der Entscheidungsfindung in teilweise komplexen klinischen Situationen zu unterstützen.

Abstract

Cardiac amyloidosis is an infiltrative cardiomyopathy characterized by the extracellular deposition of amyloid fibrils within the myocardium. Beyond heart failure, patients with cardiac amyloidosis commonly present with arrhythmias and conduction system disorders. Atrial fibrillation is observed in up to 80% of patients at the time of diagnosis, with patients typically maintaining normal heart rates due to concurrent atrioventricular nodal disease. The thromboembolic risk is particularly high in patients with cardiac amyloidosis, and left atrial thrombi have been observed even in the absence of atrial fibrillation. Conduction system diseases are also highly prevalent, often necessitating permanent pacemaker implantation. The use of implantable defibrillators in this population remains controversial. This overview of published data and therapeutic strategies related to arrhythmias and conduction system disorders aims to assist readers in decision-making in complex clinical scenarios.

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Authors and Affiliations

  1. Medizinische Klinik 3–Kardiologie/Angiologie, Department of Cardiology, University Hospital Frankfurt, Theodor-Stern-Kai 7, 60596, Frankfurt, Deutschland

    Maria Papathanasiou MDa, Lara S. Schlender MD, Victoria Louise Johnson MD & Reza Wakili MD

  2. German Center for Cardiovascular Research (DZHK), Partner Site Rhein-Main, 60596, Frankfurt, Deutschland

    Reza Wakili MD

Authors
  1. Maria Papathanasiou MDa
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  2. Lara S. Schlender MD
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  3. Victoria Louise Johnson MD
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  4. Reza Wakili MD
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Correspondence to Maria Papathanasiou MDa.

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Interessenkonflikt

M. Papathanasiou, L.S. Schlender, V.L. Johnson und R. Wakili geben an, dass kein Interessenkonflikt besteht.

Für diesen Beitrag wurden von den Autor/-innen keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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Supplementary Information

Video. Transösophageale Echokardiographie mit Nachweis einer linksatrialen Thrombose im Kontext einer kardialen Leichtketten-Amyloidose ohne Nachweis von Vorhofflimmern.

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Papathanasiou, M., Schlender, L.S., Johnson, V.L. et al. Arrhythmien bei Amyloidose. Herzschr Elektrophys (2024). https://doi.org/10.1007/s00399-024-01016-y

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  • DOI: https://doi.org/10.1007/s00399-024-01016-y

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