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Kardiale Amyloidose

Cardiac amyloidosis

  • CME Weiterbildung • Zertifizierte Fortbildung
  • Published:
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Zusammenfassung

Der Begriff kardiale Amyloidose subsumiert eine Herzbeteiligung unterschiedlicher, meist systemischer Erkrankungsformen von Amyloidose, bei der eine abnorme Faltung von Proteinen zu einer Ablagerung in unterschiedlichen Organen führt. Hieraus resultiert eine progrediente Funktionseinschränkung des jeweils betroffenen Organs bis hin zum Organversagen. Die Symptome und klinischen Befunde der einzelnen Formen bei einer kardialen Beteiligung sind unspezifisch und unterscheiden sich nicht wesentlich voneinander. Da jedoch Prognose und Therapie der einzelnen Formen stark divergieren, ist die genaue Differenzierung des spezifischen Amyloidproteins mittels immunhistochemischer Färbung von großer Bedeutung. Eine kardiale Beteiligung bei Amyloidose ist meist mit einer deutlich verschlechterten Prognose assoziiert. Diagnostisch richtungsweisend für eine kardiale Amyloidose ist eine Herzinsuffizienzsymptomatik mit Niedervoltage im EKG und zum Teil massiver linksventrikulärer Hypertrophie in der Echokardiographie. Aufgrund der schwierigen Risikostratifizierung und adäquaten Patientenauswahl für die verschiedenen Therapieformen, die mit hohem Aufwand und beträchtlicher Komplikationsrate verknüpft sind, ist eine Therapieplanung und Durchführung an einem erfahrenen Zentrum zu empfehlen. Zielgerichtetes diagnostisches Vorgehen, prognostische Faktoren und Therapiemöglichkeiten der unterschiedlichen Amyloidoseformen werden im folgenden Beitrag diskutiert.

Abstract

The term “cardiac amyloidosis” describes cardiac manifestation in several predominantly systemic amyloid diseases. Abnormal pathological folding of the proteins results in deposition of beta-sheeted fibrils in different organs causing progressive organ failure. Even though symptoms and clinical findings of each amyloid disease are largely identical, prognosis and therapy regimens differ significantly rendering precise differentiation of the underlying amyloid protein by immunohistochemistry of major clinical importance. In general, symptomatic cardiac involvement in amyloidosis is associated with poor prognosis. Characteristic findings of cardiac amyloidosis are symptoms of heart failure in combination with an ECG exhibiting a low voltage pattern and echocardiographic evidence of marked left ventricular hypertrophy. Challenging risk stratification and patient selection for the different therapeutic approaches, in part associated with high therapy-related mortality, mandates medical care at experienced centers. Targeted diagnostic algorithms, risk predictors, and therapeutic approaches for different forms of amyloidosis commonly involving the heart are discussed in the present review.

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Authors and Affiliations

  1. Abteilung für Innere Medizin III (Kardiologie, Angiologie, Pneumologie), Medizinische Klinik der Universität Heidelberg (Ludolf-Krehl-Klinik), Im Neuenheimer Feld 410, 69120, Heidelberg, Deutschland

    A.V. Kristen, E. Giannitsis, A. Bauer, S. Hardt, P.A. Schnabel, F.-U. Sack, H.A. Katus &  T.J. Dengler

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  1. A.V. Kristen
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  2. E. Giannitsis
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  3. A. Bauer
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  5. P.A. Schnabel
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  6. F.-U. Sack
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  7. H.A. Katus
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  8. T.J. Dengler
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Correspondence to T.J. Dengler.

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Kristen, A., Giannitsis, E., Bauer, A. et al. Kardiale Amyloidose. Kardiologe 1, 123–138 (2007). https://doi.org/10.1007/s12181-007-0015-3

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