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Histopathological and Immunohistochemical Characteristics of Different Types of Cardiac Amyloidosis

Zarina V Gioeva1, Liudmila M Mikhaleva1, Nikita A Gutyrchik1,2

  • 1Avtsyn Research Institute of Human Morphology, Petrovsky National Research Centre of Surgery, 117418 Moscow, Russia.

International Journal of Molecular Sciences
|October 16, 2024
PubMed

Insights

Cardiac amyloidosis impacts prognosis. This study found transthyretin amyloidosis (ATTR) is underdiagnosed, while AL amyloidosis, particularly AL kappa, indicates a poorer prognosis in heart failure patients.

Area of Science:

  • Cardiology
  • Pathology
  • Genetics

Background:

  • Cardiac involvement is a critical prognostic factor in systemic amyloidosis.
  • Understanding the types of amyloidosis affecting the heart is crucial for patient management.
  • Accurate clinical diagnosis of cardiac amyloidosis, especially transthyretin amyloidosis (ATTR), remains a challenge.

Purpose of the Study:

  • To identify amyloid types most frequently associated with cardiac involvement.
  • To describe histopathological and clinical features of cardiac amyloidosis.
  • To estimate the proportion of cardiac amyloidosis cases missed by clinical diagnosis before death.

Main Methods:

  • Retrospective observational study of 98 patients with systemic amyloidosis.
  • Comparison of cardiac amyloidosis findings between endomyocardial biopsies (EMBs) and autopsy reports.
  • Analysis of amyloid types (AL, ATTR, AA) and their prevalence in cardiac tissue.

Main Results:

  • ATTR amyloidosis was the most common type in EMBs (52%) and autopsy (40%).
  • AL amyloidosis was diagnosed in 46% of EMBs and 46% of autopsies.
  • ATTR amyloidosis was clinically diagnosed in only 9.5% of autopsy cases, indicating significant underdiagnosis.

Conclusions:

  • ATTR amyloidosis is a common and likely underdiagnosed cause of heart failure in elderly patients.
  • AL amyloidosis, particularly AL kappa, is associated with intense amyloid deposits and a poorer prognosis.
  • Histopathological examination via EMBs and autopsy is vital for accurate diagnosis and prognosis of cardiac amyloidosis.

Keywords:
amyloidcardiac amyloidosisendomyocardial biopsyimmunoglobulin light chainimmunohistochemistrytransthyretin

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