Cardiac amyloidosis across the spectrum of left ventricular function: multimodal functional and prognostic insights

Sabrina Belaidi1,2,3, Mounira Kharoubi1,2,4,5,6,7, Olivier Lairez2,7,8

  • 1French National Reference Centre for Cardiac Amyloidosis, Hospital Henri Mondor, Créteil, Île-de-France, France.

PubMed

Insights

Cardiac amyloidosis (CA) presents across all ejection fraction phenotypes, not just preserved. Integrating LVEF, GLS, and CIx improves risk prediction for better patient management.

Area of Science:

  • Cardiology
  • Cardiovascular Imaging
  • Amyloidosis Research

Background:

  • Cardiac amyloidosis (CA) is increasingly recognized but often associated with heart failure with preserved ejection fraction (HFpEF).
  • However, CA can manifest across the spectrum of left ventricular ejection fraction (LVEF), including mildly reduced (HFmrEF) and reduced (HFrEF) ejection fraction.
  • Accurate recognition of CA across all LVEF phenotypes is crucial for timely diagnosis and effective risk stratification.

Purpose of the Study:

  • To investigate the spectrum of LVEF phenotypes in cardiac amyloidosis.
  • To evaluate the prognostic relevance of LVEF in conjunction with global longitudinal strain (GLS) and cardiac index (CIx).
  • To develop a prognostic model integrating these parameters for improved risk stratification in CA patients.

Main Methods:

  • A retrospective study of 2244 CA patients (light chain, hereditary transthyretin, wild-type transthyretin amyloidosis) was conducted.
  • LVEF was classified per European Society of Cardiology guidelines.
  • Prognostic value was assessed using Kaplan-Meier analysis and a decision tree combining LVEF, GLS, and CIx, with external validation.

Main Results:

  • While HFpEF was most common, 39% of patients had HFmrEF or HFrEF.
  • Survival varied significantly by phenotype: median survival was 30 months (HFrEF), 40 months (HFmrEF), and not reached (HFpEF).
  • A decision tree integrating LVEF, GLS, and CIx identified four distinct prognostic groups with varying 4-year mortality risks.

Conclusions:

  • Cardiac amyloidosis encompasses the full range of LVEF phenotypes.
  • Combining LVEF, GLS, and CIx significantly enhances prognostic stratification in CA.
  • A multimodal imaging approach is recommended for early diagnosis and personalized management of CA.
Abstract

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