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Assessment of Cardiac Morphological and Functional Changes in Mouse Model of Transverse Aortic Constriction by Echocardiographic Imaging
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Myocardial structural and functional changes in cardiac amyloidosis: insights from a prospective observational

Franz Duca1, René Rettl1, Christina Kronberger1

  • 1Department of Internal Medicine II, Division of Cardiology, Medical University of Vienna, Waehringer Guertel 18-20, 1090 Vienna, Austria.

European Heart Journal. Cardiovascular Imaging
|August 7, 2023
PubMed

Insights

Serial extracellular volume (ECV) quantification in cardiac amyloidosis (CA) patients shows significant progression over time. This ECV change predicts patient outcomes and aids in evaluating amyloid-specific treatments.

Area of Science:

  • Cardiology
  • Radiology
  • Biomedical Imaging

Background:

  • Cardiac amyloidosis (CA) is characterized by amyloid deposition in the myocardium, leading to increased extracellular volume (ECV).
  • Longitudinal studies on ECV progression in CA patients are limited.
  • Understanding ECV dynamics is crucial for assessing disease severity and treatment efficacy.

Purpose of the Study:

  • To investigate the progression of extracellular volume (ECV) in patients with cardiac amyloidosis (CA).
  • To determine the prognostic impact of ECV changes over time in CA.
  • To evaluate the utility of serial ECV quantification in monitoring CA and treatment response.

Main Methods:

  • Serial cardiac magnetic resonance (CMR) examinations with ECV quantification were performed in 103 CA patients between 2012 and 2021.
  • ECV was measured at baseline and follow-up (median 12 months) for total, transthyretin (ATTR), and light chain (AL) CA cohorts.
  • Survival analyses were conducted to assess the prognostic value of ECV change.

Main Results:

  • Median ECV increased significantly in all CA cohorts over the follow-up period (P < 0.001).
  • Treatment-naïve ATTR patients showed a higher median ECV increase compared to treated patients (+5.7% vs. +2.3%, P = 0.004).
  • Median change in ECV was a significant predictor of outcome across all CA types (HR > 1.07, P < 0.013).

Conclusions:

  • Serial ECV quantification is valuable in managing CA patients.
  • ECV change serves as a prognostic marker for patient outcomes in CA.
  • Monitoring ECV progression can inform the evaluation of amyloid-specific therapies.
Abstract

Keywords:
T1 mappingcardiac amyloidosiscardiac magnetic resonance imagingextracellular volumelight chain amyloidosistransthyretin amyloidosis

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