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Myocardial Characteristics, Cardiac Structure, and Cardiac Function in Systemic Light-Chain Amyloidosis.

Olivier F Clerc1, Sarah A M Cuddy1, Michael Jerosch-Herold2

  • 1Division of Nuclear Medicine and Molecular Imaging, Department of Radiology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA; Cardiac Amyloidosis Program, Cardiovascular Division, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.

JACC. Cardiovascular Imaging
|July 13, 2024
PubMed
Summary
This summary is machine-generated.

Cardiac magnetic resonance imaging (MRI) detects early myocardial changes in systemic light-chain amyloidosis. Extracellular volume and global longitudinal strain improve prognosis and predict major adverse cardiac events.

Keywords:
T1 mappingcardiac magnetic resonance imagingextracellular volumeglobal longitudinal strainlight-chain (AL) amyloidosismyocardial characterization

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Area of Science:

  • Cardiology
  • Medical Imaging
  • Biomarkers

Background:

  • Systemic light-chain amyloidosis (AL) is characterized by cardiac involvement, leading to poor patient outcomes.
  • Early detection of myocardial alterations is crucial for improving prognosis in AL amyloidosis patients.

Purpose of the Study:

  • To utilize cardiac magnetic resonance imaging (MRI) for detecting early myocardial changes in AL amyloidosis.
  • To analyze longitudinal changes in cardiac parameters during therapy.
  • To predict major adverse cardiac events (MACE) using cardiac MRI findings.

Main Methods:

  • Prospective enrollment of recently diagnosed AL amyloidosis participants.
  • Classification into AL amyloidosis with cardiomyopathy (AL-CMP) and without (AL-non-CMP) based on biomarkers and wall thickness.
  • Serial cardiac MRI at baseline, 6 months, and 12 months (for AL-CMP patients).
  • MACE definition included all-cause death, heart failure hospitalization, and cardiac transplantation.

Main Results:

  • Extracellular volume (ECV) was elevated in all AL-CMP and 47% of AL-non-CMP participants.
  • ECV showed a trend of increase at 6 months and returned to baseline at 12 months in AL-CMP.
  • Global longitudinal strain (GLS) improved significantly at 6 and 12 months in AL-CMP patients.
  • Both ECV and GLS were strong predictors of MACE, outperforming the Mayo stage.

Conclusions:

  • Cardiac MRI-derived ECV effectively detects subclinical myocardial alterations in AL amyloidosis.
  • Therapy leads to distinct longitudinal changes in ECV and improvements in GLS.
  • ECV and GLS provide significant added prognostic value beyond the established Mayo stage for risk stratification.