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Myocardial Edema and Prognosis in Amyloidosis

Tushar Kotecha1, Ana Martinez-Naharro2, Thomas A Treibel3

  • 1National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom; Institute of Cardiovascular Science, University College London, London, United Kingdom; Royal Free Hospital, London, United Kingdom.

Journal of the American College of Cardiology
|June 23, 2018
PubMed

Insights

Myocardial edema, detected by cardiovascular magnetic resonance T2 mapping, is present in cardiac amyloidosis. Higher T2 values in untreated light-chain amyloidosis predict prognosis, suggesting additional damage mechanisms beyond infiltration.

Area of Science:

  • Cardiology
  • Cardiovascular Imaging
  • Medical Diagnostics

Background:

  • Cardiac involvement significantly impacts prognosis in light-chain (AL) and transthyretin (ATTR) amyloidosis.
  • ATTR amyloidosis generally has a better prognosis than AL amyloidosis, despite comparable or greater amyloid infiltration, indicating other contributing factors to disease severity.

Purpose of the Study:

  • To investigate the presence and prognostic significance of myocardial edema in patients diagnosed with cardiac amyloidosis.
  • To compare myocardial edema levels across different types of amyloidosis and healthy controls.

Main Methods:

  • Cardiovascular magnetic resonance (CMR) with T1 and T2 mapping was performed on 286 patients, including those with AL amyloidosis, ATTR amyloidosis, and healthy volunteers.
  • Endomyocardial biopsies were obtained from 16 patients to histologically assess myocardial edema.
  • Cox regression models were utilized to determine the prognostic value of myocardial T2 values.

Main Results:

  • Myocardial T2 values were elevated in all amyloidosis groups compared to controls, with the highest levels observed in untreated AL amyloidosis patients.
  • Histological examination confirmed edema in 87.5% of biopsy samples, with involvement ranging from 5% to 40% of the myocardium.
  • Elevated myocardial T2 independently predicted mortality in AL amyloidosis patients, even after adjusting for other clinical factors.

Conclusions:

  • Myocardial edema is a detectable feature in cardiac amyloidosis using both histology and CMR T2 mapping.
  • Increased myocardial T2, particularly in untreated AL amyloidosis, serves as a significant predictor of prognosis.
  • These findings suggest that myocardial edema represents an additional mechanism contributing to mortality in cardiac amyloidosis, beyond mere amyloid infiltration.
Abstract

Keywords:
CMRT2 mappingamyloidosis

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