Cardiac amyloidosis - ATTR wild-type

Discussion:

This case illustrates and reviews the MR imaging features of cardiac amyloidosis:

  • left ventricular hypertrophy, increased indexed mass, thickened interatrial septum
  • faster myocardial nulling than the blood pool 2,3
  • dark blood pool and subendocardial late gadolinium enhancement (LGE) in a non-coronary distribution with transmural pattern 1-4
  • high values in native T1 and extracellular volume (ECV) 5-9

Higher values in extracellular volume (ECV) 5-9 wall thickness and mass have been reported for ATTR amyloidosis, whereas higher native T1 values 5-9 and higher T2 values the latter as a biomarker for myocardial edema 7 have been reported for cardiac AL amyloidosis. This is, why ATTR is in favor over AL amyloidosis in this case.

An endomyocardial biopsy obtained about six months after the cardiac MRI revealed cardiac interstitial ATTR amyloidosis. Subsequent genotyping lead to the diagnosis of wild-type ATTR amyloidosis.

Also of note is that myocardial edema is of worse prognosis in cardiac AL amyloidosis 7.

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