Cardiac amyloidosis

Discussion:

Findings are consistent with cardiac amyloidosis, including concentric ventricular hypertrophy and advanced diffuse fibrosis involving all 4 cardiac chambers. Left ventricular ECV is severely elevated, 45-55%. There is: biventricular systolic dysfunction, LVEF 44% and RVEF 36%. Suspected grade 3 diastolic dysfunction. Peak systolic velocity across the mitral valve 48 cm/s (VENC adjustment is 150 cm/sec). The ratio of E to A, exceeds 2 with nearly absent A wave, suggestive of grade III diastolic dysfunction.

ECV = (1- Hematocrit) ( 1 /T1 Myo post - 1/ T1 Myo pre ) divided by  (1/ T1 blood post - 1/ T1 blood pre)1

Histopathology (microscopic description):

  • patchy moderately extensive interstitial amyloid deposition; a Congo red stain confirms the diagnosis
  • LC MS/MS detected a peptide profile consistent with ATTR (transthyretin/prealbumin)-type amyloid deposition
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