Cardiac amyloidosis

Case contributed by Azza Elgendy
Diagnosis certain


Progressive shortness of breath

Patient Data

Age: 60 years
Gender: Female

Circumferential thickening of left ventricular walls. Focal increased trabeculations about the lateral apical wall, which does not meet the criteria for a noncompaction. Mild concentric thickening of right ventricular walls. Left atrial enlargement.

There is widespread delayed enhancement involving the myocardium and the subendocardium compatible with cardiac amyloidosis. The subendocardial enhancement involves both atria and ventricles.

Annotated image

Diffuse prolongation of the native T1-weighted mapping (reaching up to 1200 ms) is demonstrated. Diffuse shortening of the T1-weighted post-mapping images. Abnormal extracellular volume imaging (ECV) is demonstrated. Abnormal patchy mid-myocardium as well as subendocardial enhancement involving most left ventricle.

Suspected grade 3 diastolic dysfunction as manifested by near absent A wave.

Nuclear medicine

The patient received an intravenous injection of 22.6 mCi of technetium 99m pyrophosphate.  Following a three-hour delay, a 5-minute planar anterior view study was acquired.  SPECT cardiac imaging with CT attenuation correction.

The SPECT study shows focal uptake of radiotracer in all left ventricular segments. There is mild right ventricular uptake. Myocardial uptake of technetium pyrophosphate in the left and right ventricles consistent with ATTR-amyloid heart disease.

Case 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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