Article

Cardiac amyloidosis: An update on diagnosis and treatment

Joseph P. Donnelly, MD and Mazen Hanna, MD
Cleveland Clinic Journal of Medicine December 2017, 84 (12 suppl 3) 12-26; DOI: https://doi.org/10.3949/ccjm.84.s3.02

Article Figures & Data

Figures

  • Figure 1
    Figure 1

    The 2 main types of amyloidosis that affect the heart. (A) Immunoglobulin light chain amyloidosis (AL) results from aberrant plasma cell production of monoclonal light chains that misfold. (B) Transthyretin amyloidosis (ATTR) results from transthyretin (TTR) produced by the liver that dissociates into monomers and misfolds. The misfolded proteins aggregate to form oligomers, protofilaments, and mature amyloid fibrils that deposit extracellulary in the interstitial space of the myocardium.

  • Figure 2
    Figure 2

    Cardiac amyloidosis pathology. (A) The heart on autopsy reveals characteristic biventricular thickening as well as biatrial dilation and thickening of both atrioventricular valves. (B) Hemotoxylin and eosin staining shows diffuse amyloid deposition. (C) The characteristic “apple-green” birefringence of Congo red stain under polarized light. (D) Example of immunohistochemistry performed for amyloid typing, in this case positive for lambda light chain and negative for kappa light chain and transthyretin.

  • Figure 3
    Figure 3

    The classic hallmark of cardiac amyloidosis. (A) A 12-lead electrocardiogram showing atrial fibrillation, low voltage in the limb leads, and a pseudoinfarct pattern with Q waves in leads V1-V2. (B) Echocardiogram, parasternal long-axis view, showing increased septal and posterior left ventricular wall thickness, dilated left atrium, and thickening of the mitral valve. (C) Echocardiogram, apical 4-chamber view, showing diffuse thickening of both ventricles, biatrial dilation, and thickened mitral and tricuspid valve leaflets.

  • Figure 4
    Figure 4

    Noninvasive imaging for cardiac amyloidosis. (A) Longitudinal strain imaging using 2-dimensional speckle tracking echocardiography reveals the characteristic bull’s-eye pattern of apical sparing. (B) Cardiac magnetic resonance imaging displays left ventricular and right ventricular thickening and (C) with contrast, a diffuse late gadolinium enhancement pattern that is diffuse and subendocardial, which also involves the right ventricle and left atrium. (D) 99mTechnetium pyrophosphate scan shows grade 3 myocardial radiotracer uptake characteristic of transthyrethin cardiac amyloidosis.

  • Figure 5
    Figure 5

    Diagnostic algorithm for cardiac amyloidosis (CA). Serum free light chain (sFLC) assay and serum/urine immunofixation are ordered to workup immunoglobulin light chain amyloidosis (AL). 99mTechnetium pyrophosphate (99mTcPYP) scan is ordered to workup transthyretin amyloidosis (ATTR). Normal serum free light chains and normal immunofixation with a strongly positive 99mTcPYP scan is diagnostic of ATTR-CA. Abnormal sFLC assay or immunofixation is suggestive but not diagnostic of AL-CA, and should prompt a bone marrow biopsy.

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