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Case Reports
. 2019 Aug 21;1(2):141-145.
doi: 10.1016/j.jaccas.2019.06.027. eCollection 2019 Aug.

Evaluating Suspected Cardiac Amyloidosis

Manish Motwani  1   2 , Parthiban Arumugam  3 , Alberto Rocci  4   5 , Luigi Venetucci  1   2
Affiliations
Case Reports

Evaluating Suspected Cardiac Amyloidosis

Manish Motwani et al. JACC Case Rep. .

Abstract

A 71-year-old-woman presented with breathlessness, general tiredness and orthopnea. Echocardiography and electrocardiogram were suspicious for cardiac amyloidosis. This case illustrates contemporary evaluation to confirm the diagnosis and distinguish between different types of amyloid. (Level of Difficulty: Beginner.).

Keywords: AL, light-chain amyloidosis; ATTR, amyloid transthyretin; CA, cardiac amyloidosis; DPD, 2,3-dicarboxypropane-1,1-diphosphonate; ECG, electrocardiogram; FLC, free light chain; LV, left ventricular; LVH, left ventricular hypertrophy; SPECT, single-photon emission computed tomography; TTE, transthoracic echocardiography; cardiac magnetic resonance; echocardiography; electrocardiogram; imaging; nuclear medicine; restrictive.

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Figures

None
Graphical abstract
Figure 1
Figure 1
Cardiac Imaging in Amyloidosis Echocardiography (top row) shows moderate concentric left ventricular hypertrophy, severely dilated atria, small pericardial effusion, thickened interatrial septum, and thickened valves. Late gadolinium enhancement with cardiac magnetic resonance (middle panel) shows extensive heterogeneous myocardial enhancement consistent with diffuse amyloid infiltration. Comparatively low-grade uptake (Perugini grade 1) with 2,3-dicarboxypropane-1,1-diphosphonate (DPD) on hybrid single-photon emission computed tomography/computed tomography (SPECT-CT) (bottom row) favors light-chain amyloid, which was subsequently confirmed on bone marrow biopsy.
Figure 2
Figure 2
Typical Electrocardiogram Features of Cardiac Amyloidosis Low-voltage limb leads with absent left ventricular hypertrophy voltage criteria despite increasd wall thickness on echocardiography, pseudo-infarction in anterior precordial leads, and coarse atrial fibrillation.
Figure 3
Figure 3
Four-Step Algorithm for Evaluating Suspected Cardiac Amyloidosis A summary flowchart and explanatory notes for contemporary evaluation of suspected cardiac amyloidosis (diagnosis and typing). +ve = positive; AL = light-chain amyloidosis; ATTR = amyloid transthyretin; ATTRm = familial amyloid transthyretin; ATTRwt = wild-type amyloid transthyretin; BM = bone marrow; CMR = cardiac magnetic resonance; DPD = 2,3-dicarboxypropane-1,1-diphosphonate; ECG = electrocardiogram; ECV = extracellular volume; FLC = free light chain; HF = heart failure; LGE = late gadolinium enhancement; LS = longitudinal strain; LVH = left ventricular hypertrophy; MGUS = monoclonal gammopathy of unknown significance; PYP = pyrophosphate; TTR = transthyretin.
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References

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