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Cleveland Clinic Journal of Medicine

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Symptoms to Diagnosis

A 50-year-old man presents with shortness of breath

Kaylee Watson, MD, David Wolinsky, MD, Mauricio Velez, MD and David Snipelisky, MD, FACC
Cleveland Clinic Journal of Medicine February 2023, 90 (2) 95-101; DOI: https://doi.org/10.3949/ccjm.90a.22021
Kaylee Watson
Fellow Researcher, Department of Cardiology and Cardiothoracic Surgery, Cleveland Clinic Florida, Weston, FL
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David Wolinsky
Section Head, Nuclear Cardiology, Medical Director, Cardiac Amyloid Center, Department of Cardiology and Cardiothoracic Surgery, Cleveland Clinic Florida, Weston, FL
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Mauricio Velez
Section of Heart Failure and Cardiac Transplant Medicine, Department of Cardiology and Cardiothoracic Surgery, Cleveland Clinic Florida, Weston, FL
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David Snipelisky
Section of Heart Failure and Cardiac Transplant Medicine, Department of Cardiology and Cardiothoracic Surgery, Cleveland Clinic Florida, Weston, FL
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    Figure 1

    Parasternal long-axis view on echocardiography demonstrates diffuse concentric left ventricular hypertrophy (arrows).

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    Figure 2

    Technetium-99m pyrophosphate scintigraphy. (A) Anterior and (B) left anterior oblique views in our patient demonstrated grade 2 to 3 myocardial uptake of the radiotracer (circles) 3 hours after injection, thus meeting diagnostic criteria for transthyretin amyloid cardiomyopathy.

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    TABLE 1

    Findings that may warrant cardiac amyloidosis workup

    Extracardiac manifestationsCardiac manifestationsImaging findings
    Bilateral carpal tunnel syndromePersistent mildly elevated troponin levelsEchocardiography: increased thickness of left ventricle, right ventricular free wall, atrioventricular valves, interatrial septum
    Unprovoked biceps tendon ruptureSymptomatic hypotension or orthostasis in response to hypertensive medicationElectrocardiography: discrepancy in QRS voltage and left ventricular thickness
    Lumbar stenosisUnexplained atrioventricular block, heart block, or bundle branch blockStrain echocardiography: longitudinal impairment with apical sparing
    Sensorimotor polyneuropathyElevated N-terminal-pro-brain natriuretic peptide not proportionate to severity of heart failureCardiac magnetic resonance imaging: increased extracellular volume or late enhancement
    Autonomic dysfunctionFamily history of cardiomyopathyChest radiography: cardiomegaly
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    TABLE 2

    Disease-modifying therapies for transthyretin amyloidosis (ATTR) approved by the US Food and Drug Administration

    DrugIndicationEffect on transthyretin
    TafamidisWild-type or hereditary ATTR cardiomyopathyStabilizer
    VutrisiranHereditary ATTR with neuropathySilencer
    PatisiranHereditary ATTR with neuropathySilencer
    InotersenHereditary ATTR with neuropathySilencer
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In this issue

Cleveland Clinic Journal of Medicine: 90 (2)
Cleveland Clinic Journal of Medicine
Vol. 90, Issue 2
1 Feb 2023
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A 50-year-old man presents with shortness of breath
Kaylee Watson, David Wolinsky, Mauricio Velez, David Snipelisky
Cleveland Clinic Journal of Medicine Feb 2023, 90 (2) 95-101; DOI: 10.3949/ccjm.90a.22021

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A 50-year-old man presents with shortness of breath
Kaylee Watson, David Wolinsky, Mauricio Velez, David Snipelisky
Cleveland Clinic Journal of Medicine Feb 2023, 90 (2) 95-101; DOI: 10.3949/ccjm.90a.22021
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  • Article
    • DIFFERENTIAL DIAGNOSIS
    • CASE CONTINUED: IMAGING RESULTS
    • RED FLAGS FOR CARDIAC AMYLOIDOSIS
    • PATHOGENESIS OF CARDIAC AMYLOIDOSIS
    • DIAGNOSIS OF AMYLOIDOSIS
    • MANAGEMENT OF CARDIAC AMYLOIDOSIS
    • FURTHER MANAGEMENT IN AMYLOIDOSIS PATIENT
    • CASE CONCLUSION
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