AMY DA site - I can identify - Video 1


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    It's important to really have a high index of suspicion when you're seeing somebody
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    with worsening heart failure who has other signs of AL amyloidosis.
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    As a cardiologist, AL cardiac amyloidosis will manifest itself to you in a couple of ways.
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    Patients will come to you with worsening heart failure symptoms.
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    They are going to develop thickening of the heart wall.
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    So we look at left ventricular wall thickness in excess of 12 mm on echo
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    in the absence of other causes, like hypertension, hypertrophic cardiomyopathy.
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    It becomes very important to be able to communicate with your nephrology colleagues
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    when you see somebody with heart failure who also has a lot of protein in their urine.
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    It becomes important to collaborate with your neurology colleagues when you see
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    somebody with heart failure who also has a peripheral neuropathy or an autonomic neuropathy.
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    That’s because this disease has myriad manifestations
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    and if you ignore other organ systems,
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    you’re going to miss the fact that the patient has AL cardiac amyloidosis.
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    So that’s why because it’s a systemic disease,
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    cross-disciplinary collaborations are imperative to an early diagnosis.
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    A cardiologist will obtain an electrocardiogram,
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    an ECG, and an echocardiogram, and those two tests will start pointing towards
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    an infiltrative cardiomyopathy or a restrictive cardiomyopathy.
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    Now, the next step becomes figuring out whether this is really cardiac amyloidosis or not.
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    Sometimes, a cardiac MRI can be helpful in making that diagnosis.
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    So your job now is to figure out what the precursor protein is,
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    and that can be done by ruling out a plasma cell dyscrasia,
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    by checking serum and urine immunofluorescence electrophoresis,
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    by checking serum-free light chains.
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    The next step is to rule out transthyretin cardiac amyloidosis,
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    and that needs to be ruled out by a pyrophosphate scan.
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    When there is suspicion of cardiac amyloidosis,
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    I think it’s important to involve a hematologist early,
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    because a hematologist can really help you with doing that workup for a plasma cell dyscrasia.