AMY DA site - I can identify - Video 1
It's important to really have a high index of suspicion when you're seeing somebody
with worsening heart failure who has other signs of AL amyloidosis.
As a cardiologist, AL cardiac amyloidosis will manifest itself to you in a couple of ways.
Patients will come to you with worsening heart failure symptoms.
They are going to develop thickening of the heart wall.
So we look at left ventricular wall thickness in excess of 12 mm on echo
in the absence of other causes, like hypertension, hypertrophic cardiomyopathy.
It becomes very important to be able to communicate with your nephrology colleagues
when you see somebody with heart failure who also has a lot of protein in their urine.
It becomes important to collaborate with your neurology colleagues when you see
somebody with heart failure who also has a peripheral neuropathy or an autonomic neuropathy.
That’s because this disease has myriad manifestations
and if you ignore other organ systems,
you’re going to miss the fact that the patient has AL cardiac amyloidosis.
So that’s why because it’s a systemic disease,
cross-disciplinary collaborations are imperative to an early diagnosis.
A cardiologist will obtain an electrocardiogram,
an ECG, and an echocardiogram, and those two tests will start pointing towards
an infiltrative cardiomyopathy or a restrictive cardiomyopathy.
Now, the next step becomes figuring out whether this is really cardiac amyloidosis or not.
Sometimes, a cardiac MRI can be helpful in making that diagnosis.
So your job now is to figure out what the precursor protein is,
and that can be done by ruling out a plasma cell dyscrasia,
by checking serum and urine immunofluorescence electrophoresis,
by checking serum-free light chains.
The next step is to rule out transthyretin cardiac amyloidosis,
and that needs to be ruled out by a pyrophosphate scan.
When there is suspicion of cardiac amyloidosis,
I think it’s important to involve a hematologist early,
because a hematologist can really help you with doing that workup for a plasma cell dyscrasia.
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