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AMY DA site - I can identify - Video 1
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00:00:15
It's important to really have a high index of suspicion when you're seeing somebody
00:00:20
with worsening heart failure who has other signs of AL amyloidosis.
00:00:25
As a cardiologist, AL cardiac amyloidosis will manifest itself to you in a couple of ways.
00:00:32
Patients will come to you with worsening heart failure symptoms.
00:00:36
They are going to develop thickening of the heart wall.
00:00:40
So we look at left ventricular wall thickness in excess of 12 mm on echo
00:00:46
in the absence of other causes, like hypertension, hypertrophic cardiomyopathy.
00:00:58
It becomes very important to be able to communicate with your nephrology colleagues
00:01:05
when you see somebody with heart failure who also has a lot of protein in their urine.
00:01:09
It becomes important to collaborate with your neurology colleagues when you see
00:01:15
somebody with heart failure who also has a peripheral neuropathy or an autonomic neuropathy.
00:01:20
That’s because this disease has myriad manifestations
00:01:24
and if you ignore other organ systems,
00:01:27
you’re going to miss the fact that the patient has AL cardiac amyloidosis.
00:01:31
So that’s why because it’s a systemic disease,
00:01:34
cross-disciplinary collaborations are imperative to an early diagnosis.
00:01:45
A cardiologist will obtain an electrocardiogram,
00:01:48
an ECG, and an echocardiogram, and those two tests will start pointing towards
00:01:55
an infiltrative cardiomyopathy or a restrictive cardiomyopathy.
00:01:59
Now, the next step becomes figuring out whether this is really cardiac amyloidosis or not.
00:02:05
Sometimes, a cardiac MRI can be helpful in making that diagnosis.
00:02:11
So your job now is to figure out what the precursor protein is,
00:02:15
and that can be done by ruling out a plasma cell dyscrasia,
00:02:20
by checking serum and urine immunofluorescence electrophoresis,
00:02:26
by checking serum-free light chains.
00:02:28
The next step is to rule out transthyretin cardiac amyloidosis,
00:02:33
and that needs to be ruled out by a pyrophosphate scan.
00:02:37
When there is suspicion of cardiac amyloidosis,
00:02:39
I think it’s important to involve a hematologist early,
00:02:43
because a hematologist can really help you with doing that workup for a plasma cell dyscrasia.