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