Transcript
Announcer:
Welcome to Heart Matters on ReachMD. On this episode, Dr. Sumeet Mitter joins us to discuss multidisciplinary care in transthyretin amyloid cardiomyopathy, or ATTR-CM. He’s the Director of Inova Schar Heart and Vascular Research and the Infiltrative and Restrictive Cardiomyopathy Center at Inova Health in Virginia. Here’s Dr. Mitter now.
Dr. Mitter:
Philosophically, transthyretin amyloidosis is a systemic disease. The first tissue it was found in was actually the lungs, but our disease endpoints and clinical trials were geared towards the heart, and at least in the variant or population with a genetic variant, the nervous system. So by that, you need a cardiologist and a neurologist—someone who can help diagnose the disease both in the heart and then also the peripheral nervous system.
But it's really important to rely on our colleagues in other disciplines because patients may have disease manifestations elsewhere. One of the big clinical conundrums is the validity of a scan, but often we have to rule out light chain amyloidosis, and so we may have to include a hematologist-oncologist in our multidisciplinary care team to truly say, "I don't see light chain amyloidosis here. So go ahead with your pyrophosphate or nuclear scintigraphy scan to diagnose or go ahead and treat for transthyretin disease." Or if we start seeing evidence of monoclonal protein, sometimes it's a monoclonal gammopathy of uncertain significance. Some of us are very comfortable looking at some of those labs to say, "This is kind of a non-issue." But more often, people aren't comfortable in making that diagnosis of a monoclonal gammopathy of uncertain significance, and so we rely on our colleagues in hematology-oncology to differentiate if this is actually light chain amyloidosis.
And within that, if there's a chance of it being light chain amyloidosis, that's a different precursor protein, and sometimes it can be associated with multiple myeloma or other hematologic malignancies, and patients may need chemotherapy. And so it's really important that we have trusted neurologists and hematologists that we can work with to help not only rule out other systemic disease but also appropriately diagnose individuals.
Also, what's really important is a genetic counselor, because we're doing genetic testing for a lot of these patients, and having a seasoned, skilled genetic counselor to talk to patients about genetic screening of disease within families at the point at which they may want to do it based off age. Some people don't like things in their records. Or if you're really concerned about affected family members and wanting to diagnose early, talking about that and then getting the amount of therapy. So that's really important.
I am blessed, even within my own program, to have nephrologists who I work with because we see a lot of, at least in terms of labs, renal involvement. And so we need their help because while TTR amyloid deposits, there's still a clinical cascade of events, congestion, advancements of renal disease, and nephropathy, and they may need other things that then are a byproduct of having amyloidosis.
Announcer:
That was Dr. Sumeet Mitter discussing multidisciplinary management of ATTR-CM. To access this and other episodes in our series, visit Heart Matters on ReachMD.com, where you can Be Part of the Knowledge. Thanks for listening!
