American Heart Association takes on cardiac amyloidosis with new initiative
A new three-year initiative from the American Heart Association (AHA) is seeking to improve how clinicians identify and manage cardiac amyloidosis. It was once considered a rare disease, but experts now recognize it is a more common cause of heart failure.
The ATTR-CM Discovery Initiative brings together multidisciplinary teams to strengthen the awareness, diagnosis and treatment of transthyretin amyloid cardiomyopathy (ATTR-CM). The goal is to catch patients early before they progress and develop advanced heart failure. Cardiovascular Business discussed the initiative with Mathew S. Maurer, MD, a volunteer member of the AHA ATTR Amyloidosis Strategic Advisory Group guiding the initiative. He is the Arnold and Arlene Goldstein Professor of Cardiology at Columbia University Irving Medical Center and director of the cardiac amyloidosis program at NewYork-Presbyterian/Columbia University Irving Medical Center.
“This is really a transformation in the field of cardiac amyloidosis,” Maurer explained.
For decades, he said cardiac amyloidosis was thought to be extremely rare and difficult to diagnose, because confirmation often required an invasive heart biopsy performed only at specialized centers. Maurer said that changed dramatically with the development of a noninvasive diagnostic pathway that combines nuclear cardiology scintigraphy imaging with blood testing for monoclonal proteins.
“Now you can diagnose a patient pretty easily,” Maurer explained. “We’ve learned that it’s actually a very common cause of heart failure and other cardiovascular conditions, so it’s not as rare of a disease as we thought.”
At the same time, treatment options have expanded significantly. Maurer said there are now three FDA-approved therapies for ATTR-CM.
“All these therapies are much more effective if administered early in the course of the disease. This national clinical initiative sponsored by the AHA is to improve awareness about the disease, make sure physicians are following the non-biopsy diagnostic algorithm appropriately, and most importantly, ensure timely administration of what is life-saving and disease-modifying therapy.”
The initiative will involve cardiologists, imaging specialists, hematologists and allied health professionals working together to improve continuity of care and establish more consistent pathways for evaluation and treatment.
Maurer emphasized that differentiating the two major forms of cardiac amyloidosis is critical, because they require very different therapies.
Light-chain (AL) amyloidosis is driven by abnormal plasma cells and is treated similarly to a blood cancer, often using chemotherapy. ATTR amyloidosis, by contrast, is caused by the misfolding of a liver-produced protein called transthyretin and requires targeted therapies that stabilize or suppress that protein.
“You can’t treat what you don’t know, but you have to make sure that you’re accurate in your diagnosis. That’s really a critically important step that the AHA is trying to put their muster behind, ensuring clinicians are doing the right thing for the right patient at the right time,” Maurer said.
The AHA program also aims to broaden education beyond cardiology specialists. Maurer said patients with amyloidosis often see multiple providers before receiving a diagnosis, including primary care physicians and other specialists. For this reason, he said the AHA initiative does not just focus on cardiologists, but all types providers who could touch one of these patients.
Early diagnosis is especially important because current therapies prevent new amyloid deposits and cannot reverse damage that is already done to the heart.
“The key here is finding people with early New York Heart Class I and II heart failure and identifying the disease at that point in time,” Maurer said.
Diagnosing amyloidosis can be challenging because patients often present with thickened heart walls that may resemble more common conditions such as hypertensive heart disease, aortic stenosis or hypertrophic cardiomyopathy.
He said modern noninvasive diagnostic approach has significantly improved access to testing. Nuclear scintigraphy uses bone tracers that were originally developed decades ago for cancer imaging. Researchers later discovered the tracers could identify transthyretin amyloid deposits in the heart.
Maurer explained that when nuclear scintigraphy is combined with blood testing to rule out AL amyloidosis, the technique becomes highly accurate for diagnosing ATTR-CM without the need for biopsy. In the past, he said endomyocardial biopsy was not something most patients wanted to do, so noninvasive imaging has dramatically changed the landscape.
The AHA initiative hopes broader adoption of these diagnostic strategies will lead to faster referrals, earlier treatment and better long-term outcomes for patients with cardiac amyloidosis.
Dave Fornell has covered healthcare for more than 17 years, with a focus in cardiology and radiology. Fornell is a 5-time winner of a Jesse H. Neal Award, the most prestigious editorial honors in the field of specialized journalism. The wins included best technical content, best use of social media and best COVID-19 coverage. Fornell was also a three-time Neal finalist for best range of work by a single author. He produces more than 100 editorial videos each year, most of them interviews with key opinion leaders in medicine. He also writes technical articles, covers key trends, conducts video hospital site visits, and is very involved with social media. E-mail: [email protected]