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ESC 2026 | Updated recommendations for ATTR-CM: treatment guidelines and monitoring disease progression
Ronald Witteles, MD, Stanford University, Stanford, CA, discusses updated recommendations for the management of transthyretin amyloid cardiomyopathy (ATTR-CM). Dr Witteles discusses current treatment guidance, the definition of “disease progression”, and recent recommendations for monitoring progression using six proposed clinical parameters. This interview took place during the 2026 European Society of Cardiology (ESC) Congress in Munich, Germany.
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Transcript
There’s a couple of guidelines we can think about. There’s guidelines of how to treat patients, and there’s guidelines about if and how to monitor for progression in the disease. So let me start with the first, how to treat patients. This is pretty clear. We have three good agents that are approved, certainly in the United States and most of Europe and in many other parts of the world, for ATTR cardiomyopathy...
There’s a couple of guidelines we can think about. There’s guidelines of how to treat patients, and there’s guidelines about if and how to monitor for progression in the disease. So let me start with the first, how to treat patients. This is pretty clear. We have three good agents that are approved, certainly in the United States and most of Europe and in many other parts of the world, for ATTR cardiomyopathy. There is not evidence that definitively says that one is better than the other. So in reality, the only wrong answer, and this is what the guidelines will generally say, the only wrong answer is to not choose any of the therapies. Any of the three are good options. Now, for patients who have polyneuropathy from ATTR amyloidosis in addition to cardiomyopathy, they should get silencer therapy because that has been what has been shown in clinical trials to positively affect outcomes. We have not yet seen that with stabilizers. In terms of progression, there’s been a lot of interest in what does progression mean, how should we measure it, et cetera. A group that I was involved with put out a position statement in Journal of Heart Failure in the early part of 2026, outlining six parameters that can be used to measure signs of quote-unquote progression. But what does that mean? And we took great pains in the manuscript to make clear that progression is really measuring that their heart failure is getting worse. It doesn’t necessarily mean that it’s because they’re having a lot more amyloid deposits or that in some way that drug therapy has failed. And for that reason, we are very clear that because a patient has quote-unquote progressed does not mean that we know that they would do better switching therapies. Some patients choose to switch, which is fine, but we don’t know the answer. And probably most of the quote-unquote progression is due to other factors like challenges with volume overload independent of their amyloid therapy or even more commonly atrial arrhythmias like atrial fibrillation.
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