Cardiac Amyloidosis

With the first drug treatments for cardiac amyloidosis recently entering the market, there has been an explosion of interest to diagnose and care for these patients. It is considered a rare disease, but many experts now say it is actually just be under diagnosed. The disease is caused by protein misfolding. Normally soluble proteins in the bloodstream become insoluble and deposit abnormally in the tissues and organs throughout the body. There are three main kinds of amyloid that affect the heart, light chain amyloid (AL) and two types of transthyretin amyloid (ATTR or TTR). The first type of ATTR is hereditary, or familial amyloid, and the second is wild type, or age-related TTR amyloid. Nuclear imaging, echocardiography, CT and MRI all play roles in diagnosing amyloid and in determining the subtype, which is required for targeted treatment. 

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Clinical trials paused due to heart patient’s hospitalization

Intellia Therapeutics paused clinical trials for ATTR-CM and ATTR-PN after a patient was hospitalized due to a significant liver injury. 

Nuclear cardiology is entering a new era—one that goes well beyond the traditional focus on myocardial perfusion imaging. According to Marcelo Di Carli, MD, editor-in-chief of the Journal of Nuclear Cardiology and chief of the Division of Nuclear Medicine at Brigham and Women’s Hospital, the field is rapidly expanding into diagnosing and monitoring complex cardiac diseases such as amyloidosis, sarcoidosis, myocarditis, and cardiac infections.

Nuclear cardiology expands beyond perfusion imaging as it enters a new era

Beyond amyloidosis, nuclear cardiology is also increasingly used to image inflammation and infection in the myocardium, especially in diseases such as cardiac sarcoidosis and myocarditis.

Prem Soman, MD, PhD, FACC, FRCP, past president of the American Society of Nuclear Cardiology (ASNC), the Richard S. Caligiuri Endowed Chair in Amyloidosis and Heart Failure, director of the Cardiac Amyloidosis Center, director of nuclear cardiology, and a professor of medicine, University of Pittsburgh Medical Center (UPMC) Heart and Vascular Institute. He is presenting the keynote Mario Verani Memorial Lecture at ASNC 2025, where he will discuss transforming cardiac amyloidosis care.

How nuclear cardiology has transformed care for cardiac amyloidosis

Prem Soman, MD, PhD, explained how early detection and new therapies have been game-changers for the treatment and diagnosis of cardiac amyloidosis.

Cardiac amyloidosis ATTR-CM drug promotion at the 2024 American Heart Association meeting.

High cost of cardiac amyloidosis drugs may limit access for low-income patients

"We cannot accept a system where only those with means can benefit from life-changing drugs. We are a wealthy nation that spends trillions on healthcare. We must ensure that income and wealth do not determine access," explained JACC Editor-in-Chief Harlan M. Krumholz, MD.

Ultromics EchoGo Amyloidosis artificial intelligence

AI-enhanced echocardiography improves early detection of cardiac amyloidosis

The first FDA-cleared AI model of its kind was found to be an accurate screening tool for cardiac amyloidosis.

heart drugs with stethoscope

Work begins on first ATTR-CM study of its kind

ACT-EARLY is testing the hypothesis that treating asymptomatic carriers of a pathogenic TTR variant with the drug acoramidis could delay amyloidosis from developing.

Alnylam Pharmaceuticals has received U.S. Food and Drug Administration (FDA) approval for vutrisiran, a subcutaneous injection the company sells under the brand name Amvuttra, to treat adult patients with transthyretin amyloid cardiomyopathy (ATTR-CM).

FDA approves vutrisiran to treat ATTR-CM

The subcutaneous drug, sold under the brand name Amvuttra, joins an ATTR-CM market that already includes Pfizer’s tafamidis (Vyndaqel, Vyndamax) and BridgeBio’s acoramidis (Attruby).

cardiac amyloidosis on bone scan

Continuation of 99mTc-PYP shortage prompts need for alternative cardiac amyloidosis imaging

After three years of intermittent shortages of nuclear imaging tracer technetium-99m pyrophosphate, there are no signs of the shortage abating.