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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Heart transplants provide value for patients with AL, ATTR cardiac amyloidosis

With the disease becoming more and more common in the United States, researchers have been hard at work determining the best possible treatment options.

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America’s most expensive CV drug needs a 93% price cut

A 92.6% reduction in the list price of tafamidis—an effective but ultimately unaffordable drug designed to treat transthyretin amyloid cardiomyopathy—would be required to make the medication accessible to the average heart patient, researchers reported in Circulation Feb. 12.

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Cardiac amyloidosis increasingly common in US

New research suggests the incidence of cardiac amyloidosis in the U.S. is trending up, bringing with it high rates of morbidity and mortality.

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ASNC releases multisocietal amyloidosis imaging guidelines

The American Society of Nuclear Cardiology, together with eight other nuclear medicine and cardiology societies, have published a consensus document outlining the best practices for imaging and diagnosing cardiac amyloidosis.

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ASNC publishes new amyloidosis imaging guidelines

“We anticipate that these expert multisocietal consensus recommendations on multimodality imaging in cardiac amyloidosis will standardize the diagnosis and improve the management of this highly morbid and underdiagnosed disease," wrote authors of the new guidelines published in the Journal of Nuclear Cardiology.

FDA OKs Pfizer’s tafamidis to treat cardiac amyloidosis

The FDA has cleared two new drugs, tafamidis and tafamidis meglumine, for the treatment of cardiomyopathy caused by a rare disorder known as transthyretin-mediated amyloidosis (ATTR-CM).

FDA fast-tracks review process for cardiac amyloidosis drug

Tafamidis, Pfizer’s treatment for transthyretin amyloid cardiomyopathy (ATTR-CM), has been granted a priority review designation from the FDA, potentially speeding its path to approval.

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Why Cleveland Clinic began screening carpal tunnel patients for cardiac amyloidosis

Cleveland Clinic researchers identified amyloid deposits in 10.2 percent of patients undergoing carpal tunnel release surgery, suggesting biopsies of hand tissue could be an early signal of life-threatening cardiac amyloidosis.

Around the web

Ron Blankstein, MD, professor of radiology, Harvard Medical School, explains the use of artificial intelligence to detect heart disease in non-cardiac CT exams.

Eleven medical societies have signed on to a consensus statement aimed at standardizing imaging for suspected cardiovascular infections.