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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Cardiac amyloidosis remains underreported in US

Twice as many U.S. deaths due to cardiac amyloidosis were reported in 2015 than in 1979, but a study in JAMA Cardiology suggests the disease remains vastly underdiagnosed.

Eidos Therapeutics Initiates Phase 2 Clinical Trial for AG10 Targeting Transthyretin Amyloidosis Cardiomyopathy

SAN FRANCISCO, May 3, 2018 /PRNewswire/ — Eidos Therapeutics, Inc., a clinical stage biopharmaceutical company developing a novel oral therapy to treat transthyretin (TTR) amyloidosis (ATTR), today announced dosing of the first patient in the Phase 2 clinical trial of AG10 in patients with ATTR cardiomyopathy.

Image reconstruction algorithm, MRI-derived heart strain values can aid prognosis in amyloidosis patients

Recent research found strain parameters taken from a cine MRI-based deformable registration algorithm (DRA) can determine the severity of amyloid buildup in the heart and may provide prognostic information on patients with light-chain (AL) amyloidosis.

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PET technique visualizes amyloid deposits in heart

PET with 11C-PIB provides a noninvasive method for visualizing amyloid deposits in the heart, according to a study published in the February issue of the Journal of Nuclear Medicine. The researchers suggest that 11C-PIB eventually may be used in the clinical setting as both a diagnostic tool and a treatment follow-up method.