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. 

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.

September 9, 2019

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).

May 6, 2019

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.

January 14, 2019

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.

October 17, 2018
heart

Tafamidis shows promise for treating cardiac amyloidosis

Research presented this week at the European Society of Cardiology Congress in Munich suggests a new treatment may be emerging for transthyretin amyloid cardiomyopathy—a condition previously thought to be rare and untreatable.

August 28, 2018

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.

July 25, 2018

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.

May 3, 2018

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.

April 24, 2018