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Top 10 Things To Know When Performing Cardiac Imaging to Assess Cardiac Amyloidosis

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source: American College of Cardiology

year: 2020

authors: Renee Bullock-Palmer

summary/abstract:

Amyloidoses are protein-folding disorders in which more than one organ is infiltrated by proteinaceous deposits known as amyloid. The deposits are derived from one of several amyloidogenic precursor proteins, and the prognosis of the disease is determined both by the organ(s) involved and the type of amyloid. Amyloid involvement of the heart, cardiac amyloidosis, carries the worst prognosis of any involved organ, and light-chain (AL) amyloidosis is the most serious form of the disease.
 
Cardiac amyloidosis may be due to myocardial deposition of transthyretin protein derived from the liver known as transthyretin cardiac amyloidosis (ATTR) or may be due to AL amyloidosis with myocardial deposition of immunoglobulin light-chain proteins derived from a clone of plasma cells.
 
Clinical features suggestive of cardiac amyloidosis are recurrent unexplained heart failure, particularly in African Americans males >60 years old with preserved left ventricular ejection fraction, an unexplained increase in left ventricular wall thickness (>12 mm), and a restrictive left ventricular filling pattern suggestive of increased filling pressures. There is usually left atrial dysfunction in the absence of atrial fibrillation and an abnormal global longitudinal strain (GLS) with apical sparing pattern.

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