What is cardiac amyloidosis?

Updated: Mar 25, 2020
  • Author: Gyanendra K Sharma, MD, FACC, FASE; Chief Editor: Terrence X O'Brien, MD, MS, FACC  more...
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Answer

Amyloidosis is a clinical disorder caused by extracellular deposition of insoluble fibrils (approximately 7.5-10 nm wide) with beta-pleated sheet configuration. [1] The protein misfolding abnormalities result in amyloid fibrils and may manifest as primary, secondary, familial, or senile amyloidosis. Amyloid deposition can occur in multiple organs (eg, heart, liver, kidney, skin, eyes, lungs, nervous system) resulting in a variety of clinical manifestations. [2] Cardiac involvement is a progressive disorder resulting in early death due to congestive heart failure (CHF) and arrhythmias. Cardiac involvement can occur as part of a systemic disease or as a localized phenomenon. [3, 4]

Educating patients about dietary restrictions and medications is useful. Education about symptoms of heart failure and stroke would be helpful in guiding patients to seek early medical advice.

This article provides an overview of the primary systemic amyloidosis (AL) that predominantly affects the heart. [5] In the last few years amyloid transthyretin (ATTR) cardiomyopathy has been better defined with ease of diagnosis and newer therapeutic options.


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