IMR Press / RCM / Volume 18 / Issue 4 / DOI: 10.3909/ricm0887
Open Access Review
A Focused Review on the Pathophysiology, Diagnosis, and Management of Cardiac Amyloidosis
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1 Department of Medicine, Mount Sinai Medical Center, Miami Beach, FL
2 Cardiac Ultrasound Laboratory, Massachusetts General Hospital, Harvard Medical School, Boston, MA
3 Columbia University Division of Cardiology, Mount Sinai Heart Institute, Miami Beach, FL
Rev. Cardiovasc. Med. 2017 , 18(4), 123–133; https://doi.org/10.3909/ricm0887
Abstract
Amyloidosis is a systemic disorder that results from abnormal protein metabolism, producing amyloid fibrils that are subsequently deposited within vital organs. Cardiac involvement is typically associated with the specific subtypes of immunoglobulin lightchain, transthyretin, secondary amyloidosis, and dialysis-related amyloidosis. The hallmark of cardiac amyloidosis is the development of restrictive cardiomyopathy and heart failure, usually with a preserved left ventricular ejection fraction. The diagnosis is based on the integration of clinical signs and symptoms, echocardiography, cardiac magnetic resonance imaging, nuclear scintigraphy, electrocardiography, and cardiac biomarkers. Traditionally, management of heart failure symptoms and prevention of heart failure exacerbations have been the cornerstones of therapy. However, various treatments are currently under investigation that aim to eliminate or neutralize the underlying amyloidogenic substrate. Herein, we provide a focused review and discussion of the cardiovascular manifestations, epidemiologic and clinical characteristics, diagnostic modalities, and treatment strategies of cardiac amyloidosis.
Keywords
Amyloidosis
Heart failure
Cardiomyopathy
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