Canadian Cardiovascular Society/Canadian Heart Failure Society Joint Position Statement on the Evaluation and Management of Patients With Cardiac Amyloidosis

The Canadian Journal of Cardiology
Nowell M FineShelley Zieroth

Abstract

Cardiac amyloidosis is an under-recognized and potentially fatal cause of heart failure and other cardiovascular manifestations. It is caused by deposition of misfolded precursor proteins as fibrillary amyloid deposits in cardiac tissues. The two primary subtypes of systemic amyloidosis causing cardiac involvement are immunoglobulin light chain (AL), a plasma cell dyscrasia, and transthyretin (ATTR), itself subdivided into a hereditary subtype caused by a gene mutation of the ATTR protein, and an age-related wild type, which occurs in the absence of a gene mutation. Clinical recognition requires a high index of suspicion, inclusive of the extracardiac manifestations of both subtypes. Diagnostic workup includes screening for serum and/or urine monoclonal protein suggestive of immunoglobulin light chains, along with serum cardiac biomarker measurement and performance of cardiac imaging for findings consistent with amyloid infiltration. Modern cardiac imaging techniques, including the use of nuclear scintigraphy with bone-seeking radiotracer to noninvasively diagnose ATTR cardiac amyloidosis, have reduced reliance on the gold standard endomyocardial biopsy. Disease-modifying therapeutic approaches have evolved significantly, parti...Continue Reading

Citations

Jun 5, 2020·The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques·Dina NamiranianRami Massie
Oct 13, 2020·Expert Opinion on Pharmacotherapy·R SpoladoreG Fragasso
Jan 25, 2021·The Canadian Journal of Cardiology·Margot K DavisSean A Virani
Jan 17, 2021·The Canadian Journal of Cardiology·Robert J H MillerNowell M Fine
Dec 15, 2020·The Canadian Journal of Cardiology·Mark B BadrovJohn S Floras
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May 25, 2021·CJC Open·Omid Kiamanesh, Mustafa Toma
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