Abstract and Introduction
Cardiac amyloidosis is a growing field, with advancements in diagnosis and management. Cardiac biomarkers are used to predict survival and to develop severity staging systems. Cardiac biomarkers are also used in clinical practice to stratify patients for treatment and to evaluate response to therapies. The current review summarizes the major clinical utility of current biomarkers in patients with cardiac amyloidosis and provides insights about future areas of investigation.
Amyloidosis refers to a group of systemic diseases caused by the extracellular accumulation of insoluble, misfolded protein aggregates in various organs (Figure 1). The types of amyloidosis that are commonly associated with cardiac involvement are acquired monoclonal immunoglobin light chain amyloidosis (AL), wild-type transthyretin amyloidosis (ATTRwt), and hereditary transthyretin amyloidosis (ATTRm). Regardless of cause, the degree of cardiac involvement at the time of diagnosis is the most important prognostic indicator.
Amyloidosis Fibril Deposition
A precursor protein misfold and an aggregate into amyloid fibrils. Amyloidosis fibrils deposit in the extracellular space of several organs. In the myocardium, it causes predominantly diastolic heart failure with release of biomarkers. BNP = brain natriuretic peptide; NT-proBNP = N-terminal pro–B-type natriuretic peptide. Adapted with permission from Nativi-Nicolau (30).
The purpose of this review was to summarize the history and current clinical use of cardiac biomarkers for assessment of prognosis and response to therapy in cardiac amyloidosis (Central Illustration) and to describe the ongoing areas of investigation into cardiac amyloidosis.
Utility of Biomarkers in Cardiac Amyloidosis
Cardiac biomarkers can be used to estimate staging and prognosis and also to assess response to therapies and disease progression. ATTR = transthyretin amyloidosis; cTnT/cTnI = cardiac troponin T/cardiac troponin I; eGFR = estimated glomerular filtration rate; NT-proBNP = N-terminal pro–B-type natriuretic peptide.
JACC Heart Fail. 2020;8(9):701-711. © 2020 American College of Cardiology Foundation