Abstract
SummaryCardiac amyloidosis is caused by deposition of abnormally folded proteins (amyloid). The most common forms of amyloidosis which present with cardiac involvement are light-chain amyloidosis (AL) and transthyretin amyloidosis (ATTR). Even with novel treatments emerging, the prognosis of these patients remains poor once amyloid deposits in the heart. Therefore, knowledge on clinical and imaging features of cardiac amyloidosis is crucial to make an early diagnosis and improve patient outcomes. This article reviews the most important diagnostic findings of cardiac amyloidosis and gives an overview on the therapeutic management of these patients, including supportive-, device- and disease-specific drug therapies focusing on AL amyloidosis.
Highlights
Cardiac amyloidosis (CA) is caused by the deposition of misfolded proteins in the extracellular myocardial space [1]
In cardiac ATTR, amyloid deposits primarily cause myocardial stiffness and impaired function by affecting myocardial relaxation and in later stages can lead to a decline of contractility
It is feasible to use a stepwise approach in diagnosing cardiac amyloidosis (CA), as some imaging modalities may not be widely available
Summary
Cardiac amyloidosis (CA) is caused by the deposition of misfolded proteins in the extracellular myocardial space [1]. In ATTR amyloidosis transthyretin (TTR), a protein mainly produced by the liver misfolds either due to point mutations in the TTR gene or due to agerelated processes. In the latter form the exact pathophysiological mechanisms are not known [3]. AL amyloid deposits on the other hand have been described to have cytotoxic effects which directly cause cell death of cardiomyocytes These effects may explain the especially dismal prognosis in patients with cardiac involvement in AL, compared to patients with ATTR amyloidosis, with median survival rates from onset of symptoms of 1.7 and 6.1 years, respectively [5,6,7]. We summarize therapeutic considerations and treatment options for patients with CA
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