Risk Prediction and Follow-Up

In this chapter, we will examine three topics: (1) the follow-up of subjects at risk of developing cardiac amyloidosis (CA); (2) risk stratification of patients diagnosed with CA, i.e., the prediction of future disease evolution; and (3) the assessment of treatment response in patients diagnosed with CA. Cardiac involvement should always be searched in patients with amyloid light-chain (AL) amyloidosis, as well as in carriers of TTR gene mutations associated with cardiac involvement. Risk stratification is important in both AL- and amyloid transthyretin (ATTR)-CA to tailor the therapeutic strategy and follow-up based on the expected disease evolution. Finally, some cardiovascular magnetic resonance and scintigraphy studies suggest that blocking further amyloid deposition allows to reduce cardiac amyloid burden, and also that tracking the response to disease-modifying therapies is feasible. Larger studies are needed to confirm these findings and define algorithms for patient management based on imaging findings.