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Sinagra et al. Vessel Plus 2022;6:44 https://dx.doi.org/10.20517/2574-1209.2021.143 Page 3 of 4

because of concerns regarding worse long-term outcomes due to the amyloid involvement of other organs
[22]
or the risk of recurrent amyloid in the graft . However, the development of specific chemotherapy
regimens enabling autologous stem cell transplantation has made long-term control of the plasma cell
[22]
dyscrasia possible . Therefore, timely diagnosis of CA is a critical issue to derive the largest treatment
benefit.

Grey areas and future directions in amyloidosis
The availability of novel therapeutic strategies turning CA from an ominous to a treatable disease has
provided new impulse towards research, but important issues still need to be addressed:

(1) Differentiate indolent “cardiac accumulation” associated with aging form CA, the authentic infiltrative
disease.

(2) Understand the prevalence of AL and ATTR amyloidosis in various clinical settings.

(3) The clinical application of SPECT imaging should be done to increase the diagnostic accuracy of bone
tracer scintigraphy and to quantify the amyloid burden in the heart.

(4) Define the minimal disease threshold to justify the initiation of novel treatments, particularly in light of
their high costs and possible side effects.

(5) Define the criteria to identify patients with a so advanced CA that no significant benefit is expected from
initiation of disease-modifying drugs.

(6) Identify the most appropriate tool to quantify the global amyloid burden and monitor its changes under
specific treatment.

(7) Define baseline parameters to predict treatment response and orient clinical decision-making related to
discontinuation of current drugs in favor of other medications or initiation on combination therapy.

A major reappraisal is underway concerning AL and ATTR amyloidosis in the modern era of precision
medicine, and the collaboration among physicians of many specialties is essential to address evidence-based
management of patients with this systemic disease.

DECLARATIONS
Authors’ contributions
Contribute to the conception, outline and writing of the manuscript: Sinagra G, Porcari A

Availability of data and materials
Not applicable.

Financial support and sponsorship
None.

Conflicts of interest
Both authors declared that there are no conflicts of interest.