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Review
. 2023 Mar 21:10:1151803.
doi: 10.3389/fcvm.2023.1151803. eCollection 2023.

Risk stratification in transthyretin-related cardiac amyloidosis

Riccardo Scirpa  1 , Edoardo Cittadini  1 , Lorenzo Mazzocchi  1 , Giacomo Tini  1   2 , Matteo Sclafani  1 , Domitilla Russo  1 , Andrea Imperatrice  1 , Alessandro Tropea  1 , Camillo Autore  3 , Beatrice Musumeci  1
Affiliations
Review

Risk stratification in transthyretin-related cardiac amyloidosis

Riccardo Scirpa et al. Front Cardiovasc Med. .

Abstract

Transthyretin related cardiac amyloidosis (TTR-CA) is an infiltrative cardiomyopathy that cause heart failure with preserved ejection fraction, mainly in aging people. Due to the introduction of a non invasive diagnostic algorithm, this disease, previously considered to be rare, is increasingly recognized. The natural history of TTR-CA includes two different stages: a presymptomatic and a symptomatic stage. Due to the availability of new disease-modifying therapies, the need to reach a diagnosis in the first stage has become impelling. While in variant TTR-CA an early identification of the disease may be obtained with a genetic screening in proband's relatives, in the wild-type form it represents a challenging issue. Once the diagnosis has been made, in order to identifying patients with a higher risk of cardiovascular events and death it is necessary to focus on risk stratification. Two prognostic scores have been proposed both based on biomarkers and laboratory findings. However, a multiparametric approach combining information from electrocardiogram, echocardiogram, cardiopulmonary exercise test and cardiac magnetic resonance may be warranted for a more comprehensive risk prediction. In this review, we aim at evaluating a step by step risk stratification, providing a clinical diagnostic and prognostic approach for the management of patients with TTR-CA.

Keywords: arrhythmias; cardiac amyloidosis; heart failure; natural history; risk stratification; transthyretin.

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Conflict of interest statement

The handling editor [JG] declared a past co-authorship with the authors [GT, RS]. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Natural history of TTR-CA: the pre-symptomatic stage and the symptomatic stage.
Figure 2
Figure 2
A multiparametric approach to predict prognosis in TTR-CA. ECV, extracellular volume; GLS, global longitudinal strain; GFR, glomerular filtration rate; LGE, late gadolinium enhancement; MR, mitral regurgitation; TR, tricuspid regurgitation; VCO2, carbon dioxide output; VE, minute ventilation; VO2, maximum oxygen consumption.
See this image and copyright information in PMC

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