Risk stratification in transthyretin-related cardiac amyloidosis

Frontiers in Cardiovascular Medicine

Cardiovascular Medicine

Edoardo Cittadini¹  Andrea Imperatrice¹  Beatrice Musumeci¹  Domitilla Russo¹  Matteo Sclafani¹  Riccardo Scirpa¹  Lorenzo Mazzocchi¹  Alessandro Tropea¹  Giacomo Tini²  Camillo Autore³ 

[1] Division of Cardiology, Department of Clinical and Molecular Medicine, Sapienza University of Rome, Rome, Italy;Division of Cardiology, Department of Clinical and Molecular Medicine, Sapienza University of Rome, Rome, Italy;IRCCS San Raffaele Pisana, Rome, Italy;San Raffaele Cassino (FR), Cassino, Italy;

关键词: risk stratification;    cardiac amyloidosis;    natural history;    transthyretin;    heart failure;    arrhythmias;   

DOI  :  10.3389/fcvm.2023.1151803

 received in 2023-01-27, accepted in 2023-03-06,  发布年份 2023

来源: Frontiers

【 摘 要 】

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.

【 授权许可】

Unknown   
© 2023 Scirpa, Cittadini, Mazzocchi, Tini, Sclafani, Russo, Imperatrice, Tropea, Autore and Musumeci.