A new staging system for hereditary transthyretin amyloidosis in the era of specific amyloidosis therapies.

ABSTRACT:

Objectives

Currently, there are two prognosis staging systems validated for transthyretin amyloidosis (ATTR). We sought to develop a new staging system dedicated to hereditary transthyretin amyloidosis (ATTRv) patients on specific treatments.

Methods and results

A total of 258 patients diagnosed with ATTRv from two cardiac amyloidosis reference centres in France and Romania were stratified into three disease stages based on NT-proBNP, estimated glomerular filtration rate (eGFR) and global longitudinal strain (GLS). A staging system was created using the following criteria: GLS ≥ -11%, NT-proBNP ≥ 2000 ng/L and eGFR ≤ 65 mL/min. Stage I was defined as the presence of none of the criteria. Stage III was defined as GLS ≥ -11% and either one or both NT-proBNP and eGFR criteria,

SUBMITTER: Neculae G 

PROVIDER: S-EPMC12719835 | biostudies-literature | 2025 Dec

REPOSITORIES: biostudies-literature