Stratifying Disease Progression in Patients With Cardiac ATTR Amyloidosis

Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved..

BACKGROUND: Transthyretin cardiac amyloidosis (ATTR-CA) is a progressive cardiomyopathy. The clinical course varies among individuals and there are no established measures to assess disease progression.

OBJECTIVES: The goal of this study was to assess the prognostic importance of an increase in N-terminal pro-B-type natriuretic peptide (NT-proBNP) and outpatient diuretic intensification (ODI) as markers of disease progression in a large cohort of patients with ATTR-CA.

METHODS: We evaluated landmark survival analysis based on worsening of NT-proBNP and requirement for ODI between time of diagnosis and a 1-year visit, and subsequent mortality in 2,275 patients with ATTR-CA from 7 specialist centers. The variables were developed in the National Amyloidosis Centre (NAC) cohort (n = 1,598) and validated in the external cohort from the remaining centers (n = 677).

RESULTS: Between baseline and 1-year visits, 551 (34.5%) NAC patients and 204 (30.1%) patients in the external validation cohort experienced NT-proBNP progression (NT-proBNP increase >700 ng/L and >30%), which was associated with mortality (NAC cohort: HR: 1.82; 95% CI: 1.57-2.10; P < 0.001; validation cohort: HR: 1.75; 95% CI: 1.32-2.33; P < 0.001). At 1 year, 451 (28.2%) NAC patients and 301 (44.5%) patients in the external validation cohort experienced ODI, which was associated with mortality (NAC cohort: HR: 1.88; 95% CI: 1.62-2.18; P < 0.001; validation cohort: HR: 2.05; 95% CI: 1.53-2.74; P < 0.001). When compared with patients with a stable NT-proBNP and stable diuretic dose, a higher risk of mortality was observed in those experiencing either NT-proBNP progression or ODI (NAC cohort: HR: 1.93; 95% CI: 1.65-2.27; P < 0.001; validation cohort: HR: 1.94; 95% CI: 1.36-2.77; P < 0.001), and those experiencing both NT-proBNP progression and ODI (NAC cohort: HR: 2.98; 95% CI: 2.42-3.67; P < 0.001; validation cohort: HR: 3.23; 95% CI: 2.17-4.79; P < 0.001).

CONCLUSIONS: NT-proBNP progression and ODI are frequent and consistently associated with an increased risk of mortality. Combining both variables produces a simple, universally applicable model that detects disease progression in ATTR-CA.

Medienart:

E-Artikel

Erscheinungsjahr:

2024

Erschienen:

2024

Enthalten in:

Zur Gesamtaufnahme - year:2024

Enthalten in:

Journal of the American College of Cardiology - (2024) vom: 01. März

Sprache:

Englisch

Beteiligte Personen:

Ioannou, Adam [VerfasserIn]
Cappelli, Francesco [VerfasserIn]
Emdin, Michele [VerfasserIn]
Nitsche, Christian [VerfasserIn]
Longhi, Simone [VerfasserIn]
Masri, Ahmad [VerfasserIn]
Cipriani, Alberto [VerfasserIn]
Zampieri, Mattia [VerfasserIn]
Colio, Federica [VerfasserIn]
Poledniczek, Michael [VerfasserIn]
Porcari, Aldostefano [VerfasserIn]
Razvi, Yousuf [VerfasserIn]
Aimo, Alberto [VerfasserIn]
Vergaro, Giuseppe [VerfasserIn]
De Michieli, Laura [VerfasserIn]
Rauf, Muhammad U [VerfasserIn]
Patel, Rishi K [VerfasserIn]
Villanueva, Eugenia [VerfasserIn]
Lustig, Yael [VerfasserIn]
Venneri, Lucia [VerfasserIn]
Martinez-Naharro, Ana [VerfasserIn]
Lachmann, Helen [VerfasserIn]
Wechalekar, Ashutosh [VerfasserIn]
Whelan, Carol [VerfasserIn]
Petrie, Aviva [VerfasserIn]
Hawkins, Philip N [VerfasserIn]
Solomon, Scott [VerfasserIn]
Gillmore, Julian D [VerfasserIn]
Fontana, Marianna [VerfasserIn]

Links:

Volltext

Themen:

Cardiac ATTR amyloidosis
Disease progression
Journal Article
NT-proBNP
Outpatient diuretic intensification

Anmerkungen:

Date Revised 25.04.2024

published: Print-Electronic

Citation Status Publisher

doi:

10.1016/j.jacc.2023.12.036

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM370202384