Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2023 Aug 14;44(31):2893-2907.
doi: 10.1093/eurheartj/ehad347.

Conventional heart failure therapy in cardiac ATTR amyloidosis

Adam Ioannou  1 , Paolo Massa  1 , Rishi K Patel  1 , Yousuf Razvi  1 , Aldostefano Porcari  1   2 , Muhammad U Rauf  1 , Anita Jiang  1 , Giacomo Cabras  1 , Stefano Filisetti  3 , Roos E Bolhuis  1 , Francesco Bandera  3 , Lucia Venneri  1 , Ana Martinez-Naharro  1 , Steven Law  1 , Tushar Kotecha  1 , Ruta Virsinskaite  1 , Daniel S Knight  1 , Michele Emdin  4   5 , Aviva Petrie  6 , Helen Lachmann  1 , Ashutosh Wechelakar  1 , Mark Petrie  7 , Alun Hughes  8 , Nick Freemantle  9 , Philip N Hawkins  1 , Carol Whelan  1 , John J V McMurray  7 , Julian D Gillmore  1 , Marianna Fontana  1
Affiliations

Conventional heart failure therapy in cardiac ATTR amyloidosis

Adam Ioannou et al. Eur Heart J. .

Erratum in

Abstract

Aims: The aims of this study were to assess prescription patterns, dosages, discontinuation rates, and association with prognosis of conventional heart failure medications in patients with transthyretin cardiac amyloidosis (ATTR-CA).

Methods and results: A retrospective analysis of all consecutive patients diagnosed with ATTR-CA at the National Amyloidosis Centre between 2000 and 2022 identified 2371 patients with ATTR-CA. Prescription of heart failure medications was greater among patients with a more severe cardiac phenotype, comprising beta-blockers in 55.4%, angiotensin-converting enzyme inhibitors (ACEis)/angiotensin II receptor blockers (ARBs) in 57.4%, and mineralocorticoid receptor antagonists (MRAs) in 39.0% of cases. During a median follow-up of 27.8 months (interquartile range 10.6-51.3), 21.7% had beta-blockers discontinued, and 32.9% had ACEi/ARBs discontinued. In contrast, only 7.5% had MRAs discontinued. A propensity score-matched analysis demonstrated that treatment with MRAs was independently associated with a reduced risk of mortality in the overall population [hazard ratio (HR) 0.77 (95% confidence interval (CI) 0.66-0.89), P < .001] and in a pre-specified subgroup of patients with a left ventricular ejection fraction (LVEF) >40% [HR 0.75 (95% CI 0.63-0.90), P = .002]; and treatment with low-dose beta-blockers was independently associated with a reduced risk of mortality in a pre-specified subgroup of patients with a LVEF ≤40% [HR 0.61 (95% CI 0.45-0.83), P = .002]. No convincing differences were found for treatment with ACEi/ARBs.

Conclusion: Conventional heart failure medications are currently not widely prescribed in ATTR-CA, and those that received medication had more severe cardiac disease. Beta-blockers and ACEi/ARBs were often discontinued, but low-dose beta-blockers were associated with reduced risk of mortality in patients with a LVEF ≤40%. In contrast, MRAs were rarely discontinued and were associated with reduced risk of mortality in the overall population; but these findings require confirmation in prospective randomized controlled trials.

Keywords: Beta-blockers; Cardiac ATTR amyloidosis; Heart failure; Heart failure medications; Mineralocorticoid receptor antagonists.

PubMed Disclaimer

Figures

Structured Graphical Abstract
Structured Graphical Abstract
Discontinuation rates of heart failure medications in patients with cardiac ATTR amyloidosis. Kaplan–Meier curves comparing survival in patients treated with heart failure medications to propensity score-matched patients not treated with heart failure medications, followed by a Cox proportional hazards regression analysis. ACEi, angiotensin-converting enzyme inhibitor; ARBs, angiotensin II receptor blockers; MRAs, mineralocorticoid receptor antagonists; LVEF, left ventricular ejection fraction; HR, hazard ratio; CI, confidence interval.
Figure 1
Figure 1
Kaplan–Meier curves comparing survival in patients treated with beta-blockers to patients not treated with beta-blockers followed by a Cox proportional hazards regression analysis: (A) treatment with beta-blockers vs. no treatment with beta-blockers in the overall population, (B) treatment with beta-blockers vs. no treatment with beta-blockers in patients with a LVEF ≤40%, (C) treatment with beta-blockers vs. no treatment with beta-blockers in patients with a LVEF >40%
Figure 2
Figure 2
Kaplan–Meier curves comparing survival in patients treated with ACEi/ARBs to patients not treated with ACEi/ARBs followed by a Cox proportional hazards regression analysis: (A) treatment with ACEi/ARBs vs. no treatment with ACEi/ARBs in the overall population, (B) treatment with ACEi/ARBs vs. no treatment with ACEi/ARBs in patients with a LVEF ≤40%, (C) treatment with ACEi/ARBs vs. no treatment with ACEi/ARBs in patients with a LVEF >40%
Figure 3
Figure 3
Kaplan–Meier curves comparing survival in patients treated with MRAs to patients not treated with MRAs followed by a Cox proportional hazards regression analysis: (A) treatment with MRAs vs. no treatment with MRAs in the overall population, (B) treatment with MRAs vs. no treatment with MRAs in patients with a LVEF ≤40%, (C) treatment with MRAs vs. no treatment with MRAs in patients with a LVEF >40%
See this image and copyright information in PMC

Comment in

References

    1. Ruberg FL, Grogan M, Hanna M, Kelly JW, Maurer MS. Transthyretin amyloid cardiomyopathy: JACC state-of-the-art review. J Am Coll Cardiol 2019;73:2872–2891. 10.1016/j.jacc.2019.04.003 - DOI - PMC - PubMed
    1. Ioannou A, Patel RK, Razvi Y, Porcari A, Knight D, Martinez-Naharro A, et al. . Multi-imaging characterization of cardiac phenotype in different types of amyloidosis. JACC Cardiovasc Imaging 2022;16:464–477. 10.1016/j.jcmg.2022.07.008 - DOI - PubMed
    1. Ioannou A, Patel RK, Razvi Y, Porcari A, Sinagra G, Venneri L, et al. . Impact of earlier diagnosis in cardiac ATTR amyloidosis over the course of 20 years. Circulation 2022;146:1657–1670. 10.1161/CIRCULATIONAHA.122.060852 - DOI - PMC - PubMed
    1. Maurer MS, Schwartz JH, Gundapaneni B, Elliott PM, Merlini G, Waddington-Cruz M, et al. . Tafamidis treatment for patients with transthyretin amyloid cardiomyopathy. N Engl J Med 2018;379:1007–1016. 10.1056/NEJMoa1805689 - DOI - PubMed
    1. Kazi DS, Bellows BK, Baron SJ, Shen C, Cohen DJ, Spertus JA, et al. . Cost-effectiveness of tafamidis therapy for transthyretin amyloid cardiomyopathy. Circulation 2020;141:1214–1224. 10.1161/CIRCULATIONAHA.119.045093 - DOI - PMC - PubMed

MeSH terms

Substances