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
. 2024 Sep 5;3(10):101232.
doi: 10.1016/j.jacadv.2024.101232. eCollection 2024 Oct.

Impact of the Noninvasive Diagnostic Algorithm on Clinical Presentation and Prognosis in Cardiac Amyloidosis

Giacomo Tini  1 , Ernesto Cristiano  2 , Mattia Zampieri  3   4 , Alberto Ponziani  5   6 , Aldostefano Porcari  7   8   9 , Margherita Zanoletti  10 , Carlotta Mazzoni  3   4 , Matteo Sclafani  1 , Giulia Saturi  5   6 , Andrea Lalario  7   8 , Marianna Eleonora Labate  11 , Camillo Autore  1 , Emanuele Barbato  1 , Federico Perfetto  4 , Elena Biagini  5   7 , Gianfranco Sinagra  7   8 , Marco Canepa  10   11 , Marco Merlo  7   8 , Simone Longhi  5   7 , Francesco Cappelli  3   4 , Beatrice Musumeci  1
Affiliations

Impact of the Noninvasive Diagnostic Algorithm on Clinical Presentation and Prognosis in Cardiac Amyloidosis

Giacomo Tini et al. JACC Adv. .

Abstract

Background: The introduction of a noninvasive diagnostic algorithm in 2016 led to increased awareness and recognition of cardiac amyloidosis (CA).

Objectives: The purpose of this study was to analyze the impact of the introduction of the noninvasive diagnostic algorithm on diagnosis and prognosis in a multicenter Italian CA cohort.

Methods: This was a retrospective analysis of 887 CA patients from 5 Italian Cardiomyopathies Referral Centers: 311 light-chain CA, 87 variant transthyretin (TTR)-related CA, 489 wild-type TTR-related CA. Clinical characteristics and outcomes (all-cause mortality and heart failure [HF] hospitalizations) were compared overall and for each CA subtype between patients diagnosed before versus after 2016. Outcomes were further compared by propensity score weighted Kaplan-Meier analysis and Cox regression analysis.

Results: CA diagnoses increased after 2016, in particular for wild-type TTR-related CA. Patients diagnosed after versus before 2016 were older, had less frequently a history of HF prior to diagnosis, and NYHA functional class III-IV at diagnosis. Over a median follow-up of 18 months, 172 (86%) patients diagnosed before 2016 died or had an HF hospitalization, versus 300 (44%) diagnosed after 2016. Propensity score weighted Kaplan-Meier analysis showed worse outcomes (P < 0.001) for patients diagnosed before 2016. At Cox regression analysis, CA diagnosis after 2016 was an independent protective factor for the composite outcome (HR: 0.69; P = 0.001), with interaction by CA subtype (significant in TTR-related CA and null in light-chain).

Conclusions: CA patients diagnosed after 2016 showed a less severe phenotype and a better prognosis. The impact of the noninvasive diagnostic algorithm on outcomes was particularly relevant in TTR-related CA.

Keywords: AL cardiac amyloidosis; cardiac amyloidosis; heart failure; noninvasive diagnosis; transthyretin cardiac amyloidosis.

PubMed Disclaimer

Conflict of interest statement

The work reported in this publication was funded by the 10.13039/501100003196Italian Ministry of Health, RC-2022-2773270 project. The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Figures

None
Graphical abstract
Figure 1
Figure 1
Number of CA Diagnoses Over Time, in the Overall Cohort and for Each CA Subtype AL = light-chain; ATTRv = variant transthyretin-related CA; ATTRwt = wild-type transthyretin-related CA; CA = cardiac amyloidosis.
Figure 2
Figure 2
Kaplan Meier Curves for the Composite Outcome Kaplan-Meier curves for the composite outcome in the overall CA cohort before (A) and after (B) PSW, in the AL group before (C) and after (D) PSW, and in the TTR group before (E) and after (F) PSW. PSW = propensity score weighted; TTR = transthyretin; other abbreviations as in Figure 1.
Central Illustration
Central Illustration
Impact of the Noninvasive Diagnostic Algorithm on Clinical Presentation and Prognosis in Light-Chain and Transthyretin-Related Cardiac Amyloidosis Evolving landscape of CA after the introduction of the noninvasive diagnostic algorithm: Increased number of diagnoses, less severe phenotype at diagnosis, better short-term prognosis. The impact of the noninvasive diagnostic algorithm onto the TTR-related CA group diagnoses and features influenced the AL group as well with a “Domino-Effect”. HF = heart failure; IVS = interventricular septum; LVEF = left ventricular ejection fraction; other abbreviations as in Figures 1 and 2.
See this image and copyright information in PMC

References

    1. Gilstrap L.G., Dominici F., Wang Y., et al. Epidemiology of cardiac amyloidosis-associated heart failure hospitalizations among fee-for-service medicare beneficiaries in the United States. Circ Heart Fail. 2019;12 - PMC - PubMed
    1. Ioannou A., Patel R.K., Razvi Y., et al. Impact of earlier diagnosis in cardiac ATTR amyloidosis over the course of 20 years. Circulation. 2022;146:1657–1670. doi: 10.1161/CIRCULATIONAHA.122.060852. - DOI - PMC - PubMed
    1. Gillmore J.D., Maurer M.S., Falk R.H., et al. Nonbiopsy diagnosis of cardiac transthyretin amyloidosis. Circulation. 2016;133:2404–2412. - PubMed
    1. Griffin J.M., Rosenblum H., Maurer M.S. Pathophysiology and therapeutic approaches to cardiac amyloidosis. Circ Res. 2021;128:1554–1575. - PMC - PubMed
    1. Barrett C.D., Dobos K., Liedtke M., et al. A changing landscape of mortality for systemic light chain amyloidosis. JACC Heart Fail. 2019;7:958–966. - PubMed

LinkOut - more resources