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

AL cardiac amyloidosis cardiac amyloidosis heart failure noninvasive diagnosis transthyretin cardiac amyloidosis

Journal

JACC. Advances
ISSN: 2772-963X
Titre abrégé: JACC Adv
Pays: United States
ID NLM: 9918419284106676

Informations de publication

Date de publication:
Oct 2024
Historique:
received: 20 02 2024
revised: 01 07 2024
accepted: 23 07 2024
medline: 18 9 2024
pubmed: 18 9 2024
entrez: 18 9 2024
Statut: epublish

Résumé

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

Sections du résumé

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

Identifiants

DOI: 10.1016/j.jacadv.2024.101232 PMID: 39290816 PMC: PMC11405895
pubmed: 39290816
doi: 10.1016/j.jacadv.2024.101232
pii: S2772-963X(24)00463-0
pmc: PMC11405895
doi:

Types de publication

Journal Article

Langues

eng

Pagination

101232

Informations de copyright

© 2024 The Authors.

Déclaration de conflit d'intérêts

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.

Auteurs

Giacomo Tini (G)

Department of Clinical and Molecular Medicine, Sapienza University of Rome, Rome, Italy.

Ernesto Cristiano (E)

Department of Electrophysiology, Humanitas Gavazzeni, Bergamo, Italy.

Mattia Zampieri (M)

Cardiomyopathy Unit, Careggi University Hospital, Florence, Italy.
Tuscan Regional Amyloid Center, Careggi University Hospital, Florence, Italy.

Alberto Ponziani (A)

Cardiology Unit, Cardiac Thoracic and Vascular Department, IRCCS Azienda Ospedaliero- Universitaria di Bologna, Bologna, Italy.
Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, Bologna, Italy.

Aldostefano Porcari (A)

European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart-ERN GUARD-Heart.
Centre for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Trieste, Trieste, Italy.
National Amyloidosis Centre, Division of Medicine, University College London, London, UK.

Margherita Zanoletti (M)

Cardiovascular Unit, Department of Internal Medicine, University of Genova, Genova, Italy.

Carlotta Mazzoni (C)

Cardiomyopathy Unit, Careggi University Hospital, Florence, Italy.
Tuscan Regional Amyloid Center, Careggi University Hospital, Florence, Italy.

Matteo Sclafani (M)

Department of Clinical and Molecular Medicine, Sapienza University of Rome, Rome, Italy.

Giulia Saturi (G)

Cardiology Unit, Cardiac Thoracic and Vascular Department, IRCCS Azienda Ospedaliero- Universitaria di Bologna, Bologna, Italy.
Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, Bologna, Italy.

Andrea Lalario (A)

European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart-ERN GUARD-Heart.
Centre for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Trieste, Trieste, Italy.

Marianna Eleonora Labate (ME)

Cardiovascular Unit, IRCCS Ospedale Policlinico San Martino, Genoa, Italy.

Camillo Autore (C)

Department of Clinical and Molecular Medicine, Sapienza University of Rome, Rome, Italy.

Emanuele Barbato (E)

Department of Clinical and Molecular Medicine, Sapienza University of Rome, Rome, Italy.

Federico Perfetto (F)

Tuscan Regional Amyloid Center, Careggi University Hospital, Florence, Italy.

Elena Biagini (E)

Cardiology Unit, Cardiac Thoracic and Vascular Department, IRCCS Azienda Ospedaliero- Universitaria di Bologna, Bologna, Italy.
European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart-ERN GUARD-Heart.

Gianfranco Sinagra (G)

European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart-ERN GUARD-Heart.
Centre for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Trieste, Trieste, Italy.

Marco Canepa (M)

Cardiovascular Unit, Department of Internal Medicine, University of Genova, Genova, Italy.
Cardiovascular Unit, IRCCS Ospedale Policlinico San Martino, Genoa, Italy.

Marco Merlo (M)

European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart-ERN GUARD-Heart.
Centre for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Trieste, Trieste, Italy.

Simone Longhi (S)

Cardiology Unit, Cardiac Thoracic and Vascular Department, IRCCS Azienda Ospedaliero- Universitaria di Bologna, Bologna, Italy.
European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart-ERN GUARD-Heart.

Francesco Cappelli (F)

Cardiomyopathy Unit, Careggi University Hospital, Florence, Italy.
Tuscan Regional Amyloid Center, Careggi University Hospital, Florence, Italy.

Beatrice Musumeci (B)

Department of Clinical and Molecular Medicine, Sapienza University of Rome, Rome, Italy.

Classifications MeSH