A Hemodynamic Echocardiographic Approach to Disease Severity and Prognosis in Transthyretin Amyloid Cardiomyopathy
7 affiliations
- 1Cardiology Unit, Azienda Ospedaliera Universitaria di Ferrara, Ferrara, Italy
- 2Centre for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Trieste, Trieste, Italy
- 3European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart-ERN GUARD-Heart
- 4National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Hospital, London, United Kingdom
- 5Department of Cardio-Thoraco-Vascular Sciences and Public Health, University of Padua, Padua, Italy
- 6Cardiology Unit, University Hospital of Padua, Padua, Italy
- 7Cardiology, ASST Spedali Civili and Department of Medical and Surgical Specialties, Radiological Sciences, and Public Health, University of Brescia, Brescia, Italy
Abstract
Background: Transthyretin amyloid cardiomyopathy (ATTR-CM) causes heart failure and poor outcomes. Prognostic stratification is fundamental, yet validated staging systems rely exclusively on biomarkers, neglecting structural and functional cardiac parameters.
Objectives: This study evaluates a noninvasive hemodynamic echocardiographic approach to stratify patients with ATTR-CM.
Methods: Patients were categorized into 3 groups based on stroke volume index (SVi) and systolic pulmonary artery pressure (sPAP), markers of forward flow and elevated filling pressures. We compared receiver operating characteristic curves for various potentially predictive hemodynamic parameters to identify these as the most robust candidates for risk stratification. Optimal cutoffs (30 mL/m2 for SVi and 40 mm Hg for sPAP) were determined using the Liu index, supported by both univariable and multivariable survival analyses. The primary endpoint was the composite of all-cause mortality and heart failure hospitalization. Prognostic utility was evaluated using multivariable Cox regression, adjusted for age, sex, and disease-modifying therapy.
Results: The cohort included 293 patients with ATTR-CM: group 1 (normal SVi/sPAP, n = 115 [39%]), group 2 (low SVi or high sPAP, n = 129), and group 3 (low SVi/high sPAP, n = 49). The primary outcome occurred in 108 (37%) patients. The multivariable analysis confirmed the staging system as an independent predictor of the composite outcome (group 2 HR: 1.70, 95% CI: 1.05-2.71; group 3 HR: 2.50, 95% CI: 1.44-4.30; P < 0.001).
Conclusions: The noninvasive hemodynamic approach provides an alternative and promising method to stratify the risk of clinical outcome in patients with ATTR-CM, potentially improving management strategies.
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BibTeX
@article{serenelli2026hemodynamic,
title = {{A Hemodynamic Echocardiographic Approach to Disease Severity and Prognosis in Transthyretin Amyloid Cardiomyopathy}},
author = {Matteo Serenelli and Anna Cantone and Aldostefano Porcari and Marco Pozzan and Marco Merlo and Gianfranco Sinagra and Gabriele Guidi Colombi and Beatrice Dal Passo and Federico Sanguettoli and Matteo Arzenton and Daniele Maio and Martina Vitali and Giulia Casolari and Jacopo Bonini and Alberto Cipriani and Laura De Michieli and Donato Mele and Giulio Sinigiani and Daniela Tomasoni and Marco Metra and Rita Pavasini and Simone Biscaglia and Gianluca Campo},
journal = {JACC: Advances},
year = {2026},
month = sep,
doi = {10.1016/j.jacadv.2026.103244},
url = {https://doi.org/10.1016/j.jacadv.2026.103244},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - A Hemodynamic Echocardiographic Approach to Disease Severity and Prognosis in Transthyretin Amyloid Cardiomyopathy
AU - Matteo Serenelli
AU - Anna Cantone
AU - Aldostefano Porcari
AU - Marco Pozzan
AU - Marco Merlo
AU - Gianfranco Sinagra
AU - Gabriele Guidi Colombi
AU - Beatrice Dal Passo
AU - Federico Sanguettoli
AU - Matteo Arzenton
AU - Daniele Maio
AU - Martina Vitali
AU - Giulia Casolari
AU - Jacopo Bonini
AU - Alberto Cipriani
AU - Laura De Michieli
AU - Donato Mele
AU - Giulio Sinigiani
AU - Daniela Tomasoni
AU - Marco Metra
AU - Rita Pavasini
AU - Simone Biscaglia
AU - Gianluca Campo
PY - 2026
DA - 2026/09/30
JO - JACC: Advances
DO - 10.1016/j.jacadv.2026.103244
UR - https://doi.org/10.1016/j.jacadv.2026.103244
AB - Background: Transthyretin amyloid cardiomyopathy (ATTR-CM) causes heart failure and poor outcomes. Prognostic stratification is fundamental, yet validated staging systems rely exclusively on biomarkers, neglecting structural and functional cardiac parameters. Objectives: This study evaluates a noninvasive hemodynamic echocardiographic approach to stratify patients with ATTR-CM. Methods: Patients were categorized into 3 groups based on stroke volume index (SVi) and systolic pulmonary artery pressure (sPAP), markers of forward flow and elevated filling pressures. We compared receiver operating characteristic curves for various potentially predictive hemodynamic parameters to identify these as the most robust candidates for risk stratification. Optimal cutoffs (30 mL/m2 for SVi and 40 mm Hg for sPAP) were determined using the Liu index, supported by both univariable and multivariable survival analyses. The primary endpoint was the composite of all-cause mortality and heart failure hospitalization. Prognostic utility was evaluated using multivariable Cox regression, adjusted for age, sex, and disease-modifying therapy. Results: The cohort included 293 patients with ATTR-CM: group 1 (normal SVi/sPAP, n = 115 [39%]), group 2 (low SVi or high sPAP, n = 129), and group 3 (low SVi/high sPAP, n = 49). The primary outcome occurred in 108 (37%) patients. The multivariable analysis confirmed the staging system as an independent predictor of the composite outcome (group 2 HR: 1.70, 95% CI: 1.05-2.71; group 3 HR: 2.50, 95% CI: 1.44-4.30; P < 0.001). Conclusions: The noninvasive hemodynamic approach provides an alternative and promising method to stratify the risk of clinical outcome in patients with ATTR-CM, potentially improving management strategies.
SN - 2772-963X
ER -