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Long-term impact of add-on sequential triple combination therapy in pulmonary arterial hypertension: real world experience

BACKGROUND: Sequential triple combination therapy is recommended for pulmonary arterial hypertension (PAH) patients who are not at therapeutic goal on dual therapy, but long-term data on efficacy and safety is scarce. OBJECTIVE: To assess the long-term impact of sequential triple combination therapy...

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Autores principales: Deshwal, Himanshu, Weinstein, Tatiana, Salyer, Rachel, Thompson, Jesse, Cefali, Frank, Fenton, Rebecca, Bondarsky, Eric, Sulica, Roxana
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10557422/
https://www.ncbi.nlm.nih.gov/pubmed/37795626
http://dx.doi.org/10.1177/17534666231199693
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author Deshwal, Himanshu
Weinstein, Tatiana
Salyer, Rachel
Thompson, Jesse
Cefali, Frank
Fenton, Rebecca
Bondarsky, Eric
Sulica, Roxana
author_facet Deshwal, Himanshu
Weinstein, Tatiana
Salyer, Rachel
Thompson, Jesse
Cefali, Frank
Fenton, Rebecca
Bondarsky, Eric
Sulica, Roxana
author_sort Deshwal, Himanshu
collection PubMed
description BACKGROUND: Sequential triple combination therapy is recommended for pulmonary arterial hypertension (PAH) patients who are not at therapeutic goal on dual therapy, but long-term data on efficacy and safety is scarce. OBJECTIVE: To assess the long-term impact of sequential triple combination therapy in patients with PAH who are not at goal on dual combination therapy. STUDY DESIGN AND METHODS: We performed a retrospective observational study in a racially/ethnically diverse cohort of consecutive PAH patients on a stable dual therapy regimen who remained in intermediate- or high-risk category and were subsequently initiated on sequential triple combination therapy. We studied interval change in functional, echocardiographic, and hemodynamic parameters, REVEAL 2.0 risk category and ERS/ESC 2022 simplified four-strata risk category. Multivariate logistic regression analysis was performed to identify independent predictors of successful risk reduction (achievement or maintenance of REVEAL 2.0 low-risk category). Kaplan–Meier survival curves were created to assess the effect of risk reduction on survival. RESULTS: Out of 414 PAH patients seen in our program, 55 patients received add-on sequential triple combination regimen and had follow-up hemodynamic data. The mean age was 57 years, with 85% women. The most common etiology of PAH was idiopathic/heritable (41.8%). Most patients were WHO functional class III (76.4%), and 34.5% of patients were in high-risk category (REVEAL 2.0). On a median follow-up of 68 weeks, there was a significant improvement in WHO Functional Class (p < 0.001), six-minute walk distance (35 m) with 61.8% of patients achieving low-risk status by REVEAL 2.0, and a 28% of patients’ improvement in pulmonary vascular resistance. Female gender was identified as a strong predictor of successful risk reduction, whereas Hispanic ethnicity estimated right atrial pressure on echocardiogram and pericardial effusion predicted lower probability of risk reduction. Patients who achieved or maintained low-risk status had significantly improved survival. CONCLUSION: Add-on sequential triple combination therapy significantly increased functional, echocardiographic, and hemodynamic parameters with improvement in risk category and survival.
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spelling pubmed-105574222023-10-07 Long-term impact of add-on sequential triple combination therapy in pulmonary arterial hypertension: real world experience Deshwal, Himanshu Weinstein, Tatiana Salyer, Rachel Thompson, Jesse Cefali, Frank Fenton, Rebecca Bondarsky, Eric Sulica, Roxana Ther Adv Respir Dis Original Research BACKGROUND: Sequential triple combination therapy is recommended for pulmonary arterial hypertension (PAH) patients who are not at therapeutic goal on dual therapy, but long-term data on efficacy and safety is scarce. OBJECTIVE: To assess the long-term impact of sequential triple combination therapy in patients with PAH who are not at goal on dual combination therapy. STUDY DESIGN AND METHODS: We performed a retrospective observational study in a racially/ethnically diverse cohort of consecutive PAH patients on a stable dual therapy regimen who remained in intermediate- or high-risk category and were subsequently initiated on sequential triple combination therapy. We studied interval change in functional, echocardiographic, and hemodynamic parameters, REVEAL 2.0 risk category and ERS/ESC 2022 simplified four-strata risk category. Multivariate logistic regression analysis was performed to identify independent predictors of successful risk reduction (achievement or maintenance of REVEAL 2.0 low-risk category). Kaplan–Meier survival curves were created to assess the effect of risk reduction on survival. RESULTS: Out of 414 PAH patients seen in our program, 55 patients received add-on sequential triple combination regimen and had follow-up hemodynamic data. The mean age was 57 years, with 85% women. The most common etiology of PAH was idiopathic/heritable (41.8%). Most patients were WHO functional class III (76.4%), and 34.5% of patients were in high-risk category (REVEAL 2.0). On a median follow-up of 68 weeks, there was a significant improvement in WHO Functional Class (p < 0.001), six-minute walk distance (35 m) with 61.8% of patients achieving low-risk status by REVEAL 2.0, and a 28% of patients’ improvement in pulmonary vascular resistance. Female gender was identified as a strong predictor of successful risk reduction, whereas Hispanic ethnicity estimated right atrial pressure on echocardiogram and pericardial effusion predicted lower probability of risk reduction. Patients who achieved or maintained low-risk status had significantly improved survival. CONCLUSION: Add-on sequential triple combination therapy significantly increased functional, echocardiographic, and hemodynamic parameters with improvement in risk category and survival. SAGE Publications 2023-10-05 /pmc/articles/PMC10557422/ /pubmed/37795626 http://dx.doi.org/10.1177/17534666231199693 Text en © The Author(s), 2023 https://creativecommons.org/licenses/by-nc-nd/4.0/This article is distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 License (https://creativecommons.org/licenses/by-nc-nd/4.0/) which permits non-commercial use, reproduction and distribution of the work as published without adaptation or alteration, without further permission provided the original work is attributed as specified on the SAGE and Open Access page (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Original Research
Deshwal, Himanshu
Weinstein, Tatiana
Salyer, Rachel
Thompson, Jesse
Cefali, Frank
Fenton, Rebecca
Bondarsky, Eric
Sulica, Roxana
Long-term impact of add-on sequential triple combination therapy in pulmonary arterial hypertension: real world experience
title Long-term impact of add-on sequential triple combination therapy in pulmonary arterial hypertension: real world experience
title_full Long-term impact of add-on sequential triple combination therapy in pulmonary arterial hypertension: real world experience
title_fullStr Long-term impact of add-on sequential triple combination therapy in pulmonary arterial hypertension: real world experience
title_full_unstemmed Long-term impact of add-on sequential triple combination therapy in pulmonary arterial hypertension: real world experience
title_short Long-term impact of add-on sequential triple combination therapy in pulmonary arterial hypertension: real world experience
title_sort long-term impact of add-on sequential triple combination therapy in pulmonary arterial hypertension: real world experience
topic Original Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10557422/
https://www.ncbi.nlm.nih.gov/pubmed/37795626
http://dx.doi.org/10.1177/17534666231199693
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