Treatment and mortality in mild SSc-PAH

<bold>Background:</bold> The efficacy of treatment for mild pulmonary arterial hypertension (PAH), defined as mean pulmonary arterial pressure (mPAP) 21–24 mmHg and pulmonary vascular resistance (PVR) 2–3 Wood units (WU), is unknown. <bold>Objectives:</bold> To assess the impact of treatment and risk factors on mortality in patients with mild PAH associated with systemic sclerosis (SSc-PAH). <bold>Methods:</bold> We searched the EUSTAR registry for patients with SSc-PAH and an mPAP < 25 mmHg and PVR 2-3 WU (CP122). Upfront therapy was defined as onset of PAH-specific treatment within 3 months of right heart catheterization (RHC). Risk was assessed using COMPERA 2.0. Cox regression was used to assess the impact on mortality. <bold>Results:</bold> Of 118 patients, 30 (25%) died over a median follow-up of 49 months. Thirty percent of the patients received treatment upfront, including 26% with monotherapy and 4% with combination therapy, while 26% had ongoing treatment for Raynaud’s and digital ulcers at the time of RHC. Age, SSc disease duration, 6MWD, and intermediate-high and high-risk profiles were significant predictors of mortality in univariable analyses (Fig. 1A). Adjusted for risk stratification, upfront treatment was associated with a non-significant risk reduction (HR 0.36, 95% CI 0.10, 1.33, p=0.13) (Fig. 1B). <bold>Conclusions:</bold> Mortality is high in SSc-PAH patients with lower hemodynamic thresholds, and risk profiles predict mortality. Our findings suggest that upfront treatment could benefit these patients. <fig><object-id>erj;64/suppl_68/PA4303/F1</object-id><object-id>F1</object-id><object-id>F1</object-id><graphic></graphic></fig>

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Published
2024-09-14
DOI
https://doi.org/10.1183/13993003.congress-2024.pa4303
Primary Topic
Cardiac Valve Diseases and Treatments
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article
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article

Treatment and mortality in mild SSc-PAH

Anna‐Maria Hoffmann‐Vold, Helle Bitter, Ivan Castellvi, Øyvind Molberg et al.
Cardiac Valve Diseases and Treatments
article

Treatment and mortality in mild SSc-PAH

Anna‐Maria Hoffmann‐Vold, Helle Bitter, Ivan Castellvi, Øyvind Molberg, H. J. Bjørkekjær, Yoshiya Tanaka, Alexandra Balbir‐Gurman, Gabriela Riemekasten, Cathrine Brunborg, Sverre Høie, Kaspar Broch, Øyvind Midtvedt, Paolo Airó, Marco Matucci‐Cerinic, Marie‐Elise Truchetet, Cosimo Bruni, Elise Siegert, Oliver Distler, Patricia Esmeralda Carreira, Mickaël Martin, Håvard Fretheim, Carmen Pilar Simeón‐Aznar, Armando Gabrielli, Christopher P. Denton, Eric Hachulla
article en

Abstract

<bold>Background:</bold> The efficacy of treatment for mild pulmonary arterial hypertension (PAH), defined as mean pulmonary arterial pressure (mPAP) 21–24 mmHg and pulmonary vascular resistance (PVR) 2–3 Wood units (WU), is unknown. <bold>Objectives:</bold> To assess the impact of treatment and risk factors on mortality in patients with mild PAH associated with systemic sclerosis (SSc-PAH). <bold>Methods:</bold> We searched the EUSTAR registry for patients with SSc-PAH and an mPAP < 25 mmHg and PVR 2-3 WU (CP122). Upfront therapy was defined as onset of PAH-specific treatment within 3 months of right heart catheterization (RHC). Risk was assessed using COMPERA 2.0. Cox regression was used to assess the impact on mortality. <bold>Results:</bold> Of 118 patients, 30 (25%) died over a median follow-up of 49 months. Thirty percent of the patients received treatment upfront, including 26% with monotherapy and 4% with combination therapy, while 26% had ongoing treatment for Raynaud’s and digital ulcers at the time of RHC. Age, SSc disease duration, 6MWD, and intermediate-high and high-risk profiles were significant predictors of mortality in univariable analyses (Fig. 1A). Adjusted for risk stratification, upfront treatment was associated with a non-significant risk reduction (HR 0.36, 95% CI 0.10, 1.33, p=0.13) (Fig. 1B). <bold>Conclusions:</bold> Mortality is high in SSc-PAH patients with lower hemodynamic thresholds, and risk profiles predict mortality. Our findings suggest that upfront treatment could benefit these patients. <fig><object-id>erj;64/suppl_68/PA4303/F1</object-id><object-id>F1</object-id><object-id>F1</object-id><graphic></graphic></fig>

University of Verona (IT), Marche Polytechnic University (IT), Hebron University (PS), Oslo University Hospital (NO), Good Samaritan Hospital (US), Université de Bordeaux (FR), Vita-Salute San Raffaele University (IT), Inserm (FR), University of Occupational and Environmental Health Japan (JP), Université de Lille (FR), The Royal Free Hospital (GB), Rambam Health Care Campus (IL), Hospital de Sant Pau (ES), Hospital of Southern Norway (NO), Centre Hospitalier Universitaire de Lille (FR), Université de Poitiers (FR), Research Institute Hospital 12 de Octubre (ES), Sørlandet Hospital Arendal (NO), University Hospital of Zurich (CH), Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia (IT), Azienda Ospedaliero-Universitaria Careggi (IT), Rappaport Family Institute for Research in the Medical Sciences (IL), Vall d'Hebron Hospital Universitari (ES), Kerckhoff Klinik (DE), University College London (GB), University of Lübeck (DE)
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Cardiac Valve Diseases and Treatments
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