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Published in: Respiratory Research 1/2022

Open Access 01-12-2022 | Systemic Sclerosis | Research

Mild pulmonary hemodynamic alterations in patients with systemic sclerosis: relevance of the new 2022 ESC/ERS definition of pulmonary hypertension and impact on mortality

Authors: Sébastien Puigrenier, Jonathan Giovannelli, Nicolas Lamblin, Pascal De Groote, Marie Fertin, Jean-François Bervar, Antoine Lamer, Jean-Louis Edmé, Marie-Hélène Balquet, Vincent Sobanski, David Launay, Éric Hachulla, Sébastien Sanges

Published in: Respiratory Research | Issue 1/2022

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Abstract

Background and objective

The definition of pre-capillary pulmonary hypertension (PH) has been modified, with lowering of the mean pulmonary arterial pressure (mPAP) threshold from 25 to 20 mmHg and addition of a mandatory criterion of pulmonary vascular resistance (PVR) ≥ 2 Wood units (WU). Our objectives were: 1/ to estimate the proportion of patients reclassified as having pre-capillary PH when using the new 2022 ESC/ERS hemodynamic criteria (i.e. mPAP 21-24 mmHg and PVR ≥ 2 WU), and to describe their clinical characteristics and outcome; and 2/ to study the relationship between PVR and survival in patients with mPAP > 20 mmHg.

Methods

We retrospectively analyzed consecutive SSc patients included in our National Reference Center for a first right-heart catheterization between 2003 and 2018. The association between survival and PVR was studied using smoothing splines.

Results

We included 126 SSc patients with mPAP > 20 mmHg. Among them, 16 (13%) had a baseline mPAP value between 21 and 24 mmHg and PVR ≥ 2 mmHg and were reclassified as pre-capillary PH; 10 of which (62%) raised their mPAP ≥ 25 mmHg during follow-up. In patients with mPAP > 20 mmHg, we observed a linear relation between PVR and mortality for values < 6 WU.

Conclusion

A significant proportion of SSc patients is reclassified as having pre-capillary PH with the new 2022 ESC/ERS hemodynamic definition. Lowering the PVR threshold from 3 to 2 WU captures patients at risk of raising their mPAP > 25 mmHg, with a possibly less severe disease.
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Literature
1.
go back to reference Sobanski V, Launay D, Hachulla E, Humbert M. Current approaches to the treatment of systemic-sclerosis-associated pulmonary arterial hypertension (SSc-PAH). Curr Rheumatol Rep. 2016;18(2):10.CrossRef Sobanski V, Launay D, Hachulla E, Humbert M. Current approaches to the treatment of systemic-sclerosis-associated pulmonary arterial hypertension (SSc-PAH). Curr Rheumatol Rep. 2016;18(2):10.CrossRef
2.
go back to reference Galiè N, Humbert M, Vachiery J-L, Gibbs S, Lang I, Torbicki A, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016;37(1):67–119.CrossRef Galiè N, Humbert M, Vachiery J-L, Gibbs S, Lang I, Torbicki A, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016;37(1):67–119.CrossRef
3.
go back to reference Tyndall AJ, Bannert B, Vonk M, Airò P, Cozzi F, Carreira PE, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010;69(10):1809–15.CrossRef Tyndall AJ, Bannert B, Vonk M, Airò P, Cozzi F, Carreira PE, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010;69(10):1809–15.CrossRef
4.
go back to reference Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RMF, Brida M, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. European Heart J. 2022;89:7. Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RMF, Brida M, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. European Heart J. 2022;89:7.
5.
go back to reference Jaafar S, Visovatti S, Young A, Huang S, Cronin P, Vummidi D, et al. Impact of the revised haemodynamic definition on the diagnosis of pulmonary hypertension in patients with systemic sclerosis. Eur Respir J. 2019;54(2):1900586.CrossRef Jaafar S, Visovatti S, Young A, Huang S, Cronin P, Vummidi D, et al. Impact of the revised haemodynamic definition on the diagnosis of pulmonary hypertension in patients with systemic sclerosis. Eur Respir J. 2019;54(2):1900586.CrossRef
6.
go back to reference Xanthouli P, Jordan S, Milde N, Marra A, Blank N, Egenlauf B, et al. Haemodynamic phenotypes and survival in patients with systemic sclerosis: the impact of the new definition of pulmonary arterial hypertension. Ann Rheum Dis. 2020;79(3):370–8.CrossRef Xanthouli P, Jordan S, Milde N, Marra A, Blank N, Egenlauf B, et al. Haemodynamic phenotypes and survival in patients with systemic sclerosis: the impact of the new definition of pulmonary arterial hypertension. Ann Rheum Dis. 2020;79(3):370–8.CrossRef
7.
go back to reference Sarı A, Şener YZ, Armağan B, Kılıç L, Kaya EB, Hekimsoy V, et al. How did the updated hemodynamic definitions affect the frequency of pulmonary hypertension in patients with systemic sclerosis. Anatol J Cardiol. 2021;25(1):30–5.PubMed Sarı A, Şener YZ, Armağan B, Kılıç L, Kaya EB, Hekimsoy V, et al. How did the updated hemodynamic definitions affect the frequency of pulmonary hypertension in patients with systemic sclerosis. Anatol J Cardiol. 2021;25(1):30–5.PubMed
8.
go back to reference Simonneau G, Montani D, Celermajer DS, Denton CP, Gatzoulis MA, Krowka M, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53:1.CrossRef Simonneau G, Montani D, Celermajer DS, Denton CP, Gatzoulis MA, Krowka M, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53:1.CrossRef
9.
go back to reference van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. Classification Criteria for Systemic Sclerosis: An ACR-EULAR Collaborative Initiative. Arthritis Rheum. 2013;65(11):2737–47.PubMedPubMedCentral van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. Classification Criteria for Systemic Sclerosis: An ACR-EULAR Collaborative Initiative. Arthritis Rheum. 2013;65(11):2737–47.PubMedPubMedCentral
10.
go back to reference Vachiéry J, Tedford RJ, Rosenkranz S, Palazzini M, Lang I, Guazzi M, et al. Pulmonary hypertension due to left heart disease. Eur Respir J. 2019;53(1):1801897.CrossRef Vachiéry J, Tedford RJ, Rosenkranz S, Palazzini M, Lang I, Guazzi M, et al. Pulmonary hypertension due to left heart disease. Eur Respir J. 2019;53(1):1801897.CrossRef
11.
go back to reference Coghlan JG, Wolf M, Distler O, Denton CP, Doelberg M, Harutyunova S, et al. Incidence of pulmonary hypertension and determining factors in patients with systemic sclerosis. Eur Respir J. 2018;51(4):1701197.CrossRef Coghlan JG, Wolf M, Distler O, Denton CP, Doelberg M, Harutyunova S, et al. Incidence of pulmonary hypertension and determining factors in patients with systemic sclerosis. Eur Respir J. 2018;51(4):1701197.CrossRef
12.
go back to reference Maron BA, Hess E, Maddox TM, Opotowsky AR, Tedford RJ, Lahm T, et al. Association of borderline pulmonary hypertension with mortality and hospitalization in a large patient cohort: insights from the veterans affairs clinical assessment, reporting, and tracking program. Circulation. 2016;133(13):1240–8.CrossRef Maron BA, Hess E, Maddox TM, Opotowsky AR, Tedford RJ, Lahm T, et al. Association of borderline pulmonary hypertension with mortality and hospitalization in a large patient cohort: insights from the veterans affairs clinical assessment, reporting, and tracking program. Circulation. 2016;133(13):1240–8.CrossRef
13.
go back to reference Pan Z, Marra AM, Benjamin N, et al. Early treatment with ambrisentan of mildly elevated mean pulmonary arterial pressure associated with systemic sclerosis: a randomized, controlled, double-blind, parallel group study (EDITA study). Arthritis Res Ther. 2019;21:217.CrossRef Pan Z, Marra AM, Benjamin N, et al. Early treatment with ambrisentan of mildly elevated mean pulmonary arterial pressure associated with systemic sclerosis: a randomized, controlled, double-blind, parallel group study (EDITA study). Arthritis Res Ther. 2019;21:217.CrossRef
14.
go back to reference Maron BA, Brittain EL, Hess E, Waldo SW, Barón AE, Huang S, et al. Pulmonary vascular resistance and clinical outcomes in patients with pulmonary hypertension: a retrospective cohort study. Lancet. 2022;67:34. Maron BA, Brittain EL, Hess E, Waldo SW, Barón AE, Huang S, et al. Pulmonary vascular resistance and clinical outcomes in patients with pulmonary hypertension: a retrospective cohort study. Lancet. 2022;67:34.
15.
go back to reference Yamamoto K, Tanabe N, Takahashi Y, Naito A, Sekine A, Suda R, et al. Characteristics of patients meeting the new definition of pre-capillary pulmonary hypertension (Nice 2018) in a single Japanese pulmonary hypertension center. BMC Pulm Med. 2021;21:1.CrossRef Yamamoto K, Tanabe N, Takahashi Y, Naito A, Sekine A, Suda R, et al. Characteristics of patients meeting the new definition of pre-capillary pulmonary hypertension (Nice 2018) in a single Japanese pulmonary hypertension center. BMC Pulm Med. 2021;21:1.CrossRef
16.
go back to reference Corte TJ, Wort SJ, Gatzoulis MA, Macdonald P, Hansell DM, Wells AU. Pulmonary vascular resistance predicts early mortality in patients with diffuse fibrotic lung disease and suspected pulmonary hypertension. Thorax. 2009;64(10):883–8.CrossRef Corte TJ, Wort SJ, Gatzoulis MA, Macdonald P, Hansell DM, Wells AU. Pulmonary vascular resistance predicts early mortality in patients with diffuse fibrotic lung disease and suspected pulmonary hypertension. Thorax. 2009;64(10):883–8.CrossRef
17.
go back to reference Chung L, Domsic RT, Lingala B, Alkassab F, Bolster M, Csuka ME, et al. Survival and predictors of mortality in systemic sclerosis-associated pulmonary arterial hypertension: outcomes from the pulmonary hypertension assessment and recognition of outcomes in scleroderma registry. Arthritis Care Res (Hoboken). 2014;66(3):489–95.CrossRef Chung L, Domsic RT, Lingala B, Alkassab F, Bolster M, Csuka ME, et al. Survival and predictors of mortality in systemic sclerosis-associated pulmonary arterial hypertension: outcomes from the pulmonary hypertension assessment and recognition of outcomes in scleroderma registry. Arthritis Care Res (Hoboken). 2014;66(3):489–95.CrossRef
18.
go back to reference Lefèvre G, Dauchet L, Hachulla E, Montani D, Sobanski V, Lambert M, et al. Survival and prognostic factors in systemic sclerosis-associated pulmonary hypertension: a systematic review and meta-analysis. Arthritis Rheum. 2013;65(9):2412–23.CrossRef Lefèvre G, Dauchet L, Hachulla E, Montani D, Sobanski V, Lambert M, et al. Survival and prognostic factors in systemic sclerosis-associated pulmonary hypertension: a systematic review and meta-analysis. Arthritis Rheum. 2013;65(9):2412–23.CrossRef
19.
go back to reference Fisher MR, Mathai SC, Champion HC, Girgis RE, Housten-Harris T, Hummers L, et al. Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum. 2006;54(9):3043–50.CrossRef Fisher MR, Mathai SC, Champion HC, Girgis RE, Housten-Harris T, Hummers L, et al. Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum. 2006;54(9):3043–50.CrossRef
20.
go back to reference Ramjug S, Hussain N, Hurdman J, Billings C, Charalampopoulos A, Elliot CA, et al. Idiopathic and systemic sclerosis-associated pulmonary arterial hypertension: a comparison of demographic, hemodynamic, and mri characteristics and outcomes. Chest. 2017;152(1):92–102.CrossRef Ramjug S, Hussain N, Hurdman J, Billings C, Charalampopoulos A, Elliot CA, et al. Idiopathic and systemic sclerosis-associated pulmonary arterial hypertension: a comparison of demographic, hemodynamic, and mri characteristics and outcomes. Chest. 2017;152(1):92–102.CrossRef
21.
go back to reference Ratwatte S, Anderson J, Strange G, Corrigan C, Collins N, Celermajer DS, et al. Pulmonary arterial hypertension with below threshold pulmonary vascular resistance. Eur Respir J. 2020;56(1):1901654.CrossRef Ratwatte S, Anderson J, Strange G, Corrigan C, Collins N, Celermajer DS, et al. Pulmonary arterial hypertension with below threshold pulmonary vascular resistance. Eur Respir J. 2020;56(1):1901654.CrossRef
Metadata
Title
Mild pulmonary hemodynamic alterations in patients with systemic sclerosis: relevance of the new 2022 ESC/ERS definition of pulmonary hypertension and impact on mortality
Authors
Sébastien Puigrenier
Jonathan Giovannelli
Nicolas Lamblin
Pascal De Groote
Marie Fertin
Jean-François Bervar
Antoine Lamer
Jean-Louis Edmé
Marie-Hélène Balquet
Vincent Sobanski
David Launay
Éric Hachulla
Sébastien Sanges
Publication date
01-12-2022
Publisher
BioMed Central
Published in
Respiratory Research / Issue 1/2022
Electronic ISSN: 1465-993X
DOI
https://doi.org/10.1186/s12931-022-02205-4

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