S. Hatano, T. Strasser, and W. H. Organization, Primary pulmonary hypertension : report on a WHO meeting, pp.15-17, 1973.

D. Sur, Report of an expert committee, World Health Organ Tech Rep Ser, vol.213, p.35, 1961.

N. Galiè, M. Humbert, J. Vachiery, S. Gibbs, I. Lang et al., 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. 1 janv, vol.37, issue.1, pp.67-119, 2015.

G. Simonneau, D. Montani, D. S. Celermajer, C. P. Denton, M. A. Gatzoulis et al., Haemodynamic definitions and updated clinical classification of pulmonary hypertension, Eur Respir J. janv, vol.53, issue.1, 2019.

G. Kovacs, A. Berghold, S. Scheidl, and H. Olschewski, Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review, Eur Respir J, vol.34, issue.4, pp.888-94, 2009.

J. G. Coghlan, M. Wolf, O. Distler, C. P. Denton, M. Doelberg et al., Incidence of pulmonary hypertension and determining factors in patients with systemic sclerosis, Eur Respir J, vol.51, issue.4, 2018.

P. Douschan, G. Kovacs, A. Avian, V. Foris, F. Gruber et al., Mild Elevation of Pulmonary Arterial Pressure as a Predictor of Mortality, Am J Respir Crit Care Med, vol.15, issue.4, pp.509-525, 2018.

J. Vachiéry, R. J. Tedford, S. Rosenkranz, M. Palazzini, I. Lang et al., Pulmonary hypertension due to left heart disease, Eur Respir J. janv, vol.53, issue.1, 2019.

J. Vachiéry, Y. Adir, J. A. Barberà, H. Champion, J. G. Coghlan et al.,

, Pulmonary hypertension due to left heart diseases, J Am Coll Cardiol. 24 déc, vol.62, pp.100-108, 1925.

E. Lau, E. Giannoulatou, D. S. Celermajer, and M. Humbert, Epidemiology and treatment of pulmonary arterial hypertension, Nature Reviews Cardiology, vol.14, issue.10, pp.603-617, 2017.

M. Humbert, O. Sitbon, A. Chaouat, M. Bertocchi, G. Habib et al., Pulmonary arterial hypertension in France: results from a national registry, Am J Respir Crit Care Med. 1 mai, vol.173, issue.9, pp.1023-1053, 2006.

D. B. Badesch, G. E. Raskob, C. G. Elliott, A. M. Krichman, H. W. Farber et al., Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry, Chest. févr, vol.137, issue.2, pp.376-87, 2010.

T. Thenappan, S. J. Shah, S. Rich, and M. Gomberg-maitland, A USA-based registry for pulmonary arterial hypertension: 1982-2006, Eur Respir J. déc, vol.30, issue.6, pp.1103-1113, 2007.

A. J. Peacock, N. F. Murphy, J. Mcmurray, L. Caballero, and S. Stewart, An epidemiological study of pulmonary arterial hypertension, Eur Respir J. juill, vol.30, issue.1, pp.104-113, 2007.

Y. Ling, M. K. Johnson, D. G. Kiely, R. Condliffe, C. A. Elliot et al., Changing demographics, epidemiology, and survival of incident pulmonary arterial hypertension: results from the pulmonary hypertension registry of the United Kingdom and Ireland, Am J Respir Crit Care Med, vol.186, issue.8, pp.790-796, 2012.

G. E. D'alonzo, R. J. Barst, S. M. Ayres, E. H. Bergofsky, B. H. Brundage et al., Survival in patients with primary pulmonary hypertension. Results from a national prospective registry, Ann Intern Med. 1 sept, vol.115, issue.5, pp.343-352, 1991.

S. Rich, D. R. Dantzker, S. M. Ayres, E. H. Bergofsky, B. H. Brundage et al., Primary pulmonary hypertension. A national prospective study, Ann Intern Med. août, vol.107, issue.2, pp.216-239, 1987.

P. Escribano-subias, I. Blanco, M. López-meseguer, C. J. Lopez-guarch, and A. Roman,

P. Morales, Survival in pulmonary hypertension in Spain: insights from the Spanish registry, Eur Respir J. sept, vol.40, issue.3, pp.596-603, 2012.

M. Humbert, O. Sitbon, and G. Simonneau, Treatment of pulmonary arterial hypertension, N Engl J Med, vol.351, issue.14, pp.1425-1461, 2004.
URL : https://hal.archives-ouvertes.fr/hal-01455600

N. Galiè, R. N. Channick, R. P. Frantz, E. Grünig, Z. C. Jing et al., Risk stratification and medical therapy of pulmonary arterial hypertension, Eur Respir J. janv, vol.53, issue.1, 2019.

O. Sitbon, C. Sattler, L. Bertoletti, L. Savale, V. Cottin et al., Initial dual oral combination therapy in pulmonary arterial hypertension, Eur Respir J, vol.47, issue.6, pp.1727-1763, 2016.
URL : https://hal.archives-ouvertes.fr/hal-01301539

R. M. Lang, L. P. Badano, V. Mor-avi, J. Afilalo, A. Armstrong et al., Recommendations for cardiac chamber quantification by echocardiography in adults: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging, Eur Heart J Cardiovasc Imaging. mars, vol.16, issue.3, pp.233-70, 2015.

M. Pahuja and D. Burkhoff, Right Ventricular Afterload Sensitivity Has Been on My Mind. Circ Heart Fail, vol.12, p.6345, 2019.

G. Kovacs, A. Olschewski, A. Berghold, H. Olschewski, D. M. Aviado et al., Pinsino A. Feasibility and usefulness of non-invasive hemodynamic monitoring in evaluating the response to intravenous therapy in decompensated heart failure, Eur Respir J. févr, vol.39, issue.2, pp.253-62, 1957.

B. Grimmer and W. M. Kuebler, The endothelium in hypoxic pulmonary vasoconstriction, J Appl Physiol, vol.01, issue.6, pp.1635-1681, 2017.

R. Mathew and B. M. Altura, Physiology and pathophysiology of pulmonary circulation

, Microcirc Endothelium Lymphatics, vol.6, pp.211-52, 1990.

R. Naeije, Physiology of the pulmonary circulation and the right heart, Curr Hypertens Rep. déc, vol.15, issue.6, pp.623-654, 2013.

S. Moncada, R. M. Palmer, and E. A. Higgs, Nitric oxide: physiology, pathophysiology, and pharmacology, Pharmacol Rev. juin, vol.43, issue.2, pp.109-151, 1991.

S. Moncada and A. Higgs, The L-arginine-nitric oxide pathway, N Engl J Med. 30 déc, vol.329, issue.27, pp.2002-2014, 1993.

A. Giaid, M. Yanagisawa, D. Langleben, R. P. Michel, R. Levy et al., Expression of endothelin-1 in the lungs of patients with pulmonary hypertension, N Engl J Med. 17 juin, vol.328, issue.24, pp.1732-1741, 1993.

P. Vermeersch, E. Buys, P. Pokreisz, G. Marsboom, F. Ichinose et al., Soluble guanylate cyclase-alpha1 deficiency selectively inhibits the pulmonary vasodilator response to nitric oxide and increases the pulmonary vascular remodeling response to chronic hypoxia, Circulation. 21 août, vol.116, issue.8, pp.936-979, 2007.

N. J. Pyne, V. Arshavsky, and A. Lochhead, cGMP signal termination, Biochem Soc Trans. nov, vol.24, issue.4, pp.1019-1041, 1996.

I. M. Lang and S. P. Gaine, Recent advances in targeting the prostacyclin pathway in pulmonary arterial hypertension, Eur Respir Rev. déc, vol.24, issue.138, pp.630-671, 2015.

J. A. Mitchell, B. Ahmetaj-shala, N. S. Kirkby, W. R. Wright, L. S. Mackenzie et al., Role of prostacyclin in pulmonary hypertension, Glob Cardiol Sci Pract, vol.2014, issue.4, pp.382-93, 2014.

R. F. O'brien, R. J. Robbins, and I. F. Mcmurtry, Endothelial cells in culture produce a vasoconstrictor substance, J Cell Physiol. août, vol.132, issue.2, pp.263-70, 1987.

P. Holm, Endothelin in the pulmonary circulation with special reference to hypoxic pulmonary vasoconstriction, Scand Cardiovasc J Suppl, vol.46, pp.1-40, 1997.

A. Kaoukis, S. Deftereos, K. Raisakis, G. Giannopoulos, G. Bouras et al., The role of endothelin system in cardiovascular disease and the potential therapeutic perspectives of its inhibition, Curr Top Med Chem, vol.13, issue.2, pp.95-114, 2013.

S. Sauvageau, E. Thorin, A. Caron, and J. Dupuis, Evaluation of endothelin-1-induced pulmonary vasoconstriction following myocardial infarction, Exp Biol Med (Maywood). juin, vol.231, issue.6, pp.840-846, 2006.

C. Cardillo, C. M. Kilcoyne, M. Waclawiw, R. O. Cannon, and J. A. Panza, Role of endothelin in the increased vascular tone of patients with essential hypertension, Hypertension. févr, vol.33, issue.2, pp.753-761, 1999.

M. Clozel, A. Maresta, and M. Humbert, Endothelin receptor antagonists, Handb Exp Pharmacol, vol.218, pp.199-227, 2013.

B. Guo, Y. Li, J. X. Liu, S. Miao, and C. , Nitric oxide/cyclic GMP pathway mediates the endothelin-1-upregulation of adiponectin expression in rat cardiomyocytes, Biomed Rep. sept, vol.7, issue.3, pp.267-71, 2017.

R. T. Schermuly, H. A. Ghofrani, M. R. Wilkins, and F. Grimminger, Mechanisms of disease: pulmonary arterial hypertension, Nat Rev Cardiol. 21 juin, vol.8, issue.8, pp.443-55, 2011.

E. Stacher, B. B. Graham, J. M. Hunt, A. Gandjeva, S. D. Groshong et al.,

K. B. Lane, R. D. Machado, M. W. Pauciulo, J. R. Thomson, and J. A. Phillips, Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension, Am J Respir Crit Care Med. 1 août, vol.186, issue.3, pp.81-85, 2000.

D. Montani, E. M. Lau, P. Dorfmüller, B. Girerd, X. Jaïs et al., Pulmonary venoocclusive disease, Eur Respir J, vol.47, issue.5, pp.1518-1552, 2016.
URL : https://hal.archives-ouvertes.fr/inserm-02561102

W. S. Kerstjens-frederikse, E. Bongers, M. Roofthooft, E. M. Leter, J. M. Douwes et al., TBX4 mutations (small patella syndrome) are associated with childhoodonset pulmonary arterial hypertension, J Med Genet. août, vol.50, issue.8, pp.500-506, 2013.

A. Vonk-noordegraaf, F. Haddad, K. M. Chin, P. R. Forfia, S. M. Kawut et al., Right heart adaptation to pulmonary arterial hypertension: physiology and pathobiology, J Am Coll Cardiol. 24 déc, vol.62, pp.22-33, 1925.

A. Vonk-noordegraaf, K. M. Chin, F. Haddad, P. M. Hassoun, A. R. Hemnes et al., Pathophysiology of the right ventricle and of the pulmonary circulation in pulmonary hypertension: an update, Eur Respir J, vol.53, issue.1, 2019.

A. Vonk-noordegraaf, B. E. Westerhof, and N. Westerhof, The Relationship Between the Right Ventricle and its Load in Pulmonary Hypertension, J Am Coll Cardiol. 17 janv, vol.69, issue.2, pp.236-279, 2017.

A. Frost, D. Badesch, J. Gibbs, D. Gopalan, D. Khanna et al., Diagnosis of pulmonary hypertension, Eur Respir J. janv, vol.53, issue.1, 2019.

P. Herve, E. M. Lau, O. Sitbon, L. Savale, D. Montani et al., Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers, J Am Coll Cardiol. 19 déc, vol.46, issue.3, pp.2546-52, 2006.

N. Galiè, H. A. Ghofrani, A. Torbicki, R. J. Barst, L. J. Rubin et al., Sildenafil citrate therapy for pulmonary arterial hypertension, N Engl J Med, vol.17, issue.20, pp.2148-57, 2005.

N. Galiè, B. H. Brundage, H. A. Ghofrani, R. J. Oudiz, G. Simonneau et al.,

, Tadalafil therapy for pulmonary arterial hypertension, Circulation. 9 juin, vol.119, issue.22, pp.2894-903, 2009.

H. Ghofrani, N. Galiè, F. Grimminger, E. Grünig, M. Humbert et al.,

, Riociguat for the treatment of pulmonary arterial hypertension, N Engl J Med. 25 juill, vol.369, issue.4, pp.330-370, 2013.

G. Simonneau, L. J. Rubin, N. Galiè, R. J. Barst, T. R. Fleming et al., Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial, Ann Intern Med, vol.149, issue.8, pp.521-551, 2008.

N. Galiè, J. A. Barberà, A. E. Frost, H. Ghofrani, M. M. Hoeper et al.,

, Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension, N Engl J Med. 27 août, vol.373, issue.9, pp.834-878, 2015.

O. Sitbon, X. Jaïs, L. Savale, V. Cottin, E. Bergot et al., Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study, Eur Respir J. juin, vol.43, issue.6, pp.1691-1698, 2014.

L. J. Rubin, D. B. Badesch, R. J. Barst, N. Galie, C. M. Black et al., Bosentan therapy for pulmonary arterial hypertension, N Engl J Med. 21 mars, vol.346, issue.12, pp.896-903, 2002.

N. Galiè, H. Olschewski, R. J. Oudiz, F. Torres, A. Frost et al., Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2, Circulation. 10 juin, vol.117, issue.23, pp.3010-3019, 2008.

T. Pulido, I. Adzerikho, R. N. Channick, M. Delcroix, N. Galiè et al.,

, Macitentan and morbidity and mortality in pulmonary arterial hypertension, N Engl J Med. 29 août, vol.369, issue.9, pp.809-827, 2013.

F. Dardi, A. Manes, M. Palazzini, C. Bachetti, G. Mazzanti et al., Combining bosentan and sildenafil in pulmonary arterial hypertension patients failing monotherapy: realworld insights, Eur Respir J. août, vol.46, issue.2, pp.414-435, 2015.

V. Mclaughlin, R. N. Channick, H. Ghofrani, J. Lemarié, R. Naeije et al.,

, Bosentan added to sildenafil therapy in patients with pulmonary arterial hypertension, Eur Respir J. août, vol.46, issue.2, pp.405-418, 2015.

L. J. Rubin, J. Mendoza, M. Hood, M. Mcgoon, R. Barst et al., Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol), Ann Intern Med. 1 avr, vol.112, issue.7, pp.485-91, 1990.

R. J. Barst, L. J. Rubin, W. A. Long, M. D. Mcgoon, S. Rich et al., Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial, Ann Intern Med. 21 mars, vol.334, issue.5, pp.425-459, 1996.

G. Simonneau, R. J. Barst, N. Galie, R. Naeije, S. Rich et al., Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial, Am J Respir Crit Care Med. 15 mars, vol.165, issue.6, pp.800-804, 2002.

O. Sitbon, R. Channick, K. M. Chin, A. Frey, S. Gaine et al., Selexipag for the Treatment of Pulmonary Arterial Hypertension, N Engl J Med. 24 déc, vol.373, issue.26, pp.2522-2555, 2015.

V. V. Mclaughlin, R. L. Benza, L. J. Rubin, R. N. Channick, R. Voswinckel et al., Addition of inhaled treprostinil to oral therapy for pulmonary arterial hypertension: a randomized controlled clinical trial, J Am Coll Cardiol, vol.55, issue.18, pp.1915-1937, 2010.

M. D'alto, R. Badagliacca, P. Argiento, E. Romeo, A. Farro et al., Risk Reduction and Right Heart Reverse Remodeling by Upfront Triple Combination Therapy in Pulmonary Arterial Hypertension, CHEST. 1 févr, vol.157, issue.2, pp.376-83, 2020.

S. Rich and B. H. Brundage, High-dose calcium channel-blocking therapy for primary pulmonary hypertension: evidence for long-term reduction in pulmonary arterial pressure and regression of right ventricular hypertrophy, Circulation. 1 juill, vol.76, issue.1, pp.135-176, 1987.

O. Sitbon, M. Humbert, X. Jaïs, V. Ioos, A. M. Hamid et al., Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension, Circulation. 14 juin, vol.111, issue.23, pp.3105-3116, 2005.

S. Rich, E. Kaufmann, and P. S. Levy, The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension, N Engl J Med. 9 juill, vol.327, issue.2, pp.76-81, 1992.

I. M. Robbins, S. M. Kawut, D. Yung, M. P. Reilly, W. Lloyd et al., A study of aspirin and clopidogrel in idiopathic pulmonary arterial hypertension, Eur Respir J. mars, vol.27, issue.3, pp.578-84, 2006.

S. M. Kawut, E. Bagiella, D. J. Lederer, D. Shimbo, E. M. Horn et al.,

, Randomized clinical trial of aspirin and simvastatin for pulmonary arterial hypertension: ASA-STAT, Circulation. 28 juin, vol.123, issue.25, pp.2985-93, 2011.

K. M. Olsson, M. Delcroix, H. A. Ghofrani, H. Tiede, D. Huscher et al.,

, Anticoagulation and survival in pulmonary arterial hypertension: results from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA), Circulation. 7 janv, vol.129, issue.1, pp.57-65, 2014.

L. Bertoletti, P. Mismetti, and H. Decousus, Letter by Bertoletti et al regarding article, « Anticoagulation and survival in pulmonary arterial hypertension: results from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) ». Circulation. 16 sept, vol.130, p.108, 2014.

I. R. Preston, K. E. Roberts, D. P. Miller, G. P. Sen, M. Selej et al., Effect of Warfarin Treatment on Survival of Patients With Pulmonary Arterial Hypertension (PAH) in the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL), Circulation. 22 déc, vol.132, issue.25, pp.2403-2414, 2015.

M. S. Khan, M. S. Usman, T. J. Siddiqi, S. U. Khan, M. H. Murad et al., Is Anticoagulation Beneficial in Pulmonary Arterial Hypertension?, Circ Cardiovasc Qual Outcomes, vol.11, issue.9, p.4757, 2018.

M. M. Hoeper, R. L. Benza, P. Corris, M. De-perrot, E. Fadel et al., Intensive care, right ventricular support and lung transplantation in patients with pulmonary hypertension, Eur Respir J, vol.53, issue.1, 2019.

M. Humbert, O. Sitbon, A. Chaouat, M. Bertocchi, G. Habib et al., Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era, Circulation. 13 juill, vol.122, issue.2, pp.156-63, 2010.

M. Humbert, O. Sitbon, A. Yaïci, D. Montani, D. S. O'callaghan et al., Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension, Eur Respir J. sept, vol.36, issue.3, pp.549-55, 2010.
URL : https://hal.archives-ouvertes.fr/hal-00929872

T. Thenappan, S. J. Shah, S. Rich, L. Tian, S. L. Archer et al., Survival in pulmonary arterial hypertension: a reappraisal of the NIH risk stratification equation, Eur Respir J. mai, vol.35, issue.5, pp.1079-87, 2010.

T. Thenappan, C. Glassner, and M. Gomberg-maitland, Validation of the pulmonary hypertension connection equation for survival prediction in pulmonary arterial hypertension, Chest. mars, vol.141, issue.3, pp.642-50, 2012.

W. Lee, Y. Ling, K. K. Sheares, J. Pepke-zaba, A. J. Peacock et al., Predicting survival in pulmonary arterial hypertension in the UK, Eur Respir J. sept, vol.40, issue.3, pp.604-615, 2012.

R. L. Benza, D. P. Miller, M. Gomberg-maitland, R. P. Frantz, A. J. Foreman et al., Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL), Circulation. 13 juill, vol.122, issue.2, pp.164-72, 2010.

R. L. Benza, M. Gomberg-maitland, D. P. Miller, A. Frost, R. P. Frantz et al., The REVEAL Registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension, Chest. févr, vol.141, issue.2, pp.354-62, 2012.

R. L. Benza, M. Gomberg-maitland, C. G. Elliott, H. W. Farber, A. J. Foreman et al., Predicting Survival in Patients With Pulmonary Arterial Hypertension: The REVEAL Risk Score Calculator 2.0 and Comparison With ESC/ERS-Based Risk Assessment Strategies, Chest, vol.156, issue.2, pp.323-360, 2019.

R. L. Benza, H. W. Farber, M. Selej, and M. Gomberg-maitland, Assessing risk in pulmonary arterial hypertension: what we know, what we don't, Eur Respir J, vol.50, issue.2, 2017.

R. L. Benza, H. W. Farber, A. Frost, H. Ghofrani, M. A. Gómez-sánchez et al., REVEAL risk scores applied to riociguat-treated patients in PATENT-2: Impact of changes in risk score on survival, J Heart Lung Transplant, vol.37, issue.4, pp.513-522, 2018.

O. Sitbon, R. L. Benza, D. B. Badesch, R. J. Barst, C. G. Elliott et al., Validation of two predictive models for survival in pulmonary arterial hypertension, Eur Respir J. juill, vol.46, issue.1, pp.152-64, 2015.

D. Kylhammar, B. Kjellström, C. Hjalmarsson, K. Jansson, M. Nisell et al., A comprehensive risk stratification at early follow-up determines prognosis in pulmonary arterial hypertension, Eur Heart J, vol.14, issue.47, pp.4175-81, 2018.

M. M. Hoeper, T. Kramer, Z. Pan, C. A. Eichstaedt, J. Spiesshoefer et al., Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model, Eur Respir J, vol.50, issue.2, 2017.

L. G. Rudski, W. W. Lai, J. Afilalo, L. Hua, M. D. Handschumacher et al., Guidelines for the echocardiographic assessment of the right heart in adults: a report from the American Society of Echocardiography endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography, J Am Soc Echocardiogr. juill, vol.23, issue.7, pp.685-713, 2010.

M. Berger, A. Haimowitz, A. Van-tosh, R. L. Berdoff, and E. Goldberg, Quantitative assessment of pulmonary hypertension in patients with tricuspid regurgitation using continuous wave Doppler ultrasound, J Am Coll Cardiol. août, vol.6, issue.2, pp.359-65, 1985.

P. J. Currie, J. B. Seward, K. L. Chan, D. A. Fyfe, D. J. Hagler et al., Continuous wave Doppler determination of right ventricular pressure: a simultaneous Dopplercatheterization study in 127 patients, J Am Coll Cardiol, vol.6, issue.4, pp.750-756, 1985.

M. R. Fisher, P. R. Forfia, E. Chamera, T. Housten-harris, H. C. Champion et al., Accuracy of Doppler echocardiography in the hemodynamic assessment of pulmonary hypertension, Am J Respir Crit Care Med. 1 avr, vol.179, issue.7, pp.615-636, 2009.

S. Greiner, J. A. Aurich, M. Hess, A. Hilbel, T. Hardt et al., Reliability of noninvasive assessment of systolic pulmonary artery pressure by Doppler echocardiography compared to right heart catheterization: analysis in a large patient population, J Am Heart Assoc. 21 août, vol.3, issue.4, 2014.

A. L. Hinderliter, P. W. Willis, R. J. Barst, S. Rich, L. J. Rubin et al., Effects of long-term infusion of prostacyclin (epoprostenol) on echocardiographic measures of right ventricular structure and function in primary pulmonary hypertension. Primary Pulmonary Hypertension Study Group, Circulation. 18 mars, vol.95, issue.6, pp.1479-86, 1997.

B. Ristow and N. B. Schiller, Stepping away from ritual right heart catheterization into the era of noninvasively measured pulmonary artery pressure, J Am Soc Echocardiogr. juill, vol.22, issue.7, pp.820-822, 2009.

A. E. Abbas, F. D. Fortuin, N. B. Schiller, C. P. Appleton, C. A. Moreno et al., A simple method for noninvasive estimation of pulmonary vascular resistance, J Am Coll Cardiol. 19 mars, vol.41, issue.6, pp.1021-1028, 2003.

F. Haddad, R. Zamanian, A. Beraud, I. Schnittger, J. Feinstein et al., A novel non-invasive method of estimating pulmonary vascular resistance in patients with pulmonary arterial hypertension, J Am Soc Echocardiogr. mai, vol.22, issue.5, pp.523-532, 2009.

L. M. Wright, N. Dwyer, D. Celermajer, L. Kritharides, and T. H. Marwick, Follow-Up of Pulmonary Hypertension With Echocardiography, JACC Cardiovasc Imaging, vol.9, issue.6, pp.733-779, 2016.

N. Galiè, M. M. Hoeper, M. Humbert, A. Torbicki, J. Vachiery et al., Guidelines for the diagnosis and treatment of pulmonary hypertension: the 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 the International Society of Heart and Lung Transplantation (ISHLT), Eur Heart J. oct, vol.30, issue.20, pp.2493-537, 2009.

P. R. Forfia, M. R. Fisher, S. C. Mathai, T. Housten-harris, A. R. Hemnes et al., Tricuspid annular displacement predicts survival in pulmonary hypertension, Am J Respir Crit Care Med, vol.174, issue.9, pp.1034-1075, 2006.

R. J. Raymond, A. L. Hinderliter, P. W. Willis, D. Ralph, E. J. Caldwell et al., Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension, J Am Coll Cardiol. 3 avr, vol.39, issue.7, pp.1214-1223, 2002.

S. B. Eysmann, H. I. Palevsky, N. Reichek, K. Hackney, and P. S. Douglas, Two-dimensional and Doppler-echocardiographic and cardiac catheterization correlates of survival in primary pulmonary hypertension, Circulation. août, vol.80, issue.2, pp.353-60, 1989.

G. Brierre, N. Blot-souletie, B. Degano, L. Têtu, V. Bongard et al., New echocardiographic prognostic factors for mortality in pulmonary arterial hypertension, Eur J Echocardiogr. juill, vol.11, issue.6, pp.516-538, 2010.

S. C. Mathai, C. T. Sibley, P. R. Forfia, J. O. Mudd, M. R. Fisher et al., Tricuspid annular plane systolic excursion is a robust outcome measure in systemic sclerosis-associated pulmonary arterial hypertension, J Rheumatol, vol.38, issue.11, pp.2410-2418, 2011.

M. Kawamukai, A. Hashimoto, M. Koyama, N. Nagano, J. Nishida et al., Risk classification of pulmonary arterial hypertension by echocardiographic combined assessment of pulmonary vascular resistance and right ventricular function. Heart Vessels, vol.34, pp.1789-800, 2019.

T. C. Yeo, K. S. Dujardin, C. Tei, D. W. Mahoney, M. D. Mcgoon et al., Value of a Doppler-derived index combining systolic and diastolic time intervals in predicting outcome in primary pulmonary hypertension, Am J Cardiol. 1 mai, vol.81, issue.9, pp.1157-61, 1998.

Y. Ogihara, N. Yamada, K. Dohi, A. Matsuda, A. Tsuji et al., Utility of right ventricular Tei-index for assessing disease severity and determining response to treatment in patients with pulmonary arterial hypertension, J Cardiol. févr, vol.63, issue.2, pp.149-53, 2014.

J. Grapsa, J. Gibbs, I. Z. Cabrita, G. F. Watson, H. Pavlopoulos et al., The association of clinical outcome with right atrial and ventricular remodelling in patients with pulmonary arterial hypertension: study with real-time three-dimensional echocardiography, Eur Heart J Cardiovasc Imaging. août, vol.13, issue.8, pp.666-72, 2012.

M. Bustamante-labarta, S. Perrone, R. L. De-la-fuente, P. Stutzbach, R. P. De-la-hoz et al., Right atrial size and tricuspid regurgitation severity predict mortality or transplantation in primary pulmonary hypertension, J Am Soc Echocardiogr, vol.15, issue.10, pp.1160-1164, 2002.

N. Nagaya, T. Nishikimi, M. Uematsu, T. Satoh, S. Kyotani et al., Plasma brain natriuretic peptide as a prognostic indicator in patients with primary pulmonary hypertension, Circulation. 22 août, vol.102, issue.8, pp.865-70, 2000.

S. Ghio, A. S. Pazzano, C. Klersy, L. Scelsi, C. Raineri et al., Clinical and prognostic relevance of echocardiographic evaluation of right ventricular geometry in patients with idiopathic pulmonary arterial hypertension, Am J Cardiol. 15 févr, vol.107, issue.4, pp.628-660, 2011.

N. M. Fine, L. Chen, P. M. Bastiansen, R. P. Frantz, P. A. Pellikka et al., Outcome prediction by quantitative right ventricular function assessment in 575 subjects evaluated for pulmonary hypertension, Circ Cardiovasc Imaging. sept, vol.6, issue.5, pp.711-732, 2013.

H. Sano, H. Tanaka, Y. Motoji, Y. Fukuda, T. Sawa et al., Right Ventricular Function and Right-Heart Echocardiographic Response to Therapy Predict Longterm Outcome in Patients With Pulmonary Hypertension, Canadian Journal of Cardiology. 1 avr, vol.31, issue.4, pp.529-565, 2015.

A. Sachdev, H. R. Villarraga, R. P. Frantz, M. D. Mcgoon, J. Hsiao et al., Right ventricular strain for prediction of survival in patients with pulmonary arterial hypertension, Chest. juin, vol.139, issue.6, pp.1299-309, 2011.

M. Haeck, R. Scherptong, N. A. Marsan, E. R. Holman, M. J. Schalij et al., Prognostic value of right ventricular longitudinal peak systolic strain in patients with pulmonary hypertension, Circ Cardiovasc Imaging. 1 sept, vol.5, issue.5, pp.628-664, 2012.

A. A. Da-costa-junior, J. S. Ota-arakaki, R. P. Ramos, M. Uellendahl, F. Mancuso et al., Diagnostic and prognostic value of right ventricular strain in patients with pulmonary arterial hypertension and relatively preserved functional capacity studied with echocardiography and magnetic resonance, Int J Cardiovasc Imaging. janv, vol.33, issue.1, pp.39-46, 2017.

L. Wright, N. Dwyer, S. Wahi, and T. H. Marwick, Relative Importance of Baseline and Longitudinal Evaluation in the Follow-Up of Vasodilator Therapy in Pulmonary Arterial Hypertension, JACC Cardiovasc Imaging, vol.12, issue.11, pp.2103-2114, 2019.

N. M. Bhave, S. H. Visovatti, B. Kulick, T. J. Kolias, and V. V. Mclaughlin, Right atrial strain is predictive of clinical outcomes and invasive hemodynamic data in group 1 pulmonary arterial hypertension, Int J Cardiovasc Imaging. juin, vol.33, issue.6, pp.847-55, 2017.

E. L. Hardegree, A. Sachdev, E. R. Fenstad, H. R. Villarraga, R. P. Frantz et al., Impaired left ventricular mechanics in pulmonary arterial hypertension: identification of a cohort at high risk, Circ Heart Fail. juill, vol.6, issue.4, pp.748-55, 2013.

N. Galiè, A. L. Hinderliter, A. Torbicki, T. Fourme, G. Simonneau et al., Effects of the oral endothelin-receptor antagonist bosentan on echocardiographic and doppler measures in patients with pulmonary arterial hypertension, J Am Coll Cardiol. 16 avr, vol.41, issue.8, pp.1380-1386, 2003.

K. S. Cha, K. I. Cho, J. S. Seo, J. H. Choi, Y. H. Park et al., Effects of inhaled iloprost on exercise capacity, quality of life, and cardiac function in patients with pulmonary arterial hypertension secondary to congenital heart disease (the Eisenmenger syndrome) (from the EIGER Study), Am J Cardiol. 1 déc, vol.112, issue.11, pp.1834-1843, 2013.

A. R. Tonelli, D. Conci, B. K. Tamarappoo, J. Newman, and R. A. Dweik, Prognostic value of echocardiographic changes in patients with pulmonary arterial hypertension receiving parenteral prostacyclin therapy, J Am Soc Echocardiogr. juill, vol.27, issue.7, pp.733-741, 2014.

N. J. Shelburne, K. S. Parikh, K. Chiswell, L. K. Shaw, J. Sivak et al.,

, Echocardiographic Assessment of Right Ventricular Function and Response to Therapy in Pulmonary Arterial Hypertension, Am J Cardiol, vol.124, issue.8, pp.1298-304, 2019.

R. Badagliacca, R. Poscia, B. Pezzuto, S. Papa, M. Reali et al., Prognostic relevance of right heart reverse remodeling in idiopathic pulmonary arterial hypertension, J Heart Lung Transplant, 2017.

A. Boucly, J. Weatherald, L. Savale, X. Jaïs, V. Cottin et al., Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension, Eur Respir J. août, vol.50, issue.2, p.1700889, 2017.
URL : https://hal.archives-ouvertes.fr/hal-01757586

C. Fauvel, O. Raitière, N. S. Belkacem, S. Dominique, E. Artaud-macari et al., Prognostic importance of Kidney, Heart and Interstitial lung diseases (KHI triad) in PH: A machine learning study, Arch Cardiovasc Dis. 1 sept, 2020.

M. C. Van-de-veerdonk, T. Kind, J. T. Marcus, G. Mauritz, M. W. Heymans et al., Progressive right ventricular dysfunction in patients with pulmonary arterial hypertension responding to therapy, J Am Coll Cardiol. 6 déc, vol.58, issue.24, pp.2511-2520, 2011.

M. C. Van-de-veerdonk, J. T. Marcus, N. Westerhof, F. S. De-man, A. Boonstra et al., Signs of Right Ventricular Deterioration in Clinically Stable Patients With Pulmonary Arterial Hypertension, Chest. 1 avr, vol.147, issue.4, pp.1063-71, 2015.

S. F. Nagueh, O. A. Smiseth, C. P. Appleton, B. F. Byrd, H. Dokainish et al., Recommendations for the Evaluation of Left Ventricular Diastolic Function by Echocardiography: An Update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging, J Am Soc Echocardiogr. avr, vol.29, issue.4, pp.277-314, 2016.

A. Menet, Y. Guyomar, P. Ennezat, P. Graux, A. L. Castel et al., Prognostic value of left ventricular reverse remodeling and performance improvement after cardiac resynchronization therapy: A prospective study, International Journal of Cardiology. 1 févr, vol.204, pp.6-11, 2016.

Y. Cheuk-man, B. Gabe, B. Fung, J. Wing-hong, S. Martin et al., Left Ventricular Reverse Remodeling but Not Clinical Improvement Predicts Long-Term Survival After Cardiac Resynchronization Therapy, Circulation. 13 sept, vol.112, issue.11, pp.1580-1586, 2005.

M. Humbert, O. Sitbon, A. Yaïci, D. Montani, D. S. O'callaghan et al., Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension, Eur Respir J. sept, vol.36, issue.3, pp.549-55, 2010.
URL : https://hal.archives-ouvertes.fr/hal-00929872

N. Galiè, V. V. Mclaughlin, L. J. Rubin, and G. Simonneau, An overview of the 6th World Symposium on Pulmonary Hypertension, Eur Respir J. janv, vol.53, issue.1, 2019.

J. A. Mazurek, A. Vaidya, S. C. Mathai, J. D. Roberts, and P. R. Forfia, Follow-up tricuspid annular plane systolic excursion predicts survival in pulmonary arterial hypertension: Pulmonary Circulation, pp.13-2017

D. Sur,

E. L. Hardegree, A. Sachdev, H. R. Villarraga, R. P. Frantz, M. D. Mcgoon et al., Role of serial quantitative assessment of right ventricular function by strain in pulmonary arterial hypertension, Am J Cardiol. 1 janv, vol.111, issue.1, pp.143-151, 2013.

N. Galiè, A. Manes, L. Negro, M. Palazzini, M. L. Bacchi-reggiani et al., A metaanalysis of randomized controlled trials in pulmonary arterial hypertension, Eur Heart J. févr, vol.30, issue.4, pp.394-403, 2009.

D. Miller, M. G. Farah, A. Liner, K. Fox, M. Schluchter et al., The relation between quantitative right ventricular ejection fraction and indices of tricuspid annular motion and myocardial performance, J Am Soc Echocardiogr. mai, vol.17, issue.5, pp.443-450, 2004.

S. Kaul, C. Tei, J. M. Hopkins, and P. M. Shah, Assessment of right ventricular function using two-dimensional echocardiography, Am Heart J. mars, vol.107, issue.3, pp.526-557, 1984.