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Current Pharmaceutical Design


ISSN (Print): 1381-6128
ISSN (Online): 1873-4286

Review Article

Prediction Models and Scores in Pulmonary Hypertension: A Review

Author(s): Sophia Anastasia Mouratoglou, Ahmed A. Bayoumy and Anton Vonk Noordegraaf*

Volume 27, Issue 10, 2021

Published on: 05 November, 2020

Page: [1266 - 1276] Pages: 11

DOI: 10.2174/1381612824999201105163437

Price: $65


Background: Pulmonary arterial hypertension (PAH) is a serious disease with increased morbidity and mortality. The need for an individualized patient treatment approach necessitates the use of risk assessment in PAH patients. That may include a range of hemodynamic, clinical, imaging and biochemical parameters derived from clinical studies and registry data.

Objective: In the current systematic review, we summarize the available data on risk prognostic models and scores in PAH and we explore the possible concordance amongst different risk stratification tools in PAH.

Methods: PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines aided the performance of this systematic review. Eligible studies were identified through a literature search in the electronic databases PubMed, Science Direct, Google Scholar and Cochrane with the use of various combinations of MeSH and non-MeSH terms, with a focus on PAH.

Results: Overall, 25 studies were included in the systematic review; out of them, 9 were studies deriving prognostic equations and risk scores and 16 were validating studies of an existing score. The majority of risk stratification scores use hemodynamic data for the assessment of prognosis, while others also include clinical and demographic variables in their equations. The risk discrimination in the overall PAH population was adequate, especially in differentiating the low versus high-risk patients, but their discrimination ability in the intermediate groups remained lower. Current ESC/ERS proposed risk stratification score utilizes a limited number of parameters with prognostic significance, whose prognostic ability has been validated in European patient populations.

Conclusion: Despite improvement in risk estimation of prognostic tools of the disease, PAH morbidity and mortality remain high, necessitating the need for the risk scores to undergo periodic re-evaluation and refinements to incorporate new data into predictors of disease progression and mortality and, thereby, maintain their clinical utility.

Keywords: Pulmonary hypertension, prognosis, prediction models, hemodynamic, demographic, PAH morbidity.

Sanz J, Sánchez-Quintana D, Bossone E, Bogaard HJ, Naeije R. Anatomy, function, and dysfunction of the right ventricle: JACC state-of-the-art review. J Am Coll Cardiol 2019; 73(12): 1463-82.
[] [PMID: 30922478]
Afifi S, Shayan S, Al-Qamari A. Pulmonary hypertension and right ventricular function: interdependence in pathophysiology and management. Int Anesthesiol Clin 2009; 47(1): 97-120.
[] [PMID: 19131755]
Galiè N, Channick RN, Frantz RP, et al. Risk stratification and medical therapy of pulmonary arterial hypertension. Eur Respir J 2019; 53(1): 1801889.
[] [PMID: 30545971]
Galiè N, Humbert M, Vachiery J-L, et al. ESC Scientific Document Group. 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.
[] [PMID: 26320113]
Kanwar M, Raina A, Lohmueller L, Kraisangka J, Benza R. The use of risk assessment tools and prognostic scores in managing patients with pulmonary arterial hypertension. Curr Hypertens Rep 2019; 21(6): 45-5.
[] [PMID: 31025123]
Cogswell R, Pritzker M, De Marco T. Performance of the REVEAL pulmonary arterial hypertension prediction model using non-invasive and routinely measured parameters. J Heart Lung Transplant 2014; 33(4): 382-7.
[] [PMID: 24534251]
Cogswell R, Kobashigawa E, McGlothlin D, Shaw R, De Marco T. Validation of the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) pulmonary hypertension prediction model in a unique population and utility in the prediction of long-term survival. J Heart Lung Transplant 2012; 31(11): 1165-70.
[] [PMID: 23062726]
Benza RL, Gomberg-Maitland M, Elliott CG, 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 2019; 156(2): 323-37.
[] [PMID: 30772387]
Benza RL, Gomberg-Maitland M, Miller DP, et al. The REVEAL Registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension. Chest 2012; 141(2): 354-62.
[] [PMID: 21680644]
Benza RL, Miller DP, Gomberg-Maitland M, et al. Predicting survival in pulmonary arterial hypertension. Circulation 2010; 122(2): 164-72.
[] [PMID: 20585012]
D’Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 1991; 115(5): 343-9.
[] [PMID: 1863023]
Humbert M, Sitbon O, Yaïci A, et al. French Pulmonary Arterial Hypertension Network. Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension. Eur Respir J 2010; 36(3): 549-55.
[] [PMID: 20562126]
Lee W-TN, Ling Y, Sheares KK, Pepke-Zaba J, Peacock AJ, Johnson MK. Predicting survival in pulmonary arterial hypertension in the UK. Eur Respir J 2012; 40(3): 604-11.
[] [PMID: 22556026]
Okada O, Tanabe N, Yasuda J, et al. Prediction of life expectancy in patients with primary pulmonary hypertension. A retrospective nationwide survey from 1980-1990. Intern Med 1999; 38(1): 12-6.
[] [PMID: 10052735]
Swift AJ, Capener D, Johns C, et al. Magnetic resonance imaging in the prognostic evaluation of patients with pulmonary arterial hypertension. Am J Respir Crit Care Med 2017; 196(2): 228-39.
[] [PMID: 28328237]
Thenappan T, Shah SJ, Rich S, Tian L, Archer SL, Gomberg-Maitland M. Survival in pulmonary arterial hypertension: a reappraisal of the NIH risk stratification equation. Eur Respir J 2010; 35(5): 1079-87.
[] [PMID: 20032020]
Anderson JJ, Lau EM, Lavender M, et al. Retrospective Validation of the REVEAL 2.0 Risk Score with the Australian and New Zealand Pulmonary Hypertension Registry Cohort. Chest 2020; 157(1): 162-72.
[] [PMID: 31563497]
Benza RL, Miller DP, Barst RJ, Badesch DB, Frost AE, McGoon MD. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest 2012; 142(2): 448-56.
[] [PMID: 22281797]
Benza RL, Miller DP, Foreman AJ, et al. Prognostic implications of serial risk score assessments in patients with pulmonary arterial hypertension: a Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) analysis. J Heart Lung Transplant 2015; 34(3): 356-61.
[] [PMID: 25447572]
Boucly A, Weatherald J, Humbert M, Sitbon O. Risk assessment in pulmonary arterial hypertension. Eur Respir J 2018; 51(3): 1800279.
[] [PMID: 29599118]
Escribano-Subias P, Blanco I, López-Meseguer M, et al. REHAP investigators. Survival in pulmonary hypertension in Spain: insights from the Spanish registry. Eur Respir J 2012; 40(3): 596-603.
[] [PMID: 22362843]
Hoeper MM, Kramer T, Pan Z, et al. Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. Eur Respir J 2017; 50(2): 1700740.
[] [PMID: 28775047]
Kane GC, Maradit-Kremers H, Slusser JP, Scott CG, Frantz RP, McGoon MD. Integration of clinical and hemodynamic parameters in the prediction of long-term survival in patients with pulmonary arterial hypertension. Chest 2011; 139(6): 1285-93.
[] [PMID: 21071530]
Kylhammar D, Kjellström B, Hjalmarsson C, et al. A comprehensive risk stratification at early follow-up determines prognosis in pulmonary arterial hypertension. Eur Heart J 2018; 39(47): 4175-81.
[] [PMID: 28575277]
Lim Y, Low T-T, Chan S-P, et al. Pulmonary arterial hypertension in a multi-ethnic Asian population: Characteristics, survival and mortality predictors from a 14-year follow-up study. Respirology 2019; 24(2): 162-70.
[] [PMID: 30180305]
Ling Y, Johnson MK, Kiely DG, 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 2012; 186(8): 790-6.
[] [PMID: 22798320]
Sandoval J, Bauerle O, Palomar A, et al. Survival in primary pulmonary hypertension. Validation of a prognostic equation. Circulation 1994; 89(4): 1733-44.
[] [PMID: 8149539]
Sitbon O, Benza RL, Badesch DB, et al. Validation of two predictive models for survival in pulmonary arterial hypertension. Eur Respir J 2015; 46(1): 152-64.
[] [PMID: 25837032]
Xiong W, Zhao Y, Xu M, Pudasaini B, Guo X, Liu J. A modified risk score in one-year survival rate assessment of group 1 pulmonary arterial hypertension. BMC Pulm Med 2018; 18(1): 161-1.
[] [PMID: 30326867]
Thenappan T, Glassner C, Gomberg-Maitland M. Validation of the pulmonary hypertension connection equation for survival prediction in pulmonary arterial hypertension. Chest 2012; 141(3): 642-50.
[] [PMID: 21885728]
Humbert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 2006; 173(9): 1023-30.
[] [PMID: 16456139]
Barst RJ, Galie N, Naeije R, et al. Long-term outcome in pulmonary arterial hypertension patients treated with subcutaneous treprostinil. Eur Respir J 2006; 28(6): 1195-203.
[] [PMID: 16899485]
Barst RJ, McGoon M, McLaughlin V, et al. Beraprost Study Group. Beraprost therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2003; 41(12): 2119-25.
[] [PMID: 12821234]
Hiremath J, Thanikachalam S, Parikh K, et al. TRUST Study Group. Exercise improvement and plasma biomarker changes with intravenous treprostinil therapy for pulmonary arterial hypertension: a placebo-controlled trial. J Heart Lung Transplant 2010; 29(2): 137-49.
[] [PMID: 20022264]
Lang I, Gomez-Sanchez M, Kneussl M, et al. Efficacy of long-term subcutaneous treprostinil sodium therapy in pulmonary hypertension. Chest 2006; 129(6): 1636-43.
[] [PMID: 16778286]
McLaughlin VV, Sitbon O, Badesch DB, et al. Survival with first-line bosentan in patients with primary pulmonary hypertension 2005; 2: 244-9.
Sitbon O, McLaughlin VV, Badesch DB, et al. Survival in patients with class III idiopathic pulmonary arterial hypertension treated with first line oral bosentan compared with an historical cohort of patients started on intravenous epoprostenol. Thorax 2005; 60(12): 1025-30.
[] [PMID: 16055621]

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