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Predicting Survival in Patients with Pulmonary Arterial Hypertension

Epidemiology, Prognosis, Outcome
ESC Working Groups

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 Feb 14. pii: S0012-3692(19)30152-7


Pulmonary arterial hypertension treatment has made substantial progress over the past decade. The current treatment strategy is based on the severity of the newly diagnosed PAH patient as assessed by a multiparametric risk stratification approach

Currently, we have two different perspectives. Fist, the 2015 ESC/ERS PH guidelines have recommended a risk assessment using a multidimensional stratification based exclusively on modifiable clinical, functional, exercise, biochemical, echocardiographic and haemodynamic variables with known prognostic significance, then are  categorized as low, intermediate, or high risk based on expected 1-year mortality. Recently, a retrospective analysis of three major registries comprising 3.135 patients: the French Pulmonary Hypertension Network (FPHN), Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA)  and Swedish PAH Register have  provided an independent validation of this approach.

Second, The REVEAL Score (The Registry to Evaluate Early and Long-term PAH Disease Management). It is a means of providing the one-year likelihood of survival  in incident and prevalent IPAH and associated PAH patients. It is derived from a cohort of 2716 PAH patients.

The present paper shows the revised REVEAL 2.0 risk score calculator ( 14 variables),  which adds  all-cause hospitalizations within the previous 6 months and glomerular filtration rate.  The REVEAL 2.0 risk score streamlines the 14 variables into 3 manageable categories comparable with the European risk scores:  low risk=REVEAL score ⩽6, intermediate risk=REVEAL score 7 and 8, and high risk=REVEAL score ⩾9. The simplified Reveal 2.0 score demonstrated greater risk discrimination than the FPNH and COMPERA risk assessment strategies when applies to patients enrolled in the REVEAL registry.

What does this study teach us?  

There is a substantial overlap between risk stratification status. The same patients could be categorized in different risk status depending on the risk assessment strategy followed. The research of prognostic scores, which can be easily applied in clinical practice, should be continued.


Galiè N, Humbert M, Vachiery JL, 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 ofCardiology (ESC) and the European Respiratory Society (ERS): 2016;37(1):67-119.

Farber HW, Benza RL. Risk assessment tools in pulmonary arterial hypertension. Prognosis for prospective trials? Am J Respir Crit Care Med. 2018;197(7):843-845.

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): 4175-4181.

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.

Boucly A, Weatherald J, Savale L, et al. Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension. Eur Respir J. 2017;50(2): 1700889.

Notes to editor

To contact P. Escribano-Subias, please use this link to address an email

The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.

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