Risk assessment in pulmonary arterial hypertension

Amresh Raina, Marc Humbert, Amresh Raina, Marc Humbert

Abstract

Regular patient assessment is essential for the management of chronic diseases, such as pulmonary arterial hypertension (PAH). Comprehensive patient assessment and risk stratification in PAH are important to guide treatment decisions and to monitor disease progression as well as patients' response to treatment. Approaches for assessing risk in PAH patients include the use of risk variables, as recommended in the 2015 European Society of Cardiology (ESC)/European Respiratory Society (ERS) pulmonary hypertension (PH) guidelines, and the application of risk equations and scores, such as the French registry risk equation and the REVEAL registry risk score. Risk stratification and risk scores are both useful predictors of survival on a population basis, and provide an estimate for individual patients' risk. The 2015 ESC/ERS PH guidelines recommend regular assessment of multiple variables at an expert centre. The respective merits and limitations of different risk assessment methods in PAH are discussed in this article, as well as some considerations that can be taken into account in the future development of risk assessment tools.

Conflict of interest statement

Editorial comment in Eur Respir Rev 2016; 25: 361–363.

Conflict of interest: Disclosures can be found alongside this article at err.ersjournals.com

Copyright ©ERS 2016.

Figures

FIGURE 1
FIGURE 1
Simplified REVEAL (Registry to Evaluate Early And Long-term PAH Disease Management) risk score. PAH: pulmonary arterial hypertension; WHO: World Health Organization; NYHA: New York Heart Association; BNP: brain natriuretic peptide; APAH; associated PAH; CTD: connective tissue disease; PoPH: portopulmonary hypertension; FPAH: familial PAH; SBP: systolic blood pressure; HR: heart rate; DLCO: diffusing capacity of the lung for carbon monoxide; mRAP: mean right atrial pressure; PVR: pulmonary vascular resistance. Reproduced from [10] with permission from the publisher.
FIGURE 2
FIGURE 2
1-year survival in the REVEAL (Registry to Evaluate Early And Long-term PAH Disease Management) validation cohort as predicted according to the a) risk prognostic equation and b) simplified risk score. PAH: pulmonary arterial hypertension. Reproduced from [10] with permission from the publisher.

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