Risk stratification in pulmonary arterial hypertension.

Abstract

Periodic risk stratification is recommended for patients with pulmonary arterial hypertension (PAH). The purpose of this article is to review the available risk stratification tools in PAH. Validated tools, such as the REVEAL (Registry to Evaluate Early And Long-term PAH Disease Management) score or European Society of Cardiology/European Respiratory Society (ESC/ERS) risk assessment table incorporate clinical, exercise, imaging, and hemodynamic variables to obtain a multidimensional estimate of prognosis. Recent studies from REVEAL and European registries have further validated these tools and improved our understanding of prognostic factors in PAH. Serial risk assessment over time, especially after treatment initiation or modification, provides more useful information than at a single time point, with changes in risk profiles being particularly informative. A brief, simplified approach includes counting the number of ESC/ERS low-risk criteria achieved, with 3 or 4 low-risk criteria associated with an excellent prognosis. When a more precise estimation of risk is desired, the REVEAL score can delineate five risk groups with 1-year survival rates between 97.3% (low risk) and 50.2% (very high risk). The REVEAL and ESC/ERS risk table are useful, validated, multidimensional risk stratification tools that should be periodically applied to patients with PAH in practice.