Risk stratification plays an important role in predicting outcomes and guiding treatment decisions of patients with pulmonary arterial hypertension (PAH). The three most important non-invasive predictive prognostic factors are the World Health Organization functional class, the 6-minute walk distance, and natriuretic peptides. These three are included in all currently validated risk stratification tools. However, due to limitations primarily related to the reduced specificity of PAH severity, these variables are not always sufficient to guide individual treatment decisions. Furthermore, with effective combination and new PAH therapies, markers associated with pulmonary vascular remodeling are expected to become increasingly relevant for guiding the treatment of patients with PAH. While achieving a low mortality risk, measured with a validated risk assessment tool, remains an important treatment goal, preliminary data suggest that invasive hemodynamics and cardiac imaging may provide additional value in guiding treatment decisions.
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