|| Checking for direct PDF access through Ovid
Step-up management algorithms are recommended by recently published clinical guidelines for use in pulmonary arterial hypertension (PAH). Decisions regarding when and which treatment should be introduced as well as whether and when to escalate the treatment by augmenting the dose, switching to or adding another drug, changing the administration route or progressing to interventional therapy require monitoring of the severity of disease and assessment of its response to applied treatment.The optimal way to monitor PAH would be to use tests and variables that are related to survival. To date decisions regarding escalation of treatment and, especially, listing for transplantation are based primarily on the assessment of World Health Organization (WHO) functional class (FC). There is evidence that persistence of III or IV WHO functional class (FC) despite specific treatment is related to an unacceptably high risk of deaths and warrants listing for transplantation. However, FC assessment is a relatively rough and subjective marker and certainly there is much room left for improvement. This paper will review current knowledge regarding noninvasive methods and strategies suitable for monitoring patients with PAH.