Quality of life
improving QoL in patients with PAH. Besides the assessment of therapy success in clinical trials, QoL is increasingly considered in the daily management of PAH. One important example is ‘shared decision making’,26 which reflects the interaction between patient and physician in order to develop a personalised, individually tailored management strategy. Individual patient needs may differ significantly. It is the physicians’ duty to recognise and support them. In addition, the goals of physicians and patients may not necessarily be the same: physicians are trained to identify prognostic relevant parameters and try to improve them, leading sometimes to the ‘treatment’ of haemodynamic or laboratory parameters instead of treating the patient. Shared decision making may even lead to the acceptance of a worsening of such parameters or to the decision against further therapy escalation if it is reasonable from the patient’s QoL point of view. This approach is well known within oncology and is being increasingly adapted in patients with severe cardiovascular diseases. A specific issue within this process is end of life care. This issue is discussed in the actual PH guidelines and it is recognised that open and sensitive communication with patients may allow advanced planning and discussion of their fears, concerns and wishes, which is essential for good care in all patients.13
non-pharmacological interventions may serve an important purpose in providing the emotional support that has been shown to be fundamental to the wellbeing of patients with PAH and their families.27
What role functional endpoints play in the daily life of PAH patients is an important question. Based on the discussed considerations, we may conclude that several functional parameters (including 6MWD) are associated with parameters reflecting patients’ daily QoL. The assessment of QoL in PAH patients is of great value because QoL has proved to be of prognostic relevance and it helps to determine minimal important clinical differences of functional tests. This facilitates the interpretation of their results and provides a connection to the daily life of patients. Clinically meaningful endpoints representing
patients’ daily QoL, such as TTCW, are now used as primary endpoints of clinical trials, allowing real-life conclusions in clinical studies to be drawn. l
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