title: Pulmonary arterial hypertension: the burden of disease and impact on quality of life.
abstract: Pulmonary arterial hypertension (PAH) is a debilitating disease that pervades all aspects of a patient's daily life. It is also increasingly acknowledged that the burden of PAH extends to older patients and carers. Until recently, the adverse effect of disease symptoms on the physical, emotional and social factors governing patient health-related quality of life (HRQoL) remained largely unrecognised. With a shift in therapeutic objectives to longer term improvements and HRQoL benefits, clinical trials now frequently include HRQoL measures as study end-points. Most HRQoL instruments used in patients with PAH are generic or non-disease-specific questionnaires and therefore may not accurately capture PAH disease burden. New PAH-specific HRQoL instruments currently undergoing validation include emPHasis-10 and Pulmonary Arterial Hypertension-Symptoms and Impact (PAH-SYMPACT; Actelion Pharmaceuticals Ltd, Allschwil, Switzerland). Using various HRQoL measures, pharmacological therapies have been shown to improve HRQoL in patients with PAH. Patients also derive HRQoL benefits from nonpharmacological strategies, which include the emotional support provided by multidisciplinary care and support groups that is fundamental to patient wellbeing. Looking to the future, validated PAH-specific HRQoL instruments together with dedicated guidelines and procedures are essential to support the translation of HRQoL scores to the clinic, thus enabling a holistic treatment approach to the management of patients with PAH.
authors.affiliation: Dept of Respiratory Diseases, KU Leuven, University of Leuven and University Hospitals Leuven, Leuven, Belgium marion.delcroix@uzleuven.be.
authors.forename: Marion
authors.initials: M
authors.lastname: Delcroix
authors.affiliation: Dept of Cardiac Sciences, Hammersmith Hospital and Imperial College London, London, UK.
authors.forename: Luke
authors.initials: L
authors.lastname: Howard
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use; D062186:Arterial Pressure / Q000187:drug effects; D017028:Caregivers / Q000523:psychology; D003131:Combined Modality Therapy; D015897:Comorbidity; D017281:Cost of Illness; D006304:Health Status; D006694:Holistic Health; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology / Q000523:psychology* / Q000628:therapy; D011651:Pulmonary Artery / Q000187:drug effects / Q000503:physiopathology*; D011788:Quality of Life; D012307:Risk Factors; D011795:Surveys and Questionnaires; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents