Symptom burden, quality of life, and attitudes toward palliative care in patients with pulmonary arterial hypertension: Results from a cross-sectional patient survey

BACKGROUND: Pulmonary arterial hypertension (PAH) is a complex disease with variable clinical manifestations; nevertheless, morbidity and mortality associated with PAH are considerable. This study examined quality of life (QOL) in PAH patients and assessed use of palliative care (PC) for addressing QOL issues and what barriers might exist regarding early PC implementation for patients with PAH. METHODS: An Internet-based survey was distributed to Pulmonary Hypertension Association patient-related listservs. Symptom burden and QOL were assessed using Linear Analog Self Assessment (LASA) QOL items and the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR). RESULTS: Of 774 eligible patients with active e-mail addresses, 315 returned surveys (41% overall response), and 276 (88%) contained analyzable responses. Responders (mean age, 48.9 years +/- 16.0) were predominantly white (85%), female (86%), and with idiopathic PAH (42%). Profound deficiency in overall QOL (40%), fatigue (57%), physical well-being (56%), social activity (49%), emotional well-being (49%), and pain (38%) were reported. Most patients believed their PAH physician had excellent understanding of PAH progression/plan of care (92%), but less were satisfied with care regarding QOL management (77%). Few patients considered PC (8%), or had pain management (4%) or PC involved (1%). Most common reasons were beliefs that patients were doing well/not sick (63%) or that PC had not been suggested (22%). CONCLUSIONS: PAH may result in symptoms or QOL impairment persisting despite optimal PAH therapy. However, PC awareness or use by PAH patients and providers is low. Opportunities may exist to integrate PC into care for PAH patients. J Heart Lung Transplant 2012;31:1102-8 (C) 2012 International Society for Heart and Lung Transplantation. All rights reserved.