Symptoms and Health-Related Quality of Life in Idiopathic and Associated Pulmonary Arterial Hypertension

Matura, Lea Ann

Introduction: Pulmonary arterial hypertension (PAH) is a chronic illness affecting primarily young and middle age women. Elevated pulmonary pressures lead to right heart failure and premature death. Patients with PAH report multiple symptoms (e.g. dyspnea, fatigue) that can be severe and impair health-related quality of life (HRQOL). PAH etiology is varied with approximately half being idiopathic PAH. Associated PAH includes those with PAH caused by other disorders such as: connective tissue disease, congenital heart disease, portopulmonary hypertension and anorexigens.

Purpose: The purpose of this study was to determine if there were differences between symptom severity and HRQOL in patients with idiopathic PAH and associated PAH.

Methods: This was a secondary analysis of an existing data set of patients with PAH. The convenience sample included 191 patients with PAH (n=104 idiopathic PAH; n=87 associated PAH). Subjects completed a socio-demographic and clinical data form, the Pulmonary Arterial Hypertension Symptom Scale (PAHSS) (dyspnea on exertion, fatigue, difficulty sleeping, chest pain, dizziness, syncope, palpitations, dyspnea at rest, dyspnea lying down, awaken at night dyspneic, swelling ankles/feet, cough, hoarseness, abdominal swelling, nausea, loss of appetite and Raynaud’s phenomenon; scores range 0-10), and the Medical Outcomes Survey Short Form 36 (SF-36) (scores range 0-100). Descriptive statistics described the sample. Independent t-tests and chi square determined differences among continuous and categorical variables for the two groups.

Results: Eighty two with idiopathic PAH were female and 22 male; in contrast, 80 with associated PAH were female and 7 were male (p=0.007). The mean age was 51.8 + 16.6 year for idiopathic PAH and 54.8 +13.0 for associated PAH (p=0.176).There were no significant differences between the groups on marital status, employment status, functional class, oxygen use or medications. There were no significant differences between symptom severity on the PAHSS. Dyspnea on exertion (idiopathic PAH 5.0 + 2.5; associated PAH 5.0 + 2.0, p=0.798) and fatigue (idiopathic PAH 6.3 + 2.7; associated PAH 6.1 + 2.8, p=0.671) were the most severe symptoms reported on the PAHSS. On the SF-36 subscales there were differences between General Health (idiopathic PAH 40.6 + 22.9; associated PAH 32.7 + 18.3, p=0.009) and Emotional (idiopathic PAH 69.1 + 20.5; associated PAH 75.2 + 17.2, p=0.029).

Conclusions: Symptom severity is similar for patients with idiopathic and associated PAH. Those with associated PAH were experiencing worse General Health but better Emotional health than idiopathic PAH. The varied disease states in associated PAH may partially explain the worse reported General Health. This is important for clinicians to understand that there may be differences among the PAH etiologies in order to assess, treat appropriately and improve HRQOL.