Health-related Quality of Life and Patient-reported Outcomes in Pulmonary Arterial Hypertension

Abstract

Advances in our understanding of the basic pathophysiology of pulmonary arterial hypertension (PAH) has led to an expanding number of therapeutic options. The ultimate goals of therapy are to lengthen survival while improving symptoms and quality of life. A wealth of research in other conditions has established health-related quality of life (HRQoL) to be an important clinical endpoint. Until recently, however, little was known about HRQoL in PAH, and how best to measure it. Over the past few years, several studies have begun contributing to this growing area of research. Instruments used to assess HRQoL have varied between studies. The extent to which these instruments are valid in PAH depend on their specific measurement properties. In this article, we provide an overview of the different types of patient-reported outcomes (PROs) used in PAH, focusing in particular on the measurement of HRQoL. In the process, we review the current literature on HRQoL in PAH, summarize the available data from randomized controlled trials, and discuss the implications of these findings on future research. Despite significant progress, the study of HRQoL in PAH remains a nascent field relative to other conditions. As the use of PROs continues to increase, additional work will be needed to begin standardizing the reporting and interpretation of such outcomes in future clinical trials.

Predictors of Health-Related Quality of Life in Patients With Idiopathic Pulmonary Arterial Hypertension

“…9,10 Several studies evaluating health status and HRQL in PAH combine the etiologies and report as a single cohort 11 ; several pharmacological studies have measured health status and/or HRQL as end points to determine treatment efficacy. [11][12][13][14][15][16] A comparison of HRQL between patients with various PAH etiologies (n = 155) showed that patients with IPAH had better HRQL than those with systemic sclerosis, although HRQL was diminished in all patients with PAH.…”

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

“…When previously studied, multiple studies have noted QOL for patients with PAH is poor or sub-optimal, [13][14][15][16][17][18] However, to our knowledge, the incorporation of PC into a management plan for PAH has not been evaluated. To better understand the need for such an intervention, this study evaluated symptom burden, QOL, and attitudes regarding PC in a large, international sample of PAH patients and was aimed at identifying barriers to effective PC as well as characterizing opportunities to improve QOL in PAH patients.…”

Effects of Surgical and Medical Treatment on Quality of Life for Patients With Chronic Thromboembolic Pulmonary Hypertension

“…12 Changes in physiological measures may not always translate into benefits that can be perceived by the patient. 13 Some studies of patients with PH have provided controversial results and have questioned whether improvement in QOL is associated with improvements in laboratory data, hemodynamics, exercise capacity, and survival. 14 Therefore, health-related QOL is often measured as an additional endpoint.…”