Abstract
BACKGROUND: Pulmonary arterial hypertension (PAH) is a debilitating and progressive pulmonary pathology that often leads to death. Guidelines recommend the use of palliative care (PC) early in the treatment course to ease the burden of symptoms; however, uptake remains low. AIM: To evaluate barriers to PC uptake and determine its association with mortality in patients with PAH. METHODS: All studies discussing PC in PAH were selected in our review and analysis. Clinical and cross-sectional studies were included. Barriers were described in a qualitative fashion. A random-effects meta-analysis was also conducted, in which the odds ratio for mortality was pooled and reported, along with 95% confidence intervals. RESULTS: A total of 19 studies were included in the review. The most common barriers identified included feeling like the patients were "not sick enough", belief that PC is only appropriate for end-of-life care, belief that it would burden family members, and general feelings of hopelessness. Physicians identified structural causes, such as a lack of funding and low levels of PC-related knowledge, as barriers to recommending PC. The meta-analysis showed no statistically significant difference in mortality across four included studies (Log odds ratio = 0.89, 95% confidence intervals: -3.06-1.28). Heterogeneity was high (I (2) = 80.32%). CONCLUSION: Uptake of PC in PAH is low due to patient and physician-level barriers, which can be overcome with systematic PC integration. Long-term studies are also needed to investigate the impact of PC on outcomes in PAH, as the current limited data show no significant difference.