The Frequency and Predictors of Pulmonary Rehabilitation Referrals Among Patients With Pulmonary Arterial Hypertension: An Analysis of the Pulmonary Hypertension Association Registry

Background: Pulmonary arterial hypertension (PAH) is a complex cardiopulmonary disease associated with exertional dyspnea and impaired health-related quality of life (HRQOL) despite medical therapy. Pulmonary rehabilitation (PR), a supervised exercise program for patients with chronic lung disease, improves symptoms, HRQOL, and exercise capacity. Despite these benefits, there is a paucity of data regarding PR in PAH. Research Question: What is the frequency of PR referrals among patients with PAH, and what factors are associated with PR referrals? Study Design and Methods: We performed a retrospective cohort study of adults with PAH enrolled in the Pulmonary Hypertension Association Registry. Patients referred to PR by the time of the second follow-up visit were compared with patients never referred to PR. Results: PR referral rates were 17.0%, 26.6%, and 32.3% at the first, second, and third follow-up visits, respectively. Patients referred to PR were older (58.3 ± 15.3 vs 55.2 ± 15.6; P < .001), more likely to be female (81.9% vs 75.9%; P = .02), and exhibited differences in race, geographic region, employment status, and PAH etiology. Referred patients had worse functional class, risk stratification, HRQOL, and 6-minute walk distance with higher rates of supplemental oxygen use and similar pulmonary hemodynamics and use of PAH therapy. In multivariable analysis, race, region, and PAH etiology and disease severity as assessed by REVEAL Lite 2.0 scores, supplemental oxygen use, and lung transplantation referral were independently associated with PR referral. In a subgroup analysis, less than one-half of patients referred to PR reported participation in PR within the preceding 6 months. PR participation, but not PR referrals, was associated with improvements in HRQOL. Interpretation: Despite well-established benefits, this study indicates that referral to PR is low among patients with PAH and is significantly impacted by demographic and socioeconomic factors as well as disease characteristics.