Health disparities and treatment approaches in portopulmonary hypertension and idiopathic pulmonary arterial hypertension: an analysis of the Pulmonary Hypertension Association Registry

Abstract

Compared to idiopathic pulmonary arterial hypertension (IPAH), patients with portopulmonary hypertension (POPH) have worse survival. Health disparities may contribute to these differences but have not been studied. We sought to compare socioeconomic factors in patients with POPH and IPAH and to determine whether socioeconomic status (SES) and/or POPH diagnosis were associated with treatment and healthcare utilization. We performed a cross-sectional study of adults enrolled in the Pulmonary Hypertension Association Registry. Patients with IPAH (n=344) and POPH (n=57) were compared. Compared with IPAH, patients with POPH were less likely to be college graduates (19.6 versus 34.9%, p=0.02) and more likely to be unemployed (54.7 versus 30.5%, p<0.001) and have an annual household income below poverty level (45.7 versus 19.0%, p<0.001). Patients with POPH had similar functional class, quality of life, 6MWD and mean pulmonary arterial pressure with a higher cardiac index. Compared with IPAH, patients with POPH were less likely to receive combination therapy (46.4 versus 62.2%, p=0.03) and endothelin receptor antagonists (ERAs) (28.6 versus 55.1%, p<0.001) at enrollment with similar treatment at follow-up. Patients with POPH had more emergency department (ED) visits (1.7±2.1 versus 0.9±1.2, p=0.009) and hospitalizations in the 6 months preceding enrollment (1.5±2.1 versus 0.8±1.1, p=0.02). Both POPH diagnosis and lower education level were independently associated with a higher number of ED visits. Compared to IPAH, patients with POPH have lower SES, are less likely to receive initial combination therapy and ERAs but have similar treatment at follow-up and have increased healthcare utilization.

Social determinants of health in pulmonary arterial hypertension patients in the United States: Clinician perspective and health policy implications

“…Experienced clinicians from PHCCCs across the US were highly aware of SDoH and shared how their patients' socioeconomic factors impacted their clinical care. Our study findings provide PAH clinicians' views that support emerging evidence indicating that PAH patients with negative SDoH correlated with poor health outcomes 9–12 . Talwar et al identified that lower income levels in PAH patients were associated with the worse disease as measured by higher WHO‐FC at initial evaluation 9 .…”

The Different Effects of Direct Bilirubin on Portopulmonary Hypertension and Idiopathic Pulmonary Arterial Hypertension

“…However, a study from the REVEAL registry, including a total of 3046 patients in which 100 were deemed as Asian or Pacific Islander, demonstrated that the race was not significantly associated with survival after age under 60 years adjustment [ 20 ]. Meanwhile, DuBrock's study [ 21 ] found that patients with POPH had lower socioeconomic status than patients with IPAH, in which lower education level would associate with more emergency department visits in American patients. Different survival among countries and regions requires more clinical investigations concerning the development of economy and medication, diets, mental situation, the spectrum of primary diseases, etc.…”

Gender dimension in cardio-pulmonary continuum

“…Pulmonary Hypertension Association Registry ( 83 ) reported that patients suffering from POPH (51% females) as compared to idiopathic PAH (75% females) have lower socioeconomic status because they are more likely to be unemployed, have lower education and lower-income further indirectly suggesting that gender-sensitive environment could have a significant impact on the disease outcome. In support, Wu et al ( 84 ) showed that a lower socioeconomic score determines a worse outcome and higher risk for mortality in idiopathic PAH, where the study population was up to 80% consisting of females.…”