PHAR data has already yielded numerous academic contributions, including peer-reviewed publications, abstracts and conference presentations. Additional literature related to the PH Care Centers (PHCC) initiative also reflects the program’s growing impact on pulmonary hypertension research and care. See below for selected publications and contributions.

  • The frequency and predictors of pulmonary rehabilitation referrals among patients with pulmonary arterial hypertension: An analysis of the Pulmonary Hypertension Association Registry
    There is limited data regarding pulmonary rehabilitation (PR) in PAH. The authors used PHAR data for a retrospective cohort study of adults with PAH comparing patients referred to PR by the time of the second follow-up visit compared to patients never referred to PR. Despite well-established benefits, referral to PR is low among patients with PAH. Patients referred to PR were older, primarily female and had worse functional class and risk stratification. In a subgroup analysis, less than half of patients referred to PR reported participation in PR within the preceding 6 months. PR participation was associated with improvements in HRQOL.
  • Medication Non-Adherence in Patients with Pulmonary Arterial Hypertension: The Pulmonary Hypertension Association Registry (PHAR)
    Self-reported PAH medication non-adherence was captured at PHAR enrollment and during follow-up visits were analyzed. Predictors of non-adherence were modeled and outcomes associated with non-adherence were modeled. The rate of self-reported non-adherence in PHAR registrants was low but was associated with male sex and several social determinants of health. While complexity or type of PAH regimen did not appear to influence non-adherence, non-adherence was associated with numerous adverse patient-centered outcomes, including higher healthcare utilization and worse HRQoL.
  • Ambient Air Pollution Exposure and Mortality in the Pulmonary Hypertension Association Registry
    The authors evaluiated the effects of long-term ambient air pollution exposure on survival and lung transplantation in PAH and CTEPH from PHAR data. Long-term ambient air pollution exposure was not significantly associated with survival in PHAR patients with PAH or CTEPH.
  • The landscape of referrals for lung transplantation in pulmonary arterial hypertension: a report from the Pulmonary Hypertension Association Registry
    The authors used PHAR to describe the current landscape of referrals for lung transplantation in patients with PAH. Only 12% of PHAR participants were referred tor transplantation. Of those, only 18-29% with functional class 4, REVEAL Lite-2 high risk scores, or 2022 ESC/ERS high-risk disease were reffered to transplant Of those referred, 30% underwent transplantation and 21% dieed without transplantation. The authors concluded that Rates of referral for lung transplantation in patients with PAH remain low and occur too late. Increased awareness of the benefit of early referral is necessary.
  • Getting Started Early in Your Pulmonary Hypertension Career
    Summary from PHCC and PHAR session at 2024 PHA Conference & Scientific Sessions. Authors describe PHAR as a unique and highly effective career development mechanism for early-career members of PHA. PHAR accomplishing this through (1) lowering the barrier to entry for trainees and early-career members and (2) providing an abundance of mentorship and support.

  • Regional variation in pulmonary arterial hypertension in the United States: The PHA Registry.
    Fling et al. Ann Am Thorac Soc 2023 September
    The authors looked at differences in the characteristics and outcomes for participants in PHAR across US census regions. Adults with PAH in PHAR were divided into regional groups (Northeast, South, Midwest, West) and baseline differences between census regions were studied. Treatment characteristics varied by census region and mortality appeared to be lower in PHAR participants in the West. The differences were not easily explained by differences in demographic characteristics, PAH etiology, baseline severity, baseline medication regimen, or disease prevalence.
  • Minimal Clinically Important Difference in the 6-Minute-Walk Distance for Patients with Pulmonary Arterial Hypertension
    Moutchia et al. American Journal of Respiratory and Critical Care Medicine 2023 April
    Six-minute walk distance is an important clinical and research metric in PAH but there is no consensus about what minimal change in 6MWD is clinically significant. The authors performed data meta-analysis using data from clinical trials and validated results with PHAR data to determine the minimal clinically important differences in 6MWD that could be used in future clinical trial design.
  • Impact of the COVID‐19 pandemic on chronic disease management and patient reported outcomes in patients with pulmonary hypertension: The Pulmonary Hypertension Association Registry
    Mayer et al. Pulmonary Circulation 2023 April
    Authors looked at the impact of COVID-19 on PH disease management and patient reported outcomes in PHAR. Results showed minimal impact of COVID on PH related outcomes. PAH patients did not experience delays in access to medications, increased emergency room visits or night in hospitals or worsening mental health metrics. The authors concluded that this may be due to PHAR patients receiving access to high quality care at PHA-accredited care centers. Patients on publicly sponsored insurance had higher healthcare utilization and showed more severe disease compared to privately uninsured patients irrespective of the pandemic.

  • Palliative care referrals in patients with pulmonary arterial hypertension: The Pulmonary Hypertension Association Registry
    Rohlfing et al. Respiratory Medicine 2023 January in print, November 2022 online
    Authors used PHAR data to characterize PAH patients who were referred to palliative care. Data was used to identify predictors of referrals. Results showed that PAH patients were not frequently referred for palliative care (5.8%). Those who were referred, were sicker patients with lower quality of life scores and close to the end of life.
  • Anticoagulation in pulmonary arterial hypertension-association with mortality, healthcare utilization, and quality of life: The Pulmonary Hypertension Association Registry (PHAR)
    Garry et al. J Heart Lung Transplant 2022 August
    Routine long-term anticoagulation in PAH is controversial and shown to be beneficial or neutral in idiopathic disease (IPAH) and neutral-to-harmful in connective tissue disease (CTD-PAH). Authors sought to examine the association between anticoagulation and mortality, healthcare utilization, and quality of life (QoL) in PAH using PHAR data. Anticoagulation was not associated with higher mortality but is associated with increased healthcare utilization in the PHAR. PAH-specific QoL may be worse in patients receiving anticoagulation.
  • Hispanic ethnicity and social determinants of health in pulmonary arterial hypertension: the Pulmonary Hypertension Association Registry
    Bernado et al. Annals of the American Thoracic Society 2022 September
    Publication looking at Hispanic ethnicity and social determinants. Hispanic PAH patients in PHAR had lower levels of social determinants such as access to health care, education level, access to health insurances and lower income level. Hispanic PHAR patients have worse disease severity and survival. However, Hispanic ethnicity was not associated with differences in survival after adjusting for social determinants of health and other factors. Social determinants of health are important to consider when assessing the association between ethnicity and outcomes in PAH.
  • Prediction of Patient Outcomes through Social Determinants of Health: The Pulmonary Hypertension Association Registry
    Evaluation Grinnan D., Kang L., DeWilde C., Badesch D. et al. Pulmonary Circulation 2022 July
    Outcomes of patients with pulmonary arterial hypertension (PAH) may be associated with social determinants of health and other baseline patient characteristics. Before this manuscript, there was not a model to predict patient outcomes in PAH based on SDOH. Using information from the PHAR, the authors developed a model to predict an important composite patient outcome based on SDOH and other patient characteristics. Variables included in our model are race, gender, ethnicity, household income, level of education, age, body mass index, drug use, alcohol use, marital status and type of health insurance.
  • Mortality in Pulmonary Arterial Hypertension in the Modern Era: Early Insights from The Pulmonary Hypertension Association Registry
    Thenapppan Thenappan (corresponding author) Journal of the American Heart Association 2022 May
    This is the first current U.S. analysis of PAH mortality rates within the U.S. as previous studies are from more than 10 years ago. Authors sought to determine contemporary mortality data in PAH in the U.S. using the PHAR. In treatment-naïve patients, initial combination therapy was associated with better 1-year survival than monotherapy. When compared with mortality rates from other registries, lower mortality was observed with idiopathic and heritable PAH in the PHAR. However, due to enrollment differences between registries, the causal reason for this is not clear. The authors concluded that mortality in the intermediate- and high-risk patients with PAH remains unacceptably high in the PHAR, suggesting the importance for early diagnosis, aggressive use of available therapies, and the need for better therapeutics.
  • Human immune deficiency virus-associated pulmonary arterial hypertension, a report from the Pulmonary Hypertension Association Registry
    Kolaitis NA., et al. American Journal of Respiratory and Critical Care Medicine 2022 February
    There is limited research characterizing HIV-PAH and directly comparing HIV-PAH patients with HIV-PAH to types of PAH, so this study provides insight into our understanding of HIV-PAH. The authors presented data that HIV-PAH might represent a higher risk patient with increased rate of methamphetamine use, worse hemodynamics, and worsening kidney function compared to idiopathic PAH.

  • Residence at Moderately High-Altitude ant its Relationship with WHO Group 1 Pulmonary Arterial Hypertension Symptom Severity and Clinical Characteristics: The Pulmonary Hypertension Association
    Registry
    Fakhari S., Hannon K., Moulden K. et al. Pulmonary Circulation 2020 November
    Examined the relationship between living at moderately high altitude and PAH from PHAR data. Patients with pulmonary arterial hypertension who live at moderately high altitude have a higher pulmonary vascular resistance and are more likely to need supplemental oxygen. Data supports a “high-altitude phenotype” of PAH.
  • Obesity in Pulmonary Arterial Hypertension (PAH): The Pulmonary Hypertension Association Registry (PHAR)
    Min J., Feng R., Badesch D. et al. Annals of the American Thoracic Society 2020
    October Obesity has been associated with PAH, but the impact on outcomes such as QoL, hospitalizations, and survival was not well understood. Overweight and obese PAH patients in PHAR had worse HRQoL but had better transplant-free survival compared to normal weight PAH PHAR patients.
  • Age-related differences in hemodynamics and functional status in pulmonary arterial hypertension: Baseline results from the Pulmonary Hypertension Association Registry
    DesJardin JT., Kolaitis NA., Kime N. et al. Heart Lung Transplant 2020 September. Limited data existed on age-related differences in PAH patients with hemodynamic and functional assessments. Authors analyzed PHAR data and showed that older PAH patients had lower pulmonary artery compliance and worse right ventricular performance. The authors concluded that age influences right ventricular loading conditions and the response of the right ventricle to increased afterload.
  • American Thoracic Society (ATS) 2020:
    • Derivation of the Pulmonary Hypertension Association Registry Evaluation
    • Health disparities and treatment patterns in portopulmonary hypertension and idiopathic pulmonary arterial hypertension: analysis of The Pulmonary Hypertension Association Registry
    • Hospitalization In Connective Tissue Disease Associated Pulmonary Arterial Hypertension: The Pulmonary Hypertension Association Registry
    • Pulmonary Artery Compliance In Pulmonary Arterial Hypertension And Chronic Thromboembolic Pulmonary Hypertension: The Pulmonary Hypertension Association Registry
    • Quality Of Life Of Patients With Chronic Thromboembolic Pulmonary Hypertension (CTEPH) And Idiopathic Pulmonary Arterial Hypertension (IPAH): The Pulmonary Hypertension Association Registry (PHAR)
    • Risk Prediction Models Are Associated With Patient Centered Outcomes In PAH: The Pulmonary Hypertension Association Registry