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

Hilary M. DuBrock, Charles D. Burger, Sonja D. Bartolome, Jeremy P. Feldman, D. Dunbar Ivy, Erika B. Rosenzweig, Jeffrey S. Sager, Kenneth W. Presberg, Stephen C. Mathai, Matthew R. Lammi, James R. Klinger, Michael Eggert, Teresa De Marco, Jean M. Elwing, David Badesch, Todd M. Bull, Linda M. Cadaret, Gautam Ramani, Thenappan Thenappan, H. James FordNadine Al-Naamani, Marc A. Simon, Sula Mazimba, James R. Runo, Murali Chakinala, Evelyn M. Horn, John J. Ryan, Robert P. Frantz, Michael J. Krowka, behalf of the PHAR Investigators on behalf of the PHAR Investigators

Research output: Contribution to journalArticlepeer-review

8 Scopus citations

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 and/or POPH diagnosis were associated with treatment and health-care 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% vs. 34.9%, p = 0.02) and more likely to be unemployed (54.7% vs. 30.5%, p < 0.001) and have an annual household income below poverty level (45.7% vs. 19.0%, p < 0.001). Patients with POPH had similar functional class, quality of life, 6-min walk distance, 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% vs. 62.2%, p = 0.03) and endothelin receptor antagonists (28.6% vs. 55.1%, p < 0.001) at enrollment with similar treatment at follow-up. Patients with POPH had more emergency department visits (1.7 ± 2.1 vs. 0.9 ± 1.2, p = 0.009) and hospitalizations in the six months preceding enrollment (1.5 ± 2.1 vs. 0.8 ± 1.1, p = 0.02). Both POPH diagnosis and lower education level were independently associated with a higher number of emergency department visits. Compared to IPAH, patients with POPH have lower socioeconomic status, are less likely to receive initial combination therapy and endothelin receptor antagonists but have similar treatment at follow-up, and have increased health-care utilization.

Original languageEnglish (US)
JournalPulmonary Circulation
Volume11
Issue number3
DOIs
StatePublished - Jun 24 2021

Bibliographical note

Funding Information:
The Pulmonary Hypertension Association Registry (PHAR) is supported by Pulmonary Hypertension Care Centers, Inc., a supporting organization of the Pulmonary Hypertension Association. The authors thank the other investigators, the staff, and particularly the participants of the PHAR for their valuable contributions. A full list of participating PHAR sites and institutions can be found at www.PHAssociation.org/PHAR. The authors would like to acknowledge the PHAR site principal investigators: Roblee P. Allen, MD, Jeffrey Fineman, MD, Roham T. Zamanian, MD, Raymond Foley, MD, Paul Boyce, MD, MPH, Michael Duncan, MD, Timothy Williamson, MD, Wesley McConnell, MD, Stacy Mandras, MD, Paul Hassoun, MD, Rana Awdish, MD, R. James White, MD, PhD, Kishan Parikh, MD, Terry Fortin, MD, MS, Russel Hirsch, MD, Jeffrey C. Robinson, MD, Steven M. Kawut, MD, Amresh Raina, MD, Stephen Chan, MD, PhD, Corey Ventetuolo, MD, MS, Abhijit Raval, MD, John Swisher, MD, PhD, Eric D. Austin, MD, Anna R. Hemnes, MD, Nidhy Varghese, MD, Sahil Bakshi, MD, Oksana A. Shlobin, MD, Steven Nathan, MD, Daniel Grinnan, MD, Peter Leary, MD, PhD, and Dianne L. Zwicke, MD. This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Funding Information:
The Pulmonary Hypertension Association Registry (PHAR) is supported by Pulmonary Hypertension Care Centers, Inc., a supporting organization of the Pulmonary Hypertension Association. The authors thank the other investigators, the staff, and particularly the participants of the PHAR for their valuable contributions. A full list of participating PHAR sites and institutions can be found at www.PHAssociation.org/PHAR . The authors would like to acknowledge the PHAR site principal investigators: Roblee P. Allen, MD, Jeffrey Fineman, MD, Roham T. Zamanian, MD, Raymond Foley, MD, Paul Boyce, MD, MPH, Michael Duncan, MD, Timothy Williamson, MD, Wesley McConnell, MD, Stacy Mandras, MD, Paul Hassoun, MD, Rana Awdish, MD, R. James White, MD, PhD, Kishan Parikh, MD, Terry Fortin, MD, MS, Russel Hirsch, MD, Jeffrey C. Robinson, MD, Steven M. Kawut, MD, Amresh Raina, MD, Stephen Chan, MD, PhD, Corey Ventetuolo, MD, MS, Abhijit Raval, MD, John Swisher, MD, PhD, Eric D. Austin, MD, Anna R. Hemnes, MD, Nidhy Varghese, MD, Sahil Bakshi, MD, Oksana A. Shlobin, MD, Steven Nathan, MD, Daniel Grinnan, MD, Peter Leary, MD, PhD, and Dianne L. Zwicke, MD.

Publisher Copyright:
© The Author(s) 2021.

Keywords

  • health-care utilization
  • pulmonary hypertension
  • socioeconomic status

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