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 Ford; Nadine Al-Naamani; Marc A Simon; Sula Mazimba; James R Runo; Murali Chakinala; Evelyn M Horn; John J Ryan; Robert P Frantz; Michael J Krowka

doi:10.1177/20458940211020913

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

Pulm Circ. 2021 May 17;11(3):20458940211020913. doi: 10.1177/20458940211020913. eCollection 2021 Jul-Sep.

Authors

Hilary M DuBrock¹, Charles D Burger², Sonja D Bartolome³, Jeremy P Feldman⁴, D Dunbar Ivy⁵, Erika B Rosenzweig⁶, Jeffrey S Sager⁷, Kenneth W Presberg⁸, Stephen C Mathai⁹, Matthew R Lammi¹⁰, James R Klinger¹¹, Michael Eggert¹², Teresa De Marco¹³, Jean M Elwing¹⁴, David Badesch¹⁵, Todd M Bull¹⁵, Linda M Cadaret¹⁶, Gautam Ramani¹⁷, Thenappan Thenappan¹⁸, H James Ford¹⁹, Nadine Al-Naamani²⁰, Marc A Simon^{21

22}, Sula Mazimba²³, James R Runo²⁴, Murali Chakinala²⁵, Evelyn M Horn²⁶, John J Ryan²⁷, Robert P Frantz²⁸, Michael J Krowka¹

Affiliations

¹ Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA.
² Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Mayo Clinic, Jacksonville, FL, USA.
³ Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX, USA.
⁴ Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Arizona Pulmonary Specialists, Ltd, Scottsdale, AZ, USA.
⁵ Division of Cardiology, Department of Pediatrics, Children's Hospital Colorado, Aurora, CO, USA.
⁶ Division of Cardiology, Department of Pediatrics, Columbia University Medical Center, New York, NY, USA.
⁷ Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Cottage Pulmonary Hypertension Center, Santa Barbara, CA, USA.
⁸ Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Medical College of Wisconsin, Milwaukee, WI, USA.
⁹ Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Johns Hopkins University, Baltimore, MD, USA.
¹⁰ Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Comprehensive Pulmonary Hypertension Center, Louisiana State University Health Sciences Center, New Orleans, LA, USA.
¹¹ Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Brown University, Providence, RI, USA.
¹² Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Sentara Cardiovascular Research Institute, Norfolk, VA, USA.
¹³ Division of Cardiology, Department of Internal Medicine, University of California San Francisco, San Francisco, CA, USA.
¹⁴ Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Cincinnati, Cincinnati, OH, USA.
¹⁵ Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Colorado, Denver, CO, USA.
¹⁶ Division of Cardiology, Department of Internal Medicine, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA, USA.
¹⁷ Division of Cardiology, Department of Internal Medicine, University of Maryland School of Medicine, Baltimore, MD, USA.
¹⁸ Division of Cardiology, Department of Internal Medicine, University of Minnesota, Minneapolis, MN, USA.
¹⁹ Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.
²⁰ Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Pennsylvania, Philadelphia, PA, USA.
²¹ Division of Cardiology, Department of Internal Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.
²² Division of Cardiology, Department of Internal Medicine, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
²³ Division of Cardiology, Department of Internal Medicine, University of Virginia Health System, Charlottesville, VA, USA.
²⁴ Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Wisconsin-Madison, Madison, WI, USA.
²⁵ Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Washington University at Barnes-Jewish, St. Louis, MO, USA.
²⁶ Division of Cardiology, Department of Internal Medicine, Weill Cornell Medical School, New York, NY, USA.
²⁷ Division of Cardiology, Department of Internal Medicine, University of Utah, Salt Lake City, UT, USA.
²⁸ Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA.

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.

Keywords: health-care utilization; pulmonary hypertension; socioeconomic status.