Title

Health-related quality of life and hospitalizations in chronic thromboembolic pulmonary hypertension versus idiopathic pulmonary arterial hypertension: An analysis from the Pulmonary Hypertension Association Registry (PHAR)

Authors

Jasleen Minhas, Division of Pulmonary, Allergy and Critical Care Medicine, University of Pennsylvania, Philadelphia, PA, USA.
Sai Prasanna Narasimmal, Division of Pulmonary, Allergy and Critical Care Medicine, University of Pennsylvania, Philadelphia, PA, USA.
Todd M Bull, Division of Pulmonary Sciences & Critical Care, University of Colorado, Denver, CO, USA.
Teresa De Marco, Division of Cardiology, University of California, San Francisco, CA, USA.
John Wesley McConnell, Norton Healthcare, Louisville, KY, USA.
Matthew R. Lammi, Division of Pulmonary and Critical Care Medicine, Louisiana State University, New Orleans, LO, USA.
Thenappan Thenappan, Division of Cardiology, University of Minnesota, Minneapolis, MN, USA.
Jeremy P Feldman, Division of Pulmonary and Critical Care Medicine, Arizona Pulmonary Specialists, Phoenix, AZ, USA.
Jeffrey S Sager, Division of Pulmonary and Critical Care Medicine, Cottage PH center, Santa Barbara, CA, USA.
David B Badesch, Division of Pulmonary Sciences & Critical Care, University of Colorado, Denver, CO, USA.
John J. Ryan, Division of Cardiology, University of Utah, Salt Lake City, UT, USA.
Daniel C Grinnan, Division of Pulmonary and Critical Care Medicine, Virginia Commonwealth University, Richmond, VA, USA.
Dianne Zwicke, Advocate Aurora Health
Evelyn M Horn, Division of Cardiology, Weill Conrell Medicine, New York City, NY, USA.
Jean M. Elwing, Division of Pulmonary and Critical Care Medicine, University of Cincinnati, Cincinnati, OH, USA.
John E. Moss, Department of Pulmonary Medicine and Division of Critical Care, Mayo Clinic, Jacksonville, FL, USA.
Michael Eggert, Division of Pulmonary and Critical Care Medicine, Sentara Hospital, Norfolk, VA, USA.
Oksana A. Shlobin, Division of Pulmonary and Critical Care Medicine, Inova Fairfax Hospital, Advanced Lung Disease and Transplant, Falls Church, VA, USA.
Robert P Frantz, Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA.
Sonja D Bartolome, Division of Pulmonary and Critical Care Medicine, University of Texas, Southwestern Medical Center, Dallas, TX, USA.
Stephen C. Mathai, Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Sula Mazimba, Division of Cardiology, University of Virginia Health System, Charlottesville, VA, USA.
Steven C Pugliese, Division of Pulmonary, Allergy and Critical Care Medicine, University of Pennsylvania, Philadelphia, PA, USA.
Nadine Al-Naamani, Division of Pulmonary, Allergy and Critical Care Medicine, University of Pennsylvania, Philadelphia, PA, USA.

Affiliations

Aurora Cardiovascular Services

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, morbid, potentially curable subtype of pulmonary hypertension that negatively impacts health-related quality of life (HRQoL). Little is known about differences in HRQoL and hospitalization between CTEPH patients and idiopathic pulmonary arterial hypertension (IPAH) patients. Using multivariable linear regression and mixed effects models, we examined differences in HRQoL assessed by emPHasis-10 (E10) and SF-12 between CTEPH and IPAH patients in the Pulmonary Hypertension Association Registry, a prospective multicenter cohort of patients newly evaluated at a Pulmonary Hypertension Care Center. Multivariable negative binomial regression models were used to estimate incidence rate ratios (IRR) for hospitalization amongst the two groups. We included 461 IPAH patients and 169 CTEPH patients. Twenty-one percent of CTEPH patients underwent pulmonary thromboendarterectomy (PTE) before the end of follow-up. At baseline, patients with CTEPH had significantly worse HRQoL (higher E10 scores) (ß 2.83, SE 1.11, p = 0.01); however, differences did not persist over time. CTEPH patients had higher rates of hospitalization (excluding the hospitalization for PTE) compared to IPAH patients after adjusting for age, sex, body mass index, WHO functional class and six-minute walk distance (IRR 1.66, 95%CI 1.04-2.65, p = 0.03). CTEPH patients who underwent PTE had improved HRQoL as compared to those who were medically managed, but patients who underwent PTE were younger, had higher cardiac outputs and greater six-minute walk distances. In this large, prospective, multicenter cohort, CTEPH patients had significantly worse baseline HRQoL and higher rates of hospitalizations than those with IPAH. CTEPH patients who underwent PTE had significant improvements in HRQoL.

Document Type

Article

PubMed ID

34671455

Link to Full Text

 

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