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)

Jasleen Minhas, Sai Prasanna Narasimmal, Todd M. Bull, Teresa De Marco, John Wesley McConnell, Matthew R. Lammi, Thenappan Thenappan, Jeremy P. Feldman, Jeffrey S. Sager, David B. Badesch, John J. Ryan, Daniel C. Grinnan, Dianne Zwicke, Evelyn M. Horn, Jean M. Elwing, John E. Moss, Michael Eggert, Oksana A. Shlobin, Robert P. Frantz, Sonja D. BartolomeStephen C. Mathai, Sula Mazimba, Steven C. Pugliese, Nadine Al-Naamani

Research output: Contribution to journalArticlepeer-review

3 Scopus citations

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.

Original languageEnglish (US)
JournalPulmonary Circulation
Volume11
Issue number4
DOIs
StatePublished - 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 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 .

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 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 author(s) disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: This work was supported by the NIH/NHLBI (grant number K23 HL141584 (NAN) and T32-HL007891 (JM)).

Funding Information:
The author(s) disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: This work was supported by the NIH/NHLBI (grant number K23 HL141584 (NAN) and T32-HL007891 (JM)).

Publisher Copyright:
© The Author(s) 2021.

Keywords

  • hospitalizations
  • pulmonary thromboendarterectomy
  • quality of life

PubMed: MeSH publication types

  • Journal Article

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