Patients With CTEPH Have Worse Baseline HRQOL Than Patients With IPAH

At baseline, patients with chronic thromboembolic pulmonary hypertension (CTEPH) had worse health-related quality of life (HRQOL) than patients with idiopathic pulmonary arterial hypertension (IPAH), but over time the scores were similar.

The differences between patients with chronic thromboembolic pulmonary hypertension (CTEPH) and idiopathic pulmonary arterial hypertension (IPAH) are not well understood, but a new study has found that patients with CTEPH have significantly worse baseline health-related quality of life (HRQUL and high rates of hospitalization. The findings were published in Pulmonary Circulation.

Patients with CTEPH and pulmonary arterial hypertension are often treated with the same therapies, and clinical studies often group patients with CTEPH and IPAH together; however, the differences in HRQOL and hospitalization between the 2 groups has not been studied and little is known. The authors used data from the Pulmonary Hypertension Association Registry (PHAR), a large, prospective, multicenter cohort study, to investigate the differences.

HRQOL was assessed during the initial visit and at follow-up visits using 2 questionnaires: the medical outcome short form-12 (SF-12) and the emphasis-10 (E10). At the initial visit, demographics, clinical and social history, measurements of exercise performance, and hemodynamics via right heart catheterizations were recoded, and patients were followed at approximately 6-month intervals. At follow-ups, patients reported all-cause hospitalizations since their last visit.

A total of 1361 patients were enrolled in PHAR, and 630 (73% IPAH and 27% CTEPH) were included in this study. At baseline, patients with IPAH were younger (mean age 55 years vs 58 years), more likely to be female (76%), and were a majority non-Hispanic White (71%). The CTEPH group had a higher proportion of Black patients (24.3% vs 11.3%) compared with the IPAH group. Otherwise, there were no differences in education level, household income, employment, and marital status.

The researchers also found patients with CTEPH had:

  • Lower median mean pulmonary artery pressures
  • Lower pulmonary vascular resistance
  • Higher cardiac output

Only slightly more than half (54%) of patients with CTEPH were receiving anticoagulation when they enrolled, but that increased to 95% no follow-up. Patients with CTEPH were most commonly treated with riociguat and anticoagulation therapy compared with patients with IPAH who were treated with phosphodiesterase-5 inhibitors, endothelin receptor antagonists, or a combination of both.

Adjusting for age, sex, body mass index (BMI), 6-minute walk distance, and World Health Organization (WHO) functional class, CTEPH patients had worse HRQOL than patients with IPAH. The 2 groups had no difference in the baseline mental or physical components of the SF-12. However, over time there were no differences in HRQOL score.

Ultimately 515 patients had at least 1 subsequent follow-up visit and during the follow-ups there were 939 hospitalizations recorded. After adjustment for age, sex, BMI, 6-minute walk distance, WHO functional class, and pulmonary thromboendarterectomy (PTE) status, patients with CTEPH had a higher incidence rate ratio of all-cause hospital admissions than patients with IPAH.

By the end of the follow-up, 22% of patients with CTEPH underwent PTE and 78% were managed medically. Patients who underwent PTE were younger (mean age 54 years) and more likely to be male (62%), and they also had a higher cardiac output and worse HRQOL prior to PTE. After PTE, these patients had improved HRQOL and greater 6-minute walk test. This subgroup of patients with CTEPH who underwent PTE may have driven the overall improved HRQOL scores for patients with CTEPH that resulted in HRQOL scores being similar over time between the 2 groups, the authors noted.

“The worse baseline quality of life and higher hospitalization rate of CTEPH patients present an opportunity for improvement in clinical management of these patients,” they concluded.


Minha J, Narasimmal SP, Bull TM, et al. 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). Pulm Circ. 2021;11(4):20458940211053196. doi:10.1177/20458940211053196