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)

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.

Keywords: hospitalizations; pulmonary thromboendarterectomy; quality of life.

Fig. 1.

Flow diagram for study participants.

Fig. 2.

Expected mean estimates for patients with IPAH vs. CTEPH of (a) emPHasis-10 scores, (b) Short Form-12 physical component and (c) Short Form-12 mental component scores at the time of enrollment adjusted for age, sex, BMI, six-minute walk distance and WHO functional class.

Fig. 3.

Expected mean estimates for patients with IPAH vs. CTEPH of (a) emPHasis-10 scores, (b) Short Form-12 physical component and (c) Short Form-12 mental component scores over time adjusted for age, sex, BMI, six-minute walk distance, WHO functional class and pulmonary thromboendarterectomy status.

Fig. 4.

Cumulative mean hospitalizations over time in patients with chronic thromboembolic pulmonary hypertension (CTEPH) vs. those with idiopathic pulmonary arterial hypertension (IPAH).

Fig. 5.

Spaghetti plot of emPHasis-10 scores for individual patients with chronic thromboembolic pulmonary hypertension (CTEPH) (a) post-pulmonary thromboendarterectomy  (PTE) and (b) medical management only.

Fig. 6.

Expected unadjusted mean estimates for patients with CTEPH who underwent pulmonary thromboendarterectomy (PTE) vs. those who were medically managed of (a) emPHasis-10 scores, (b) Short Form-12 physical component and (c) Short Form-12 mental component scores over time.