Abstract
Background: Pulmonary Hypertension (PH) is associated with significant morbidity and mortality, greatly affecting patients’ quality of life (QoL). The emPHasis-10 (E10) questionnaire has recently been proposed as a short yet effective means of routinely assessing QoL in PH patients. We assessed the relationship between E10 score and mortality in a large cohort of patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic PH (CTEPH).
Methods and Results: 2487 E10 scores were collected from 687 patients (239 male, 34.8%, mean age 51.7±18.4 years). The vast majority of patients had PAH (n=534, 80.9%): 109(19.9%) with iPAH/Heritable/Drug-related PH, 314(57.2%) congenital heart disease (CHD) and 111(20.2%) connective tissue disease (CTD). CTEPH was present in 131(19.1%) patients.
Over a median 2.1 years, 87 patients died. The first E10 score was strongly associated with the risk of death in the overall population (HR 1.59, 95%CI:1.31-1.94, p<0.0001), even when adjusting for age and functional class (HR 1.27, 95%CI:1.03-1.56, p=0.03). E10 score was a predictor of death in the iPAH/Heritable/Drug-related (Logrank p=0.02) and CHD groups (p=0.0002), but E10 did not relate to death in CTD or CTEPH patients. When using repeat E10 scores for each patient in a “start-stop” Cox model, E10 was a significant predictor of death in the overall population (HR 1.62, 95%CI:1.35-1.94, p<0.0001), iPAH/Heritable/Drug-related PH (HR 1.72, 95% CI:1.17-2.51, p=0.006), and the CHD population (HR 2.03, 95% CI:1.43-2.88, p<0.0001).
Conclusions: E10 score is a strong predictor of outcome in routine clinical practice.
Footnotes
Cite this article as: European Respiratory Journal 2018 52: Suppl. 62, OA273.
This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).
- Copyright ©the authors 2018