Long-term Survival and Quality of Life: Results From the United States Chronic Thromboembolic Pulmonary Hypertension Registry
Background: Chronic thromboembolic pulmonary hypertension (CTEPH) causes significant morbidity and mortality, but long-term outcomes from contemporary multicenter studies are lacking. Research Question: How are survival and health-related quality of life characterized in patients with CTEPH who are classified as inoperable, who are operable but have not undergone surgery, and who have undergone pulmonary thromboendarterectomy surgery? Study Design and Methods: Patients with CTEPH recruited from 30 US sites from 2015 through 2018 completed the 36-item Short Form Health Survey (SF-36) and emPHasis-10 survey at baseline and 6-month intervals. Mixed model repeated measures analysis was used to compare between-group differences in score up to 2 years vs baseline. Multivariable Cox proportional hazards models were used to analyze survival by CTEPH group. Results: Seven hundred fifty patients with a median age of 59 years were enrolled; 566 patients, 88 patients, and 96 patients were in the operated, operable but no surgery, and inoperable groups, respectively. Survival at 1, 2, and 3 years was 93%, 91%, and 87%, respectively. Patients in the inoperable and the operable but no surgery groups showed higher mortality rates relative to the operated group (hazard ratios, 2.10 [95% CI, 1.17-3.77] and 2.19 [95% CI, 1.20-3.99], respectively). The EmPHasis-10 and both SF-36 component scores improved during follow-up, with larger increases for the operated group (P <.05, unadjusted and adjusted vs inoperable and operable but no surgery groups at all time points up to 2 years for the SF-36 physical component score and EmPHasis-10 and at some time points for the SF-36 mental component score). Interpretation: Better survival and quality-of-life outcomes were observed in patients undergoing pulmonary thromboendarterectomy.
