Functional and Quality of Life Improvements in Treated Patients with Chronic Thromboembolic Pulmonary Hypertension

Abstract

Purpose Chronic thromboembolic hypertension (CTEPH) is a form of pulmonary hypertension (PH) best treated surgically, and evidence in support of targeted therapy in inoperable patients is weak. This study aimed to demonstrate the effects of targeted therapy in patients with CTEPH in whom pulmonary endarterectomy (PEA) was not feasible. Methods and Materials A retrospective audit of CTEPH patients in the Newcastle PH Clinic was conducted. Six-minute walk distance (6MWD), Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) scores, and pulmonary hemodyamics were collected. Changes in 6MWD and CAMPHOR six months after intervention (surgery or starting of targeted therapies) were evaluated. Patients were stratified as inoperable patients (distal disease, patient preference, and comborid conditions), patients with residual PH post-PEA, or those with no PH post-PEA. Means were compared using student’s t-test; p-values Results Of 160 patients diagnosed with CTEPH, serial CAMPHOR and 6MWD data have been collected on the last 82, of which 37 were inoperable and on targeted therapy (Group A), 12 underwent PEA with residual PH (Group B), and 33 underwent PEA with no residual PH (Group C). Baselines were similar, except 6MWD and age between Group A and Group C (mean 241m vs 325m, p=0.003 and 64.4 vs 54.5 years, p=0.002). Mean 6MWD increased 49 m in Group A, 67 m in Group B, and 109 m in Group C (p Conclusions In inoperable CTEPH, targeted therapy can improve 6MWD and WHO functional class, but perhaps not quality of life. In contrast, patients undergoing PEA with normalization of pulmonary hemodynamics have improved 6MWD, WHO functional class, and quality of life.