Long-term effects of medical treatment in patients with chronic thromboembolic pulmonary hypertension

Background The New International Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Registry, conducted between 2015 and 2019, revealed the significant impact of pulmonary endarterectomy (PEA) and balloon pulmonary angioplasty (BPA) on the long-term survival of CTEPH patients. The objective of the current prespecified analysis was to evaluate the effect of medical treatment, including the approved oral guanylate cyclase stimulator (sGC) riociguat, in patients with or without mechanical intervention. Methods 1009 newly diagnosed patients were included in the study, with recruitment conducted at 34 centres across 20 countries. At the outset of the registry, sGC was available in 85% of the countries involved. Results 52% of all patients included were treated with pulmonary hypertension (PH) drugs. The proportion of patients receiving PH drugs was 38% among the 605 patients who underwent PEA, 78% among the 185 patients who underwent BPA, and 76% among the 219 remaining patients who did not undergo PEA or BPA. In the BPA and 'no PEA/BPA' groups, three-year survival was superior in patients who received sGC in comparison to other PH drugs. Cox regression confirmed that sGC treatment was associated with reduced mortality in the global cohort, in the BPA group and in the 'no PEA/BPA’ group. Conclusion This international CTEPH registry suggests that, although an increasing proportion of patients with CTEPH benefited from mechanical treatment, medical treatment with sGC may be associated with a survival advantage in patients undergoing BPA or no intervention. These observations confirm the results of previous randomised controlled trials in a real-world setting.