We selected patients with CTEPH (age 20–85 years) as confirmed by a pulmonary ventilation/perfusion scan, pulmonary angiography and a chest computed tomography scan, two or more of which revealed areas of deficient pulmonary blood flow. Pulmonary haemodynamic variables at rest, as determined by right heart catheterisation, were set as the baseline. The mean pulmonary arterial pressure (mPAP) was set at ≥25 mmHg; the pulmonary artery wedge pressure (PAWP) was set at ≤15 mmHg; and PVR was set at >360 dyn·s·cm−5. The population consisted of patients who could not undergo PEA due to the presence of organised peripheral thrombus. This study also included patients who could not undergo PEA due to their high risk (e.g. comorbidities or old age), or for other reasons (e.g. refusal to undergo surgery). These disease classifications were assessed by each investigator at their own institution. The population also consisted of some patients who had persistent or recurrent PH after PEA or BPA.
Those who had received prostacyclin and/or its derivatives were excluded. Concomitant use of riociguat, an endothelin receptor antagonist (ERA), a phosphodiesterase-5 inhibitor or a calcium antagonist was allowed if the doses administered had been stable for ≥90 days before the baseline right heart catheterisation and it was maintained until the end of this double-blind study. While patients who had undergone PEA and/or BPA were included, PEA and BPA were not allowed during the study. Details of the inclusion and exclusion criteria are provided in thesupplementary material .
This study was conducted in accordance with the ethical principles set out by the institutional human ethics committees of the participating facilities or regions and the Declaration of Helsinki. The study design was approved by the institutional review board at each study site, including the National Cerebral and Cardiovascular Centre (reference number #924). All subjects provided written informed consent to participate in the study.
Those who had received prostacyclin and/or its derivatives were excluded. Concomitant use of riociguat, an endothelin receptor antagonist (ERA), a phosphodiesterase-5 inhibitor or a calcium antagonist was allowed if the doses administered had been stable for ≥90 days before the baseline right heart catheterisation and it was maintained until the end of this double-blind study. While patients who had undergone PEA and/or BPA were included, PEA and BPA were not allowed during the study. Details of the inclusion and exclusion criteria are provided in the
This study was conducted in accordance with the ethical principles set out by the institutional human ethics committees of the participating facilities or regions and the Declaration of Helsinki. The study design was approved by the institutional review board at each study site, including the National Cerebral and Cardiovascular Centre (reference number #924). All subjects provided written informed consent to participate in the study.
