Pulmonary Hypertension: Intensification and Personalisation of Combination Rx (PHoenix)
Pulmonary Arterial Hypertension
About this trial
This is an interventional treatment trial for Pulmonary Arterial Hypertension focused on measuring remote monitoring, therapeutic development, personalised medicine
Eligibility Criteria
Inclusion Criteria: Able to provide informed consent Age 18-80 years PAH which is idiopathic, heritable or associated with drugs, toxins or connective tissue disease Stable PAH therapeutic regime comprising any combination of ERA and PDE5i for at least 1 month prior to screening (unless unable to tolerate therapy) WHO functional class III Resting mPAP ≥20 mmHg, pulmonary capillary wedge pressure ≤15 mmHg, pulmonary vascular resistance ≥2 Wood Units measured by right heart catheterisation at time of diagnosis 6MWT >50m at entry Estimated glomerular filtration rate (eGFR)>30 ml/min/1.73 m² at entry (Appendix C) Inadequate treatment response (clinically determined) Exclusion Criteria: Unable to provide informed consent Pregnancy Unprovoked pulmonary embolism (at any time) Acute infection at time of screening (rescreening is permitted) PAH due to human immunodeficiency virus, portal hypertension, schistosomiasis, congenital heart disease Pulmonary hypertension due to left heart, lung, thromboembolic or unclear/multifactorial disease (Group II-V) Unable to tolerate aspirin or P2Y12 inhibitor Hypersensitivity to selexipag or riociguat Clinically-significant renal disease (eGFR≤30 ml/min/1.73m2) Anaemia (haemoglobin <10 g/dl) Left-sided heart disease and/or clinically significant cardiac disease, including but not limited to any of the following: aortic or mitral valve disease greater than mild aortic insufficiency; mild aortic stenosis; mild mitral stenosis; or moderate mitral regurgitation
Sites / Locations
- Sheffield Teaching Hospitals NHS FTRecruiting
Arms of the Study
Arm 1
Arm 2
Active Comparator
Active Comparator
Arm A (selexipag/riociguat)
Arm B (riociguat/selexipag)
Baseline - established PDE/ERA therapy Week 1 - initiate OPA (PDE/ERA/OPA therapy) Weeks 2-12 - uptitration of OPA to maximal therapy (PDE/ERA/OPA therapy) Weeks 13-14 - reduce OPA (PDE/ERA/OPA therapy) Week 15 - washout OPA (PDE/OPA therapy) Week 16 - washout PDE (ERA therapy) Week 17 - initiate sGCS (ERA/sGCS therapy) Weeks 18-27 - uptitration of sGCS to maximal therapy (ERA/sGCS therapy)
Baseline - established PDE/ERA therapy Week 1 - washout PDE (ERA therapy only) Week 2 - initiate sGCS (ERA/sGCS therapy) Weeks 3-12 - uptitration of sGCS to maximal therapy (ERA/sGCS therapy) Week 13 - reduce sGCS (ERA/sGCS therapy) Week 14 - reduce and washout sGCS (ERA therapy) Week 15 - initiate PDE (PDE/ERA therapy) Week 16 - initiate OPA (PDE/ERA/OPA therapy) Weeks 17-27 - uptitration of OPA to maximal therapy (PDE/ERA/OPA therapy)