Right Ventricular Compensation With Sotatercept: A Prospective Single Arm Open Label Phase
Pulmonary arterial hypertension (PAH) is a progressive disease characterized by vascular remodelling resulting in elevated pressures in the pulmonary artery (PA). This elevated pressure ultimately leads to fulminant right heart failure. Current therapeutic options are limited and are centred around vasodilatory medicat
| Condition(s) | Pulmonary Arterial Hypertension PAH, Pulmonary Arterial Hypertension WHO Group I, Pulmonary Arterial Hypertension |
|---|---|
| Status | Recruiting |
| Phase | Phase 4 |
| Study type | Interventional |
| Summary | Pulmonary arterial hypertension (PAH) is a progressive disease characterized by vascular remodelling resulting in elevated pressures in the pulmonary artery (PA). This elevated pressure ultimately leads to fulminant right heart failure. Current therapeutic options are limited and are centred around vasodilatory medications such as phosphodiesterase-5 inhibitors and prostacyclin. While these medications allow for the widening of blood vessels that are narrowed due to remodelling, they have no effect on the remodelling itself. Sotatercept is a novel medication which targets the BMPR2/TGF-β pathway and restore a pro- and anti- proliferative balance to ultimately counteract vascular remodelling. Recent phase 2 and 3 trials showed that treatment with sotatercept led to lower resistance and pres |
| Who can participate | Inclusion Criteria: 1. Adult patients between 18-70 years of age 2. Able to provide signed informed consent 3. WHO FC II to IV 4. NTproBNP \> 300 ng/L 5. PAH etiology belonging to one of the following groups (Nice classification): * Idiopathic PAH * Heritable PAH 6. Hemodynamic diagnosis of PAH confirmed by RHC during screening showing: * mPAP \> 20 mmHg * Pulmonary capillary wedge pressure (PCWP) or left ventricular end diastolic pressure (LVEDP) ≤ 15 mmHg * PVR ≥ 4WU (320 dyn.sec.cm-5) 7. For patients treated with oral diuretics, treatment dose must have been stable at least 1 month prior to RHC during the screening period 8. All patients are on stable background therapy at least 3 months prior to RHC during the screening period 9. Women of childbearing potential must have a negati |
| Ages | 18 Years to 70 Years |
| Sex | All |
| Lead sponsor | Amsterdam UMC, location VUmc |
| Locations | Amsterdam, North Holland, Netherlands |
| Start date | 2025-05-14 |
| NCT ID | NCT06658522 |
| Official listing | https://clinicaltrials.gov/study/NCT06658522 |