pulmonary arterial hypertension
Conditions
Interventions
Reduction and discontinuation of continuous intravenous or subcutaneous administration of prostacyclin analogs during treatment with sotatercept
Sponsors
Kyorin University School of Medicine
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1 Patients with a confirmed diagnosis of pulmonary arterial hypertension. 2 Patients are currently receiving two or more therapeutic agents, including prostacyclin analogue injectables, continuous intravenous, or continuous subcutaneous infusion. 3 Patients who have been on sotatercept for at least 3 months. 4 Patients who have maintained a low risk score for at least 3 months based on the REVEAL Lite 2 criteria. 5 Individuals who meet any of the following criteria, and for whom the continuation of injectable therapy clearly causes significant disadvantages in daily life. Quality of life is clearly impaired due to pain, gastrointestinal symptoms, or other side effects of the injectable medication. Individuals with a history of central venous catheter-related infection who are deemed to be at high risk of reinfection. Carrying a pump or managing treatment at home significantly limits the patient's functional capacity. Individuals who wish to discontinue prostacyclin analogue injectable therapy. 6 Individuals aged 18 years or older. 7 Individuals who understand the content of this study and can provide written consent.
Exclusion criteria
Exclusion criteria: 1. Contraindications for sotatercept (history of hypersensitivity to the drug, platelet count <50,000/mm3) 2. Patients with mPAP >40 mmHg at enrollment 3. Patients who required hospitalization or intensified treatment for PAH within 3 months prior to enrollment 4. Patients with serious comorbidities (e.g., severe liver or kidney dysfunction, uncontrolled heart disease, or infections) 5. Women who are pregnant, breastfeeding, or planning to become pregnant during study participation 6. Cases where the principal investigator or a co-investigator determines that participation in this study is inappropriate 7. Patients with mPAP <25 mmHg and PAP/CO slope <3 (based on iCPET)
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| The rate of achieving non-worsening at 90 days after discontinuation of prostacyclin-based injectables. 1. No clinical events (death, PAH-related hospitalization, syncope, reintroduction of injectables, etc.) 2. Maintenance of REVEAL Lite 2: 5 or fewer than 5 3. No worsening on RHC: PVR: Does not meet the criteria of an increase of 15% or more than 15% from baseline and 1 or more than 1 WU CI: Does not decrease to less than 2.0 L/min/m2 | — |
Countries
Japan
Contacts
Public ContactTakumi Inami
Kyorin University School of Medicine Department of Cardiovascular Medicine
Outcome results
None listed