Effects of Combination Medical Therapy Followed by BPA on Right Ventricular-PA Coupling and Hemodynamics in CTEPH (EPIPHANY)

Dr Sudarshan Rajagopal

Status and phase: Enrolling; Phase 3

Conditions: CTEPH

Treatments: Drug: Riociguat; Drug: Macitentan Tablets; Device: balloon pulmonary angioplasty

Study type: Interventional

Funder types: Other; Industry

Identifiers

NCT05140525

Pro00105903

Details and patient eligibility

About

The main goal of this study is to determine the effects of combination medical therapy (Riociguat and Macitentan) and balloon pulmonary angioplasty (BPA) on hemodynamics and right ventricular (RV) function (including advanced assessments of RV-pulmonary artery (PA) coupling from invasive hemodynamics) in participants with inoperable or post-PTE residual CTEPH.

Full description

Recent presented but unpublished results from trials of BPA vs riociguat for inoperable CTEPH (NCT02634203) have demonstrated that BPA provides a more significant hemodynamic benefit than medical therapy. The investigators hypothesize that participants who are treated with upfront combination medical therapy followed by BPA will have significant improvements in their hemodynamics and RV-PA coupling that can be monitored over time.

Enrollment: 15 estimated patients

Sex: All

Ages: 18+ years old

Volunteers: No Healthy Volunteers

Inclusion criteria

Subjects must meet all of the following inclusion criteria to be eligible for enrollment into the trial:

1. Age ≥ 18 years' old
2. Diagnosis of CTEPH
3. Not a candidate for PTE
4. Candidate for BPA based on suitable anatomy and disease burden
5. Treatment-naïve (no CTEPH or pulmonary arterial hypertension (PAH)-specific medical therapies) with plans for initiation of CTEPH/PAH-specific medical therapy and treatment with BPA.
6. Willing and able to give informed consent and adhere to visit/protocol schedules (Consent must be given before any study procedures are performed).

Exclusion criteria

• Subjects presenting with any of the following will not be included in the trials:

  1. Moderate to severe heart disease (LVEF < 45% or severe LV Hypertrophy)
  2. Sarcoidosis
  3. Active cancer
  4. Sickle cell anemia
  5. Liver disease (Childs-Pugh class C)
  6. Prisoners
  7. Pregnant, planning pregnancy or lactating
  8. Conditions that will prohibit MRI scanning (metal in eye, claustrophobia, inability to lie supine)
  9. Contraindication to riociguat or macitentan
  10. Medical or psychological conditions which, in the opinion of the investigator, might create undue risk to the subject or interfere with the subject's ability to comply with the protocol requirements

Trial design

Primary purpose: Treatment

Allocation: Non-Randomized

Interventional model: Parallel Assignment

Masking: None (Open label)

---

15 participants in 2 patient groups

participants with inoperable CTEPH

Active Comparator group

Description:

subject with inoperable Chronic thromboembolic Pulmonary Hypertension

Treatment:

Device: balloon pulmonary angioplasty

Drug: Riociguat

Drug: Macitentan Tablets

post PTE residual CTEPH

Active Comparator group

Description:

Subject with post pulmonary endarterectomy (PTE) residual Chronic Thromboembolic Pulmonary Hypertension

Treatment:

Device: balloon pulmonary angioplasty

Drug: Riociguat

Drug: Macitentan Tablets