Riociguat Versus Balloon Pulmonary Angioplasty in Non-operable Chronic thromboEmbolic Pulmonary Hypertension
Condition: Chronic Thromboembolic Pulmonary Hypertension
MeSH terms: Hypertension, Pulmonary; Hypertension
Study type: Interventional

Summary: 
      "Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe form of pulmonary
      hypertension characterized by obstruction of the pulmonary vasculature by residual organized
      thrombi, leading to increased pulmonary vascular resistance (PVR), progressive pulmonary
      hypertension, and right failure.

      In patients deemed operable, pulmonary endarterectomy (PEA) is the gold standard treatment
      and is the only potentially curative treatment. However, some patients are ineligible for
      surgery owing to occlusion of distal vessels. The best treatment option for these
      non-operable CTEPH patients is not yet established.
    

Detailed description: 
      Currently, riociguat is the only drug approved in Europe and US for the treatment of
      non-operable CTEPH. However, medical therapy with riociguat does not address obstructive
      lesions. In this sense, another treatment option, balloon pulmonary angioplasty (BPA), began
      recently to gain widespread interest after development in several centers. This procedure
      uses the standard balloon angioplasty technique to dilate selected pulmonary arteries. The
      main aim is to reopen vessels occluded by webs and bands. Several teams, mainly from Japan,
      have reported their experiences with BPA for the treatment of non-operable CTEPH and
      demonstrated impressive decrease in pulmonary vascular resistance and improvement in
      functional status and exercise capacity with an acceptable procedure-related risk. Although
      BPA has never been prospectively evaluated, most of the leading CTEPH centers worldwide have
      currently added BPA to their therapeutic options. However, no randomized controlled trial
      comparing safety and efficacy of medical therapy with riociguat versus pulmonary balloon
      angioplasty has been performed so far. Therefore, the respective places of medical treatment
      and of BPA in the management of inoperable patients with CTEPH need to be further evaluated.

      An ancillary study will evaluate the efficacy and safety of employment of two sequentially
      used treatments at 12 months. The studied population is that of the study RACE. The patient
      follow-up period will be extended by 6 months, its duration of participation will be in total
      of 13 months. The exams will be the same as those of the V3 of the RACE study.
    

Intervention name(s): Balloon Pulmonary Angioplasty (BPA); Riociguat
Intervention type(s): Procedure; Drug
Intervention description(s): Balloon pulmonary angioplasty (BPA) will be done via femoral vein. 2 sessions will be performed during the same hospitalization following by additional sessions at an interval of 2-3 weeks until a mean PAP< 30 mmHg is achieved A pulmonary angiography and a right heart catheterization will be performed at each BPA session; The starting dose will be 1 mg three times daily as recommended. The dose will be titrated every 2 weeks according to the peripheral systolic pressure. Dose should be increased by 0.5 mg three times daily every two weeks to a maximum of 2.5 mg three times daily, if systolic blood pressure is ≥95 mmHg and the patient has no signs or symptoms of hypotension.
Other intervention name(s): Medical therapy with Riociguat
Intervention MeSH terms: Riociguat
Allocation: Randomized
Intervention model: Parallel Assignment
Masking: None (Open Label)
Primary purpose: Treatment
Keywords: Chronic Thromboembolic Pulmonary Hypertension; Balloon Pulmonary Angioplasty; Riociguat