Treatment of Exercise-Induced Pulmonary Vascular Dysfunction in Symptomatic Patients After Pulmonary Thromboendarterectomy

Study Overview
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Brief Summary
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This is an open label study of Riociguat in patients with continued exercise intolerance at least 6 months following pulmonary endarterectomy (PEA).

Official Title
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Treatment of Exercise-Induced Pulmonary Vascular Dysfunction in Symptomatic Patients After Pulmonary Thromboendarterectomy

Conditions
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Chronic Thromboembolic Disease (CTED), Exercise Intolerance Post PEA Surgery

Intervention / Treatment
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* Drug: Adempas


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Adult patients who underwent PTE for Chronic thromboembolic pulmonary hypertension (CTEPH) at least six months prior to screening and report ongoing subjective exercise limitation, able to give consent able to perform a exercise protocol Exclusion Criteria: Patients with known Residual Pulmonary Hypertension (RPH) by RHC following PTE for CTEPH ongoing PAH-specific vasodilator therapy known contraindication to riociguat a physical limitation to completing an exercise protocol

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 100 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Study Drug<br>Riociguat (Adempas) 0.5mg to 2.5 mg three time daily - oral medication | Drug: Adempas<br>* After obtaining baseline data, patients will be placed on oral riociguat at a dose of 0.5 mg TID which will be up titrated over two months to a maximum dose of 2.5 mg TID or maximum tolerated dose. After 3 months from onset of riociguat initiation, the University of California San Diego (UCSD) shortness of breath score, 6 Minute Walk Test (6MWD), and resting/exercise Right Heart Catheterization (RHC) will be repeated.<br>* Other names: Riociguat;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Change in mean pulmonary arterial pressure | Measurements include mPAP (mean pulmonary arterial pressure) measured by right heart catheterization (RHC) | Baseline and 3 months | 
| Change in cardiac output | Measurements include CO (cardiac output) mPAP/CO slope measured by right heart catheterization (RHC) | Baseline and 3 months | 
| Change in Pulmonary artery compliance | Measurements include Pulmonary artery compliance measured by right heart catheterization (RHC) | Baseline and 3 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Change in 6 minute walk test | Measurements include change in 6 minute walk test (6 MWT) following 3 months of study drug | Baseline and 3 months | 
| Change in New York Heart Association (NYHA) functional class | Measurements include Change in New York Heart Association (NYHA) functional class following 3 months of study drug | Baseline and 3 months | 
| Change in the University of California San Diego (UCSD) shortness of Breath Score | Measurements include: Change in the University of California San Diego (UCSD) shortness of Breath Score following 3 months of study drug | Baseline and 3 months | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Chronic Thromboembolic Disease, CTED, Treatment Post PEA surgery