Treatment of Exercise-Induced Pulmonary Vascular Dysfunction After Pulmonary Thromboendarterectomy or Balloon Pulmonary Angioplasty

University of Colorado, Denver10 enrolled

Overview

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

Study Type

INTERVENTIONAL

Allocation

Purpose

TREATMENT

Masking

NONE

Enrollment

Conditions

Chronic Thromboembolic Disease (CTED)Exercise Intolerance Post PEA Surgery

Interventions

AdempasDRUG

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.

Eligibility

Sex: ALLMin age: 18 YearsMax age: 100 Years

Medical Language ↔ Plain English

Inclusion Criteria: * Adult patients who underwent PTE or BPA 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

Locations (1)

University of Colorado, Anschutz Medical Campus

Aurora, Colorado, United States

Outcomes

Primary Outcomes

Change in mean pulmonary arterial pressure

Measurements include mPAP (mean pulmonary arterial pressure) measured by right heart catheterization (RHC)