CTEPH is a potentially treatable disease; the treatment of choice for operable patients is PEA. With removal of the obstruction, PEA improves systolic pulmonary artery pressure and hemodynamic status by decreasing the pulmonary vascular resistance. Results from an international prospective registry reported a 90% 3-year survival rate. However, up to 40% of patients are considered inoperable.
BPA is considered for treatment in patients with inoperable CTEPH owing to distal obstruction, severe hemodynamic impairment, or presence of multiple comorbid conditions.
Sildenafil, a phosphodiesterase-5 inhibitor, has shown to reduce pulmonary vascular resistance but is currently only used for off-label treatment. The only approved medication for the treatment of patients with inoperable and persistent or recurrent CTEPH is the soluble guanylate cyclase stimulator, riociguat. The CHEST-1 and CHEST-2 studies demonstrated that riociguat improved performance on the 6-minute walking distance test and the World Health Organization functional class and decreased pulmonary vascular resistance compared with placebo. In addition, riociguat sustained exercise and functional capacity benefits for up to 1 year.
Learn more about CTEPH.
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Cite this: Vikramjit Khangoora. Fast Five Quiz: Chronic Thromboembolic Pulmonary Hypertension - Medscape - May 17, 2022.