Riociguat use in sickle cell related chronic thromboembolic pulmonary hypertension: A case series. (July 2018)

• Record Type: Journal Article
• Authors: Weir, Nargues A.; Conrey, Anna; Lewis, Denise; Mehari, Alem
• Is Part Of: Pulmonary circulation. Volume 8:Number 4(2018)
• Publication Date: 2018-07
• Subjects: sickle cell disease -- chronic thromboembolic pulmonary hypertension -- riociguat; Pulmonary circulation -- Periodicals; Pulmonary circulation; Electronic journals -- Sciences; Periodicals; 616.24005
• DOI: 10.1177/2045894018791802
• Languages: English
• ISSNs: 2045-8932

Abstract

Adults with sickle cell disease can develop pulmonary hypertension from a multitude of etiologies. Classified as WHO Group 5, there are no therapies approved for the treatment of sickle cell disease-pulmonary hypertension. Thromboembolic disease is prevalent in sickle cell disease and can lead to pulmonary hypertension. The only approved medical therapy for chronic thromboembolic pulmonary hypertension is riociguat. We report the experience, safety and tolerability of riociguat use in a series of sickle cell disease patients with chronic thromboembolic pulmonary hypertension.