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Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet
Published on: November 4, 2015
Riociguat use in sickle cell related chronic thromboembolic pulmonary hypertension: A case series
Nargues A Weir1,2, Anna Conrey1, Denise Lewis2
11 Cardiovascular and Pulmonary Branch and Hematology Branch, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, USA.
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.
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