• Pulmonary circulation · Oct 2018

    Case Reports

    Riociguat use in sickle cell related chronic thromboembolic pulmonary hypertension: A case series.

    • Nargues A Weir, Anna Conrey, Denise Lewis, and Alem Mehari.
    • 1 Cardiovascular and Pulmonary Branch and Hematology Branch, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, USA.
    • Pulm Circ. 2018 Oct 1; 8 (4): 2045894018791802.

    AbstractAdults 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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