Pulmonary circulation
-
Pulmonary circulation · Oct 2018
Case ReportsRiociguat use in sickle cell related chronic thromboembolic pulmonary hypertension: A case series.
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. ⋯ 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.