Riociguat 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. Thromboembolic disease is prevalent in sickle cell disease and can lead to pu...

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Bibliographic Details
Main Authors: Nargues A. Weir, Anna Conrey, Denise Lewis, Alem Mehari
Format: Article
Language:English
Published: Wiley 2018-07-01
Series:Pulmonary Circulation
Online Access:https://doi.org/10.1177/2045894018791802
Description
Summary: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.
ISSN:2045-8940