Open AccessRiociguat use in sickle cell related chronic thromboembolic pulmonary hypertension: A case series
Author(s) -
Weir Nargues A.,
Conrey Anna,
Lewis Denise,
Mehari Alem
Publication year - 2018
Publication title -
pulmonary circulation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.791
H-Index - 40
ISSN - 2045-8940
DOI - 10.1177/2045894018791802
Subject(s) - riociguat , medicine , chronic thromboembolic pulmonary hypertension , pulmonary hypertension , tolerability , disease , acute chest syndrome , etiology , sickle cell anemia , cardiology , intensive care medicine , adverse effect
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.