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

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.

journal_name

Pulm Circ

journal_title

Pulmonary circulation

authors

Weir NA,Conrey A,Lewis D,Mehari A

doi

10.1177/2045894018791802

subject

Has Abstract

pub_date

2018-10-01 00:00:00

pages

2045894018791802

issue

4

eissn

2045-8932

issn

2045-8940

journal_volume

8

pub_type