• Join Today!

Become a member and connect with:

  • An Active Online Community
  • Articles and Advice on SCD
  • Help Understanding Clinical Trials
scientific articles

Prostacyclin-analog therapy in sickle cell pulmonary hypertension

key information

source: Haematologica

year: 2017

authors: Weir NA, Saiyed R, Alam S, Conrey A, Desai HD, George MP, Keeley JH, Klings ES, Mehari A, Taylor JG 6th, Minniti CP, Kato GJ


Sickle cell disease (SCD) is associated with pulmonary hypertension (PH) which results in high morbidity and mortality. There are well-established therapies for pulmonary arterial hypertension (PAH), but few reports about their use in SCDPH.2 We report the clinical course of 11 SCDPH patients on maximal supportive therapy including other pulmonary vasodilators, who received compassionate therapy with prostacyclin-analogs at four PH treatment centers. Inclusion criteria for this retrospective study included PH diagnosis by standard right heart catheterization (RHC), and prostacyclin-analog therapy via any route of delivery for at least four weeks. Data were collected subject to availability in the medical record, including type of prostacyclin-analog therapy, maximal dose and duration, and follow-up data at least 100 days after initiating therapy. Statistical analyses were performed using GraphPad Prism version 5.0 (La Jolla, CA, USA), using paired t-test and Pearson product-moment correlation. P<0.05 was considered significant.

organization: National Institutes of Health, Bethesda; Eastern Virginia Medical School; University of Pittsburgh School of Medicine; Howard University, Washington DC; Albert Einstein College of Medicine, Bronx, New York

DOI: 10.3324/haematol.2015.131227

read more full text source

To improve your experience on this site, we use cookies. This includes cookies essential for the basic functioning of our website, cookies for analytics purposes, and cookies enabling us to personalize site content. By clicking on 'Accept' or any content on this site, you agree that cookies can be placed. You may adjust your browser's cookie settings to suit your preferences. More information

The cookie settings on this website are set to "allow cookies" to give you the best browsing experience possible. If you continue to use this website without changing your cookie settings or you click "Accept" below then you are consenting to this.