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.

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