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- W3023897776 abstract "Until recently, the prevalence and characteristics of pulmonary hypertension (PH) in adults with sickle cell disease (SCD) remained uncertain. During the past decade several studies have reported the prevalence of PH as defined by a tricuspid valve regurgitant jet velocity (TRJV) ≧2.5 m/s as high as 30%. However, in these studies the diagnosis of PH was not systematically confirmed on right heart catheterization (RHC), a procedure that is recommended in international guidelines. Most recent studies have reported that the prevalence of PH confirmed on RHC in the SCD population is lower than expected (6–10%). Moreover, postcapillary PH was the most frequent cause of PH. Actually, these two studies demonstrated that the positive predictive value of echocardiography for the detection of PH in this population was low (25–32%) when using a TRJV ≧2.5 m/s to define PH. Patients with SCD and precapillary PH have a distinct hemodynamic profile, with less marked increase in mean pulmonary artery pressure, higher cardiac output, and lower pulmonary vascular resistance than patients with idiopathic pulmonary arterial hypertension (PAH). However, PH confirmed by RHC confers an increased risk of death even when mild or moderate. None of the specific therapies approved for the treatment of PAH are currently approved for the treatment of PAH associated with SCD due to the lack of data in this specific population." @default.
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- W3023897776 date "2012-01-01" @default.
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- W3023897776 title "Pulmonary Hypertension in Sickle Cell Disease" @default.
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- W3023897776 doi "https://doi.org/10.1159/000335667" @default.
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