title: Estimated pulmonary artery systolic pressure and sickle cell disease: a meta-analysis and systematic review.
abstract: Many studies report estimated pulmonary artery systolic pressure (ePASP) in patients with sickle cell disease (SCD) screened by echocardiography. To better understand the prevalence and outcomes of elevated ePASP in clinically stable SCD patients, we conducted a random-effects meta-analysis. A total of 45 studies, representing 15 countries and including 6109 individuals, met our inclusion criteria. In most (70%) studies, elevated ePASP was defined by a tricuspid regurgitant velocity of 2.5 m/s. The prevalence of elevated ePASP was 21% (17-26%) in children and 30% (26-35%) in adults. After adjustment for sex, SCD genotype, haemoglobin, hydroxycarbamide (hydroxyurea) treatment, country and publication year, age remained associated with elevated ePASP, yielding a 12% (0.4-23%) higher adjusted prevalence in adults. Few studies reported 6-min walk tests or mortality outcomes, and estimates were highly heterogeneous. In random effects meta-analyses, patients with elevated ePASP walked an estimated 30.4 (6.9-53.9) metres less than those without elevated ePASP and had an associated mortality hazard ratio of 4.9 (2.4-9.7).
authors: Department of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.
authors: Melissa C
authors: MC
authors: Caughey
authors: Department of Epidemiology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.
authors: Charles
authors: C
authors: Poole
authors: Department of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.
authors: Kenneth I
authors: KI
authors: Ataga
authors: Department of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.
authors: Alan L
authors: AL
authors: Hinderliter
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000401:mortality / Q000503:physiopathology*; D000986:Antisickling Agents / Q000627:therapeutic use; D062186:Arterial Pressure; D004452:Echocardiography; D005260:Female; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D008297:Male; D015995:Prevalence; D011651:Pulmonary Artery / Q000000981:diagnostic imaging / Q000503:physiopathology*
chemical_list: D000986:Antisickling Agents; D006918:Hydroxyurea
keywords: echocardiography; meta-analysis; pulmonary hypertension; sickle cell disease