Estimated pulmonary artery systolic pressure and sickle cell disease

a meta-analysis and systematic review

Melissa C. Caughey, Charles Poole, Kenneth Ataga, Alan L. Hinderliter

Research output: Contribution to journalReview article

16 Citations (Scopus)

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).

Original languageEnglish (US)
Pages (from-to)416-424
Number of pages9
JournalBritish Journal of Haematology
Volume170
Issue number3
DOIs
StatePublished - Jan 1 2015
Externally publishedYes

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Sickle Cell Anemia
Pulmonary Artery
Meta-Analysis
Blood Pressure
Hydroxyurea
Mortality
Echocardiography
Publications
Hemoglobins
Genotype

All Science Journal Classification (ASJC) codes

  • Hematology

Cite this

Estimated pulmonary artery systolic pressure and sickle cell disease : a meta-analysis and systematic review. / Caughey, Melissa C.; Poole, Charles; Ataga, Kenneth; Hinderliter, Alan L.

In: British Journal of Haematology, Vol. 170, No. 3, 01.01.2015, p. 416-424.

Research output: Contribution to journalReview article

Caughey, Melissa C. ; Poole, Charles ; Ataga, Kenneth ; Hinderliter, Alan L. / Estimated pulmonary artery systolic pressure and sickle cell disease : a meta-analysis and systematic review. In: British Journal of Haematology. 2015 ; Vol. 170, No. 3. pp. 416-424.
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