Estimated pulmonary artery systolic pressure and sickle cell disease: a meta‐analysis and systematic review
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Summary: 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).
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A meta-analysis of 45 screening studies from 15 countries of more than 6,000 patients indicates that the prevalence of elevated tricuspid regurgitant jet velocity ≥2.5 m/s is 30% (range 26-35%) in adults.
“A meta-analysis of 45 screening studies from 15 countries of more than 6,000 patients indicates that the prevalence of elevated tricuspid regurgitant jet velocity ≥2.5 m/s is 30% (range 26–35%) in adults.”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [191]
The Results report 45 studies from 15 countries involving 6,109 people and an estimated 30% prevalence of elevated estimated pulmonary artery systolic pressure among adults with sickle cell disease. However, the studies used varying definitions of elevation: the reported 30% (26–35%) is not specifically the prevalence of TRV ≥2.5 m/s, although the Results say restricting analyses to that threshold did not substantially change the estimates. The 26–35% figures are a 95% confidence interval, not a range of observed prevalence. (LLM, conf=0.88)
Experimental — accuracy not yet measuredPatients with elevated tricuspid regurgitant jet velocity walked an estimated 30.4 (6.9-53.9) meters less than those without elevated tricuspid regurgitant jet velocity, with an associated hazard ratio for death of 4.9 (2.4-9.7).
“Patients with elevated tricuspid regurgitant jet velocity walked an estimated 30.4 (6.9–53.9) meters less than those without elevated tricuspid regurgitant jet velocity, with an associated hazard ratio for death of 4.9 (2.4–9.7) (191).”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [191]
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- Authors
- Caughey, M. C., Poole, C., Ataga, K. I., & Hinderliter, A. L.
- Affiliations
- University of North Carolina at Chapel Hill (US)
- Corresponding author
- Caughey, Melissa C.
- Venue
- British Journal of Haematology
- Publisher
- Wiley
- Published
- 2015-04-09
- Volume
- 170
- Issue
- 3
- Pages
- 416-424
- ISSN
- 0007-1048 (ISSN-L)
- 1365-2141
- OpenAlex
- W2138288711
- Cited by
- 41
- References
- 61
- Citations by year
- 2026: 1 · 2025: 2 · 2024: 2 · 2023: 1 · 2022: 3 · 2021: 6 · 2020: 3 · 2019: 5 · 2018: 2 · 2017: 6 · 2016: 8 · 2015: 1
- FWCI
- 0.80
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- British Journal of Haematology (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Genetics › Hemoglobinopathies and Related Disorders
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
- Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
- Keywords
- sickle cell disease, pulmonary artery systolic pressure, tricuspid regurgitation velocity, echocardiography, prevalence, 6-minute walk test, mortality, hydroxyurea, children and adults
- MeSH terms
- Adult, Anemia, Sickle Cell, Antisickling Agents, Echocardiography, Female, Humans, Hydroxyurea, Male, Pulmonary Artery, Prevalence, Arterial Pressure
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