The global burden of pulmonary hypertension in sickle cell disease: a systematic review and meta-analysis

Abstract

Elevated tricuspid regurgitant jet velocity (TRJV) is a surrogate measure of pulmonary hypertension (PH) in persons with sickle cell disease (SCD). We sought to estimate the burden of PH in people living with sickle cell disease based on TRJV. From 2000 to 2015, we searched electronic databases for eligible publications and included 29 studies (n = 5358 persons). We used random effects modeling to determine the pooled estimate of elevated TRJV. The overall pooled prevalence of elevated TRJV was 23.5 %(95 % CI 19.5–27.4) in persons with SCD. The pooled prevalence of elevated TRJV in children and adults with SCD was 20.7 % (95 % CI 15.7–-25.6) and 24.4 % (95 % CI 18.4–30.4), respectively. TRJV is prevalent among adults and children with SCD. Our finding support international recommendations that call for screening for PH in SCD patients.

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Acknowledgments

We wish to acknowledge the Vanderbilt Institute for Global Health, for Training on Research Ethics.

Authors’ contributions

Concept and design of study (BMM).

Acquisition of data, (BMM, NAG, CM) analysis and interpretation of data (BMM, SB, AMH).

Revision and intellectual contribution (BMM, NAG, CMSB, AMH).

All authors approved the final version.

Drafting the article or revising it critically for important intellectual (BMM, NAG, CM SB, AMH).

Final approval of the version to be published, (BMM, NAG, CM SB, AMH).

The manuscript has been read and approved by all the authors that the requirements for authorship as stated earlier in this document have been met, and each of us believes that the manuscript represents honest work.

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Correspondence to B. M. Musa.

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The authors declare that they have no conflict of interest.

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This study is not supported by any grant.

Funding sources

Scottie Bussell contributed to this work during his fellowship, which was supported by National Institutes of Health (NIH) Research Training Grant R25 TW009337, funded by Fogarty International Center, the NIH Office of the Director, and the National institute of Mental Health.

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Musa, B.M., Galadanci, N.A., Coker, M. et al. The global burden of pulmonary hypertension in sickle cell disease: a systematic review and meta-analysis. Ann Hematol 95, 1757–1764 (2016). https://doi.org/10.1007/s00277-016-2693-z

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Keywords

  • Africa
  • Pulmonary hypertension
  • Tricuspid velocity
  • Sickle cell disease
  • Meta-analysis