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

Ann Hematol. 2016 Oct;95(11):1757-64. doi: 10.1007/s00277-016-2693-z. Epub 2016 May 16.

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.

Keywords: Africa; Meta-analysis; Pulmonary hypertension; Sickle cell disease; Tricuspid velocity.

Publication types

  • Meta-Analysis
  • Review
  • Systematic Review

MeSH terms

  • Adult
  • Age Distribution
  • Anemia, Sickle Cell / complications*
  • Anemia, Sickle Cell / physiopathology
  • Child
  • Echocardiography, Doppler
  • Epidemiologic Studies
  • Humans
  • Hypertension, Pulmonary / epidemiology
  • Hypertension, Pulmonary / etiology*
  • Hypertension, Pulmonary / physiopathology
  • Models, Cardiovascular
  • Prevalence
  • Tricuspid Valve Insufficiency / diagnostic imaging
  • Tricuspid Valve Insufficiency / etiology*
  • Tricuspid Valve Insufficiency / physiopathology