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Published on: May 11, 2015

High prevalence of pulmonary hypertension in children with sickle cell disease

Stephen C Nelson1, Beverly B Adade, Elizabeth A McDonough

  • 1Children's Hospitals and Clinics of Minnesota, Minneapolis and St Paul, MN 55404, USA. stephen.nelson@childrensmn.org

Journal of Pediatric Hematology/Oncology
|May 8, 2007
PubMed

Insights

Pulmonary hypertension (PHT) affects 31% of children aged 10 and older with sickle cell disease. Early screening for PHT in these children is recommended to improve outcomes.

Area of Science:

  • Pediatric Hematology
  • Cardiology
  • Pulmonology

Background:

  • Sickle cell disease (SCD) is a genetic blood disorder with significant cardiovascular complications.
  • Pulmonary hypertension (PHT) is a known complication in adults with SCD, associated with increased mortality.
  • The prevalence and associated factors of PHT in children with SCD are not well-established.

Purpose of the Study:

  • To determine the prevalence of pulmonary hypertension (PHT) in children with sickle cell disease (SCD).
  • To identify factors associated with the presence of PHT in this pediatric cohort.
  • To establish screening recommendations for PHT in children with SCD.

Main Methods:

  • Prospective study design.
  • Doppler echocardiography used to assess for evidence of PHT.
  • Analysis of demographic and clinical factors, including sex and reticulocyte count.

Main Results:

  • Pulmonary hypertension (PHT) was detected in 31% of children aged 10 years and older with sickle cell disease (SCD).
  • Male sex was identified as a significant factor associated with PHT.
  • Elevated reticulocyte count was also associated with the presence of PHT.

Conclusions:

  • Pulmonary hypertension (PHT) is prevalent in children with sickle cell disease (SCD) aged 10 and older.
  • Screening for PHT should be initiated in children with SCD starting at age 10.
  • Early detection and management of PHT in pediatric SCD may improve long-term outcomes.

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