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Published on: November 5, 2019

Clinical differences between children and adults with pulmonary hypertension and sickle cell disease

R Ward Hagar1, Jennifer G Michlitsch, Jennifer Gardner

  • 1Hematology/Oncology, Children's Hospital & Research Center Oakland, Oakland, CA 94609, USA. whagar@mail.cho.org

British Journal of Haematology
|October 6, 2007
PubMed

Insights

Pulmonary hypertension (PHT) in sickle cell disease (SCD) differs between adults and children. PHT significantly increases mortality risk in both groups, highlighting the need for better recognition and management in pediatric SCD patients.

Area of Science:

  • Cardiology
  • Hematology
  • Pediatrics

Background:

  • Pulmonary hypertension (PHT) is a known complication in sickle cell disease (SCD).
  • The prevalence and clinical implications of PHT in pediatric SCD patients remain largely unknown.
  • Existing research primarily focuses on adult SCD populations.

Purpose of the Study:

  • To investigate the prevalence and clinical associations of PHT in children and adults with SCD.
  • To identify factors associated with echocardiographic screening for PHT.
  • To determine the mortality impact of PHT in pediatric and adult SCD patients.

Main Methods:

  • Retrospective chart review of 362 SCD patients.
  • Analysis of clinical variables, echocardiographic screening data, and mortality outcomes.
  • Comparison of PHT phenotypes and associations in adult versus pediatric cohorts.

Main Results:

  • Patients with lung issues or chronic transfusions were more likely to receive echocardiograms, but PHT was often missed.
  • Distinct clinical phenotypes of PHT were observed in adults and children.
  • Adult PHT associated with age, renal/lung disease, hepatitis C, transfusions; ACS was protective.
  • Pediatric PHT associated with sepsis, ACS, and obstructive lung disease.
  • PHT diagnosis significantly increased mortality risk (Hazard Ratio: 17.3).

Conclusions:

  • PHT presents differently in pediatric versus adult SCD patients, suggesting age-specific mechanisms.
  • PHT is a critical factor associated with increased mortality in SCD.
  • Improved recognition and management strategies for PHT are crucial in pediatric SCD.

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