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Tricuspid regurgitant jet velocity elevation and its relationship to lung function in pediatric sickle cell disease

Robert I Liem1, Mary A Nevin, Adrienne Prestridge

  • 1Division of Hematology, Oncology & Stem Cell Transplant, Children's Memorial Hospital, Northwestern University Feinberg School of Medicine, Chicago, Illinois 60614, USA. rliem@childrensmemorial.org

Pediatric Pulmonology
|February 11, 2009
PubMed

Insights

Tricuspid regurgitant jet velocity (TRJV) elevation, a marker for pulmonary hypertension, is common in children with sickle cell disease (SCD). This elevation is not strongly linked to lung function but correlates with lower oxygen saturation and lung capacity.

Area of Science:

  • Pediatric Cardiology
  • Hematology
  • Pulmonology

Background:

  • Pulmonary hypertension (PHT) is a serious concern in adults with sickle cell disease (SCD).
  • Screening for elevated tricuspid regurgitant jet velocity (TRJV), an indicator of PHT, is increasingly considered for pediatric SCD patients.

Purpose of the Study:

  • To determine the prevalence of elevated TRJV in children and young adults with SCD.
  • To investigate the relationship between TRJV elevation and pulmonary function in this population at baseline.

Main Methods:

  • Prospective screening of 78 SCD subjects (10-24 years old) using Doppler echocardiogram (ECHO).
  • Comprehensive lung function evaluation and laboratory testing were performed during steady state.
  • Tricuspid regurgitation was quantified, and peak TRJV was assessed.

Main Results:

  • Elevated peak TRJV (>=2.5 m/sec) was observed in 33.3% of subjects.
  • No significant difference in obstruction, restriction, or gas exchange abnormalities was found between subjects with and without TRJV elevation.
  • TRJV showed significant inverse correlations with % predicted forced vital capacity (FVC) and oxygen saturation.
  • Subjects with TRJV elevation had lower FEV(1), FVC, and oxygen saturation compared to those without.
  • Low hemoglobin and low % predicted FVC were the best predictors of TRJV elevation (70% accuracy).

Conclusions:

  • Elevated TRJV is common in young individuals with SCD at baseline.
  • TRJV elevation in this cohort is not strongly associated with abnormal lung function.
  • Further research is needed to understand the implications of TRJV elevation in pediatric SCD.

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