Document Detail

Prevalence and risk factors of elevated pulmonary artery pressures in children with sickle cell disease.
MedLine Citation:
PMID:  18381543     Owner:  NLM     Status:  MEDLINE    
OBJECTIVES: The objectives of this study were (1) to determine the prevalence and risk factors of elevated pulmonary artery pressures in children with homozygous SS or Sbeta(0) thalassemia using Doppler echocardiography and (2) to determine a correlation between abnormal transcranial Doppler examinations and elevated pulmonary artery pressures. METHODS: Screening echocardiograms were prospectively performed during an annual comprehensive clinic visit on children who were older than 6 years and had homozygous SS or Sbeta(0) thalassemia. Detailed history, examination, and laboratory tests were done, and transcranial Doppler examinations were obtained in children 2 to 14 years of age. Pulmonary hypertension was defined as pulmonary artery systolic pressure of at least 30 mmHg corresponding to a peak tricuspid regurgitant jet velocity of > or = 2.5 m/second. Mild pulmonary hypertension was defined as tricuspid regurgitant jet velocity > or = 2.5 to 2.9 m/second. Moderate pulmonary hypertension was defined as tricuspid regurgitant jet velocity > or = 3 m/second. Patients with pulmonary stenosis or right outflow obstruction were excluded. Characteristics were compared between patients with mild, moderate, and no pulmonary hypertension using 1-way analysis of variance for continuous variable and Fisher's exact test for categorical variables. RESULTS: Of the 75 patients who had homozygous SS/Sbeta(0) thalassemia and were older than 6 years, echocardiograms were obtained for 62 (82.6%). Thirty percent (19 of 62) of patients had elevated tricuspid regurgitant jet velocity > or = 2.5 m/second. One third of these patients had tricuspid regurgitant jet velocity > or = 3 m/second. All patients with elevated tricuspid regurgitant jet velocity had SS disease. A high reticulocyte count, low oxygen saturation, and a high platelet count were significantly associated with elevated pulmonary artery pressures. There was no difference in age, gender, history of acute chest syndrome, hydroxyurea therapy, chronic blood transfusion, stroke, hemoglobin, and bilirubin between patients with and without elevated pulmonary artery pressures. A total of 47% patients with elevated tricuspid regurgitant jet velocity and 57% without elevated tricuspid regurgitant jet velocity had screening transcranial Doppler examinations. Transcranial Doppler examinations were normal for all patients. CONCLUSIONS: High pulmonary artery pressures do occur in children with sickle cell disease. Screening by echocardiography can lead to early detection and intervention that may potentially reverse this disease process. There was no correlation between elevated pulmonary artery pressures and abnormal transcranial Doppler examination in our study.
Farzana D Pashankar; Judith Carbonella; Alia Bazzy-Asaad; Alan Friedman
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Pediatrics     Volume:  121     ISSN:  1098-4275     ISO Abbreviation:  Pediatrics     Publication Date:  2008 Apr 
Date Detail:
Created Date:  2008-04-02     Completed Date:  2008-04-30     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0376422     Medline TA:  Pediatrics     Country:  United States    
Other Details:
Languages:  eng     Pagination:  777-82     Citation Subset:  AIM; IM    
Department of Pediatrics, Yale University School of Medicine, LMP 2073, 333 Cedar St, PO Box 208064, New Haven, CT 06520-8064, USA.
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MeSH Terms
Age Distribution
Anemia, Sickle Cell / diagnosis,  epidemiology*
Child, Preschool
Cohort Studies
Follow-Up Studies
Hypertension, Pulmonary / epidemiology*,  ultrasonography
Mass Screening / methods*
Prospective Studies
Pulmonary Wedge Pressure
Risk Assessment
Severity of Illness Index
Sex Distribution
Survival Analysis
Tricuspid Valve Insufficiency / epidemiology*,  ultrasonography
Ultrasonography, Doppler, Transcranial

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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