Abstract | BACKGROUND: DESIGN AND METHODS: We conducted a prospective, longitudinal, multi-center study of 160 individuals aged 3-20 years with hemoglobin SS, performing baseline and follow-up determinations of clinical markers, six-minute walk distance less than tricuspid regurgitation velocity and E/Etdi ratio by echocardiography. RESULTS: At baseline, 14.1% had tricuspid regurgitation velocity of 2.60 m/sec or over, which suggests elevated systolic pulmonary artery pressure, and 7.7% had increased E/Etdi, which suggests elevated left ventricular filling pressure. Over a median of 22 months, baseline elevation in tricuspid regurgitation velocity was associated with an estimated 4.4-fold increase in the odds of a 10% or more decline in age-standardized six-minute-walk distance (P = 0.015). During this interval, baseline values above the median for a hemolytic component derived from four markers of hemolysis were associated with a 9.0-fold increase in the odds of the new onset of elevated tricuspid regurgitation velocity (P = 0.008) and baseline E/Etdi elevation was associated with an estimated 6.1-fold increase in the odds (P = 0.039). In pathway analysis, higher baseline hemolytic component and E/Etdi predicted elevated tricuspid regurgitation velocity at both baseline and follow up, and these elevations in turn predicted decline in six-minute-walk distance. CONCLUSIONS: Further studies should define the long-term risks of elevated tricuspid regurgitation velocity in childhood and identify potential interventions to prevent increased pulmonary artery pressure and preserve function.
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Authors | Victor R Gordeuk, Caterina P Minniti, Mehdi Nouraie, Andrew D Campbell, Sohail R Rana, Lori Luchtman-Jones, Craig Sable, Niti Dham, Gregory Ensing, Josef T Prchal, Gregory J Kato, Mark T Gladwin, Oswaldo L Castro |
Journal | Haematologica
(Haematologica)
Vol. 96
Issue 1
Pg. 33-40
(Jan 2011)
ISSN: 1592-8721 [Electronic] Italy |
PMID | 20884713
(Publication Type: Journal Article, Multicenter Study, Research Support, N.I.H., Extramural, Research Support, N.I.H., Intramural, Research Support, Non-U.S. Gov't)
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Topics |
- Adolescent
- Adult
- Anemia, Sickle Cell
(complications, physiopathology)
- Child
- Child, Preschool
- Echocardiography
- Exercise
- Female
- Follow-Up Studies
- Hemolysis
- Humans
- Male
- Mitral Valve Insufficiency
- Prospective Studies
- Tricuspid Valve Insufficiency
(etiology, physiopathology)
- Young Adult
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