Document Detail


Elevated tricuspid regurgitation velocity and decline in exercise capacity over 22 months of follow up in children and adolescents with sickle cell anemia.
MedLine Citation:
PMID:  20884713     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: While in adults with sickle cell disease an elevation of tricuspid regurgitation velocity is associated with increased mortality, the importance of this finding in children has not been established. The role of intravascular hemolysis in the development of this complication is controversial.
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.
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
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Publication Detail:
Type:  Journal Article; Multicenter Study; Research Support, N.I.H., Extramural; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't     Date:  2010-09-30
Journal Detail:
Title:  Haematologica     Volume:  96     ISSN:  1592-8721     ISO Abbreviation:  Haematologica     Publication Date:  2011 Jan 
Date Detail:
Created Date:  2011-01-03     Completed Date:  2011-05-26     Revised Date:  2011-10-19    
Medline Journal Info:
Nlm Unique ID:  0417435     Medline TA:  Haematologica     Country:  Italy    
Other Details:
Languages:  eng     Pagination:  33-40     Citation Subset:  IM    
Affiliation:
Center for Sickle Cell Disease, Howard University, Washington, DC 20060, USA. vgordeuk@howard.edu
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MeSH Terms
Descriptor/Qualifier:
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
Grant Support
ID/Acronym/Agency:
1 R01 HL079912-02/HL/NHLBI NIH HHS; 2 R25 HL003679-08/HL/NHLBI NIH HHS; 2MOI RR10284-10/RR/NCRR NIH HHS; Z01 CL008098-01/CL/CLC NIH HHS; Z01 CL008098-02/CL/CLC NIH HHS; ZIA HL006016-01/HL/NHLBI NIH HHS; ZIA HL006016-02/HL/NHLBI NIH HHS
Comments/Corrections
Comment In:
Haematologica. 2011 Jan;96(1):1-5   [PMID:  21193426 ]

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