Elevated tricuspid regurgitation velocity and decline in exercise capacity over 22 months of follow up in children and adolescents with sickle cell anemia

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

Research output: Contribution to journalArticle

47 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)33-40
Number of pages8
JournalHaematologica
Volume96
Issue number1
DOIs
StatePublished - Jan 2011
Externally publishedYes

Fingerprint

Tricuspid Valve Insufficiency
Sickle Cell Anemia
Exercise
Hemolysis
Pulmonary Artery
Sickle Hemoglobin
Pressure
Ventricular Pressure
Echocardiography
Biomarkers
Mortality

Keywords

  • E/Etdi
  • Hemolysis
  • Sickle cell anemia
  • Six minute walk
  • Tricuspid regurgitation velocity

ASJC Scopus subject areas

  • Hematology

Cite this

Elevated tricuspid regurgitation velocity and decline in exercise capacity over 22 months of follow up in children and adolescents with sickle cell anemia. / Gordeuk, Victor R.; Minniti, Caterina P.; Nouraie, Mehdi; Campbell, Andrew D.; Rana, Sohail R.; Luchtman-Jones, Lori; Sable, Craig; Dham, Niti; Ensing, Gregory; Prchal, Josef T.; Kato, Gregory J.; Gladwin, Mark T.; Castro, Oswaldo L.

In: Haematologica, Vol. 96, No. 1, 01.2011, p. 33-40.

Research output: Contribution to journalArticle

Gordeuk, VR, Minniti, CP, Nouraie, M, Campbell, AD, Rana, SR, Luchtman-Jones, L, Sable, C, Dham, N, Ensing, G, Prchal, JT, Kato, GJ, Gladwin, MT & Castro, OL 2011, 'Elevated tricuspid regurgitation velocity and decline in exercise capacity over 22 months of follow up in children and adolescents with sickle cell anemia', Haematologica, vol. 96, no. 1, pp. 33-40. https://doi.org/10.3324/haematol.2010.030767
Gordeuk, Victor R. ; Minniti, Caterina P. ; Nouraie, Mehdi ; Campbell, Andrew D. ; Rana, Sohail R. ; Luchtman-Jones, Lori ; Sable, Craig ; Dham, Niti ; Ensing, Gregory ; Prchal, Josef T. ; Kato, Gregory J. ; Gladwin, Mark T. ; Castro, Oswaldo L. / Elevated tricuspid regurgitation velocity and decline in exercise capacity over 22 months of follow up in children and adolescents with sickle cell anemia. In: Haematologica. 2011 ; Vol. 96, No. 1. pp. 33-40.
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abstract = "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.",
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T1 - Elevated tricuspid regurgitation velocity and decline in exercise capacity over 22 months of follow up in children and adolescents with sickle cell anemia

AU - Gordeuk, Victor R.

AU - Minniti, Caterina P.

AU - Nouraie, Mehdi

AU - Campbell, Andrew D.

AU - Rana, Sohail R.

AU - Luchtman-Jones, Lori

AU - Sable, Craig

AU - Dham, Niti

AU - Ensing, Gregory

AU - Prchal, Josef T.

AU - Kato, Gregory J.

AU - Gladwin, Mark T.

AU - Castro, Oswaldo L.

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N2 - 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.

AB - 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.

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KW - Hemolysis

KW - Sickle cell anemia

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KW - Tricuspid regurgitation velocity

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