Risk factors for death in 632 patients with sickle cell disease in the United States and United Kingdom

Mark T. Gladwin, Robyn J. Barst, J. Simon R. Gibbs, Mariana Hildesheim, Vandana Sachdev, Mehdi Nouraie, Kathryn L. Hassell, Jane A. Little, Dean E. Schraufnagel, Lakshmanan Krishnamurti, Enrico Novelli, Reda E. Girgis, Claudia R. Morris, Erika Berman Rosenzweig, David B. Badesch, Sophie Lanzkron, Oswaldo L. Castro, James G. Taylor VI, Jonathan C. Goldsmith, Gregory J. Kato & 2 others Victor R. Gordeuk, Roberto Machado

Research output: Contribution to journalArticle

38 Citations (Scopus)

Abstract

Background: The role of pulmonary hypertension as a cause of mortality in sickle cell disease (SCD) is controversial. Methods and Results: We evaluated the relationship between an elevated estimated pulmonary artery systolic pressure and mortality in patients with SCD. We followed patients from the walk-PHaSST screening cohort for a median of 29 months. A tricuspid regurgitation velocity (TRV)≥3.0 m/s cuttof, which has a 67-75% positive predictive value for mean pulmonary artery pressure ≥25 mm Hg was used. Among 572 subjects, 11.2% had TRV≥3.0 m/sec. Among 582 with a measured NT-proBNP, 24.1% had values ≥160 pg/mL. Of 22 deaths during follow-up, 50% had a TRV≥3.0 m/sec. At 24 months the cumulative survival was 83% with TRV≥3.0 m/sec and 98% with TRV<3.0 m/sec (p<0.0001). The hazard ratios for death were 11.1 (95%CI 4.1-30.1; p<0.0001) for TRV≥3.0 m/sec, 4.6 (1.8-11.3; p = 0.001) for NT-proBNP≥160 pg/mL, and 14.9 (5.5-39.9; p<0.0001) for both TRV≥3.0 m/sec and NT-proBNP≥160 pg/mL. Age >47 years, male gender, chronic transfusions, WHO class III-IV, increased hemolytic markers, ferritin and creatinine were also associated with increased risk of death. Conclusions: A TRV≥ 3.0 m/sec occurs in approximately 10% of individuals and has the highest risk for death of any measured variable. The study is registered in ClinicalTrials.gov with identifier: NCT00492531.

Original languageEnglish (US)
Article numbere99489
JournalPLoS One
Volume9
Issue number7
DOIs
StatePublished - Jul 2 2014
Externally publishedYes

Fingerprint

sickle cell anemia
Sickle Cell Anemia
United Kingdom
Tricuspid Valve Insufficiency
risk factors
pulmonary artery
death
Pulmonary Artery
Ferritins
Creatinine
Screening
Mortality
ferritin
Pulmonary Hypertension
creatinine
hypertension
lungs
Blood Pressure
screening
Pressure

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)
  • Agricultural and Biological Sciences(all)

Cite this

Gladwin, M. T., Barst, R. J., Gibbs, J. S. R., Hildesheim, M., Sachdev, V., Nouraie, M., ... Machado, R. (2014). Risk factors for death in 632 patients with sickle cell disease in the United States and United Kingdom. PLoS One, 9(7), [e99489]. https://doi.org/10.1371/journal.pone.0099489

Risk factors for death in 632 patients with sickle cell disease in the United States and United Kingdom. / Gladwin, Mark T.; Barst, Robyn J.; Gibbs, J. Simon R.; Hildesheim, Mariana; Sachdev, Vandana; Nouraie, Mehdi; Hassell, Kathryn L.; Little, Jane A.; Schraufnagel, Dean E.; Krishnamurti, Lakshmanan; Novelli, Enrico; Girgis, Reda E.; Morris, Claudia R.; Rosenzweig, Erika Berman; Badesch, David B.; Lanzkron, Sophie; Castro, Oswaldo L.; Taylor VI, James G.; Goldsmith, Jonathan C.; Kato, Gregory J.; Gordeuk, Victor R.; Machado, Roberto.

In: PLoS One, Vol. 9, No. 7, e99489, 02.07.2014.

Research output: Contribution to journalArticle

Gladwin, MT, Barst, RJ, Gibbs, JSR, Hildesheim, M, Sachdev, V, Nouraie, M, Hassell, KL, Little, JA, Schraufnagel, DE, Krishnamurti, L, Novelli, E, Girgis, RE, Morris, CR, Rosenzweig, EB, Badesch, DB, Lanzkron, S, Castro, OL, Taylor VI, JG, Goldsmith, JC, Kato, GJ, Gordeuk, VR & Machado, R 2014, 'Risk factors for death in 632 patients with sickle cell disease in the United States and United Kingdom', PLoS One, vol. 9, no. 7, e99489. https://doi.org/10.1371/journal.pone.0099489
Gladwin MT, Barst RJ, Gibbs JSR, Hildesheim M, Sachdev V, Nouraie M et al. Risk factors for death in 632 patients with sickle cell disease in the United States and United Kingdom. PLoS One. 2014 Jul 2;9(7). e99489. https://doi.org/10.1371/journal.pone.0099489
Gladwin, Mark T. ; Barst, Robyn J. ; Gibbs, J. Simon R. ; Hildesheim, Mariana ; Sachdev, Vandana ; Nouraie, Mehdi ; Hassell, Kathryn L. ; Little, Jane A. ; Schraufnagel, Dean E. ; Krishnamurti, Lakshmanan ; Novelli, Enrico ; Girgis, Reda E. ; Morris, Claudia R. ; Rosenzweig, Erika Berman ; Badesch, David B. ; Lanzkron, Sophie ; Castro, Oswaldo L. ; Taylor VI, James G. ; Goldsmith, Jonathan C. ; Kato, Gregory J. ; Gordeuk, Victor R. ; Machado, Roberto. / Risk factors for death in 632 patients with sickle cell disease in the United States and United Kingdom. In: PLoS One. 2014 ; Vol. 9, No. 7.
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abstract = "Background: The role of pulmonary hypertension as a cause of mortality in sickle cell disease (SCD) is controversial. Methods and Results: We evaluated the relationship between an elevated estimated pulmonary artery systolic pressure and mortality in patients with SCD. We followed patients from the walk-PHaSST screening cohort for a median of 29 months. A tricuspid regurgitation velocity (TRV)≥3.0 m/s cuttof, which has a 67-75{\%} positive predictive value for mean pulmonary artery pressure ≥25 mm Hg was used. Among 572 subjects, 11.2{\%} had TRV≥3.0 m/sec. Among 582 with a measured NT-proBNP, 24.1{\%} had values ≥160 pg/mL. Of 22 deaths during follow-up, 50{\%} had a TRV≥3.0 m/sec. At 24 months the cumulative survival was 83{\%} with TRV≥3.0 m/sec and 98{\%} with TRV<3.0 m/sec (p<0.0001). The hazard ratios for death were 11.1 (95{\%}CI 4.1-30.1; p<0.0001) for TRV≥3.0 m/sec, 4.6 (1.8-11.3; p = 0.001) for NT-proBNP≥160 pg/mL, and 14.9 (5.5-39.9; p<0.0001) for both TRV≥3.0 m/sec and NT-proBNP≥160 pg/mL. Age >47 years, male gender, chronic transfusions, WHO class III-IV, increased hemolytic markers, ferritin and creatinine were also associated with increased risk of death. Conclusions: A TRV≥ 3.0 m/sec occurs in approximately 10{\%} of individuals and has the highest risk for death of any measured variable. The study is registered in ClinicalTrials.gov with identifier: NCT00492531.",
author = "Gladwin, {Mark T.} and Barst, {Robyn J.} and Gibbs, {J. Simon R.} and Mariana Hildesheim and Vandana Sachdev and Mehdi Nouraie and Hassell, {Kathryn L.} and Little, {Jane A.} and Schraufnagel, {Dean E.} and Lakshmanan Krishnamurti and Enrico Novelli and Girgis, {Reda E.} and Morris, {Claudia R.} and Rosenzweig, {Erika Berman} and Badesch, {David B.} and Sophie Lanzkron and Castro, {Oswaldo L.} and {Taylor VI}, {James G.} and Goldsmith, {Jonathan C.} and Kato, {Gregory J.} and Gordeuk, {Victor R.} and Roberto Machado",
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T1 - Risk factors for death in 632 patients with sickle cell disease in the United States and United Kingdom

AU - Gladwin, Mark T.

AU - Barst, Robyn J.

AU - Gibbs, J. Simon R.

AU - Hildesheim, Mariana

AU - Sachdev, Vandana

AU - Nouraie, Mehdi

AU - Hassell, Kathryn L.

AU - Little, Jane A.

AU - Schraufnagel, Dean E.

AU - Krishnamurti, Lakshmanan

AU - Novelli, Enrico

AU - Girgis, Reda E.

AU - Morris, Claudia R.

AU - Rosenzweig, Erika Berman

AU - Badesch, David B.

AU - Lanzkron, Sophie

AU - Castro, Oswaldo L.

AU - Taylor VI, James G.

AU - Goldsmith, Jonathan C.

AU - Kato, Gregory J.

AU - Gordeuk, Victor R.

AU - Machado, Roberto

PY - 2014/7/2

Y1 - 2014/7/2

N2 - Background: The role of pulmonary hypertension as a cause of mortality in sickle cell disease (SCD) is controversial. Methods and Results: We evaluated the relationship between an elevated estimated pulmonary artery systolic pressure and mortality in patients with SCD. We followed patients from the walk-PHaSST screening cohort for a median of 29 months. A tricuspid regurgitation velocity (TRV)≥3.0 m/s cuttof, which has a 67-75% positive predictive value for mean pulmonary artery pressure ≥25 mm Hg was used. Among 572 subjects, 11.2% had TRV≥3.0 m/sec. Among 582 with a measured NT-proBNP, 24.1% had values ≥160 pg/mL. Of 22 deaths during follow-up, 50% had a TRV≥3.0 m/sec. At 24 months the cumulative survival was 83% with TRV≥3.0 m/sec and 98% with TRV<3.0 m/sec (p<0.0001). The hazard ratios for death were 11.1 (95%CI 4.1-30.1; p<0.0001) for TRV≥3.0 m/sec, 4.6 (1.8-11.3; p = 0.001) for NT-proBNP≥160 pg/mL, and 14.9 (5.5-39.9; p<0.0001) for both TRV≥3.0 m/sec and NT-proBNP≥160 pg/mL. Age >47 years, male gender, chronic transfusions, WHO class III-IV, increased hemolytic markers, ferritin and creatinine were also associated with increased risk of death. Conclusions: A TRV≥ 3.0 m/sec occurs in approximately 10% of individuals and has the highest risk for death of any measured variable. The study is registered in ClinicalTrials.gov with identifier: NCT00492531.

AB - Background: The role of pulmonary hypertension as a cause of mortality in sickle cell disease (SCD) is controversial. Methods and Results: We evaluated the relationship between an elevated estimated pulmonary artery systolic pressure and mortality in patients with SCD. We followed patients from the walk-PHaSST screening cohort for a median of 29 months. A tricuspid regurgitation velocity (TRV)≥3.0 m/s cuttof, which has a 67-75% positive predictive value for mean pulmonary artery pressure ≥25 mm Hg was used. Among 572 subjects, 11.2% had TRV≥3.0 m/sec. Among 582 with a measured NT-proBNP, 24.1% had values ≥160 pg/mL. Of 22 deaths during follow-up, 50% had a TRV≥3.0 m/sec. At 24 months the cumulative survival was 83% with TRV≥3.0 m/sec and 98% with TRV<3.0 m/sec (p<0.0001). The hazard ratios for death were 11.1 (95%CI 4.1-30.1; p<0.0001) for TRV≥3.0 m/sec, 4.6 (1.8-11.3; p = 0.001) for NT-proBNP≥160 pg/mL, and 14.9 (5.5-39.9; p<0.0001) for both TRV≥3.0 m/sec and NT-proBNP≥160 pg/mL. Age >47 years, male gender, chronic transfusions, WHO class III-IV, increased hemolytic markers, ferritin and creatinine were also associated with increased risk of death. Conclusions: A TRV≥ 3.0 m/sec occurs in approximately 10% of individuals and has the highest risk for death of any measured variable. The study is registered in ClinicalTrials.gov with identifier: NCT00492531.

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