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

Gladwin, MT and Barst, RJ and Gibbs, JSR and Hildesheim, M and Sachdev, V and Nouraie, M and Hassell, KL and Little, JA and Schraufnagel, DE and Krishnamurti, L and Novelli, E and Girgis, RE and Morris, CR and Rosenzweig, EB and Badesch, DB and Lanzkron, S and Castro, OL and Taylor VI, JG and Goldsmith, JC and Kato, GJ and Gordeuk, VR and Machado, RF (2014) Risk factors for death in 632 patients with sickle cell disease in the United States and United Kingdom. PLoS ONE, 9 (7).

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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 with identifier: NCT00492531.


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Item Type: Article
Status: Published
CreatorsEmailPitt UsernameORCID
Gladwin, MTmtg16@pitt.eduMTG16
Barst, RJ
Gibbs, JSR
Hildesheim, M
Sachdev, V
Nouraie, M
Hassell, KL
Little, JA
Schraufnagel, DE
Krishnamurti, L
Novelli, Eemn3@pitt.eduEMN3
Girgis, RE
Morris, CR
Rosenzweig, EB
Badesch, DB
Lanzkron, S
Castro, OL
Taylor VI, JG
Goldsmith, JC
Kato, GJgjk22@pitt.eduGJK22
Gordeuk, VR
Machado, RF
ContributionContributors NameEmailPitt UsernameORCID
Centers: Other Centers, Institutes, Offices, or Units > Vascular Medicine Institute
Date: 2 July 2014
Date Type: Publication
Journal or Publication Title: PLoS ONE
Volume: 9
Number: 7
DOI or Unique Handle: 10.1371/journal.pone.0099489
Schools and Programs: School of Medicine > Medicine
Refereed: Yes
Date Deposited: 16 Sep 2014 19:43
Last Modified: 31 Jul 2020 16:00


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