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PLoS One. 2014 Jul 2;9(7):e99489. doi: 10.1371/journal.pone.0099489. eCollection 2014.

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

Author information

1
Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, Pennsylvania, United States of America; Division of Pulmonary Allergy and Critical Care Medicine, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, United States of America.
2
Columbia University, New York, New York, United States of America.
3
National Heart & Lung Institute, Imperial College London, London, United Kingdom.
4
Cardiovascular Branch, NHLBI, Bethesda, Maryland, United States of America.
5
Howard University, Washington, DC, United States of America.
6
University of Colorado HSC, Denver, Colorado, United States of America.
7
Case Western Reserve University, Cleveland, Ohio, United States of America.
8
University of Illinois, Chicago, Illinois, United States of America.
9
Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, Pennsylvania, United States of America; Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania, United States of America.
10
Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, Pennsylvania, United States of America.
11
Johns Hopkins University, Baltimore, Maryland, United States of America.
12
Emory University School of Medicine, Atlanta, Georgia, United States of America.
13
National Heart Lung and Blood Institute/NIH, Bethesda, Maryland, United States of America.

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.

PMID:
24988120
PMCID:
PMC4079316
DOI:
10.1371/journal.pone.0099489
[Indexed for MEDLINE]
Free PMC Article
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