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- Title
Risk Factors for Death in 632 Patients with Sickle Cell Disease in the United States and United Kingdom.
- Authors
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.; Berman Rosenzweig, Erika; Badesch, David B.; Lanzkron, Sophie; Castro, Oswaldo L.; Taylor VI, James G.; Goldsmith, Jonathan C.; Kato, Gregory J.
- 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
- Subjects
UNITED Kingdom; UNITED States; PULMONARY hypertension; SICKLE cell anemia; COHORT analysis; MORTALITY; FOLLOW-up studies (Medicine); PATIENTS; DISEASE risk factors
- Publication
PLoS ONE, 2014, Vol 9, Issue 7, p1
- ISSN
1932-6203
- Publication type
Article
- DOI
10.1371/journal.pone.0099489