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, Victor R Gordeuk, Roberto F Machado and on behalf of the walk-PHaSST Investigators and Patients

PLOS ONE, 2014, vol. 9, issue 7, 1-8

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 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

Date: 2014
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Persistent link: https://EconPapers.repec.org/RePEc:plo:pone00:0099489

DOI: 10.1371/journal.pone.0099489

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