Journal Article

Risk Factors for Death in 632 Patients with Sickle Cell Disease in the United States and United Kingdom

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

2014

Overview

The role of pulmonary hypertension as a cause of mortality in sickle cell disease (SCD) is controversial. 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. 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.

• Publisher: Public Library of Science,Public Library of Science (PLoS)
• Subject: Adult / Anemia, Sickle Cell - mortality / Anemia, Sickle Cell - pathology / Biology and Life Sciences / Blood Flow Velocity / Blood Pressure / Cohort Studies / Creatinine / Creatinine - blood / Critical care / Death / Family medical history / Female / Ferritin / Ferritins - analysis / Follow-Up Studies / Health aspects / Health risk assessment / Heart / Hemolysis / Humans / Hypertension / Hypertension, Pulmonary - pathology / Intubation / Kaplan-Meier Estimate / Male / Medicine / Medicine and Health Sciences / Mercury / Middle Aged / Mortality / Natriuretic Peptide, Brain - blood / Patients / Peptide Fragments - blood / Peptides / Proportional Hazards Models / Pulmonary arteries / Pulmonary artery / Pulmonary hypertension / Regurgitation / Risk analysis / Risk Factors / Severity of Illness Index / Sickle cell anemia / Sickle cell disease / Studies / Systolic pressure / United Kingdom / United States / Walking