Author(s): Gladwin MT, Barst RJ, Gibbs JS, Hildesheim M, Sachdev V,
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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.
This article was published in PLoS One
and referenced in Translational Medicine