Cardiopulmonary disease is the leading cause of mortality in adults with sickle cell disease (SCD). Elevated tricuspid regurgitant jet velocity (TRJV) and reduced forced expiratory volume in 1 second (FEV1) %predicted are associated with early mortality in SCD; however their relationship and combined effect on survival is unknown. We investigated the relationship between TRJV and FEV1 %predicted, and their combined effect on mortality, in a retrospective cohort of 189 adults with SCD who underwent both pulmonary function testing and echocardiography. Nineteen (9.9%) of 189 patients died over a median follow-up of 1.4 years; cardiopulmonary disease was the major cause of death in 52.6%. FEV1%predicted was negatively associated with TRJV (Spearman rho, −0.34, P < 0.001). Individuals with FEV1%predicted ≤70% were more likely to have an elevated TRJV ≥2.5 m/second, compared to those with FEV1%predicted >70% [45.8% versus 17.1%; odds ratio (OR) 4.1 (95% Confidence interval ([CI] 2.1–8.0); P = 0.001]. In a multivariable cox regression model, the combination of TRJV ≥2.5 m/second and FEV1%predicted ≤70% predicted earlier mortality [hazard ratio (HR) 4.97 (95% CI 1.30-18.91; P = 0.019)] after adjusting for age, sex, and nephropathy. Both FEV1 %predicted ≤70% and TRJV ≥2.5 m/second were independently associated with nephropathy [OR 4.48 (95% CI 1.51–13.31); P = 0.004] and [OR 3.27 (95% CI 1.19–9.00); P = 0.017], respectively. In conclusion, pulmonary and cardiac impairment are associated with, and contribute to mortality in SCD. Therapies aimed at improving reduced FEV1%predicted and elevated TRJV could improve survival in patients with SCD. Am. J. Hematol. 92:125–130, 2017.
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