Elevated tricuspid regurgitant jet velocity, reduced forced expiratory volume in 1 second, and mortality in adults with sickle cell disease

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.