Longitudinal study of echocardiography‐derived tricuspid regurgitant jet velocity in sickle cell disease

Summary Although echocardiography‐derived tricuspid regurgitant jet velocity ( TRV ) is associated with increased mortality in sickle cell disease ( SCD ), its rate of increase and predictive markers of its progression are unknown. We evaluated 55 subjects (median age: 38 years, range: 20–65 years) with at least two measurable TRV s, followed for a median of 4·5 years (range: 1·0–10·5 years) in a single‐centre, prospective study. Thirty‐one subjects (56%) showed an increase in TRV , while 24 subjects (44%) showed no change or a decrease in TRV . A linear mixed effects model indicated an overall rate of increase in the TRV of 0·02 m/s per year ( P  = 0·023). The model showed that treatment with hydroxycarbamide was associated with an initial TRV that was 0·20 m/s lower than no such treatment ( P  = 0·033), while treatment with angiotensin converting enzyme inhibitors and angiotensin receptor blockers was associated with an increase in the TRV ( P  = 0·006). In summary, although some patients have clinically meaningful increases, the overall rate of TRV increase is slow. Treatment with hydroxycarbamide may decrease the progression of TRV . Additional studies are required to determine the optimal frequency of screening echocardiography and the effect of therapeutic interventions on the progression of TRV in SCD .