title: Longitudinal study of echocardiography-derived tricuspid regurgitant jet velocity in sickle cell disease.
abstract: 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 TRVs, 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.
authors.affiliation: Division of Hematology/Oncology, UNC, Chapel Hill, NC 27599-7305, USA.
authors.forename: Payal C
authors.initials: PC
authors.lastname: Desai
authors.forename: Ryan C
authors.initials: RC
authors.lastname: May
authors.forename: Susan K
authors.initials: SK
authors.lastname: Jones
authors.forename: Dell
authors.initials: D
authors.lastname: Strayhorn
authors.forename: Melissa
authors.initials: M
authors.lastname: Caughey
authors.forename: Alan
authors.initials: A
authors.lastname: Hinderliter
authors.forename: Kenneth I
authors.initials: KI
authors.lastname: Ataga
mesh_terms: D000328:Adult; D000368:Aged; D000755:Anemia, Sickle Cell / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000503:physiopathology*; D000986:Antisickling Agents / Q000627:therapeutic use; D004452:Echocardiography / Q000379:methods*; D005260:Female; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D006976:Hypertension, Pulmonary / Q000097:blood / Q000000981:diagnostic imaging / Q000503:physiopathology; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012307:Risk Factors; D014262:Tricuspid Valve Insufficiency / Q000097:blood / Q000000981:diagnostic imaging* / Q000503:physiopathology; D055815:Young Adult
chemical_list: D000986:Antisickling Agents; D006918:Hydroxyurea
keywords: Sickle cell disease; hydroxycarbamide; progression; pulmonary hypertension; tricuspid regurgitant jet velocity