Journal
BRITISH JOURNAL OF HAEMATOLOGY
Volume 162, Issue 3, Pages 400-408Publisher
WILEY
DOI: 10.1111/bjh.12391
Keywords
sickle cell disease; pulmonary hypertension; mortality; survival; tricuspid regurgitation velocity
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Funding
- Fundacao para a Ciencia e a Tecnologia (FCT), Portugal [SFRH/BD/47751/2008]
- National Institute for Health Research (NIHR) under the Biomedical Research Centre Scheme
- Fundação para a Ciência e a Tecnologia [SFRH/BD/47751/2008] Funding Source: FCT
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Raised tricuspid regurgitant velocity (TRV) occurs in approximately 30% of adults with sickle cell disease (SCD), and has been shown to be an independent risk factor for death. TRV was assessed in 164 SCD patients who were subsequently followed up for survival. Raised pulmonary pressures were defined as a TRV jet >= 2 center dot 5m/s on echocardiography. Elevated TRV was present in 29 center dot 1% of patients and it was associated with increased age and left atrial diameter.There were 15 deaths (9 center dot 1%) over a median of 68 center dot 1 months follow up; seven patients had increased TRV, and eight patients had a TRV<2 center dot 5 m/s. Higher TRV values were associated with a greater than 4-fold increased risk of death (Hazard Ratio: 4 center dot 48, 99% confidence interval 1 center dot 01-1 center dot 98), although we found a lower overall mortality rate than has been reported in previous studies. TRV was not an independent risk factor for death. We have confirmed the association between raised TRV and mortality in a UK SCD population whose disease severity appears to be less than that reported in previous studies. Further prospective studies are needed to more clearly characterize which patient factors modify survival in SCD patients with raised TRV.
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