Abstract
Recent studies have recognised the importance of pulmonary hypertension (PH) in sickle cell disease (SCD). The aim of this study was to determine the prevalence and prognostic impact of PH and its features in patients with SCD.
80 patients with SCD underwent baseline clinical evaluation, laboratory testing, 6-min walk tests (6MWTs) and echocardiography. Patients with a peak tricuspid regurgitant jet velocity (TRV) of ≥2.5 m·s−1were further evaluated through right heart catheterisation (RHC) to assure the diagnosis of PH.
Our study evidenced a 40% prevalence of patients with elevated TRV at echocardiography. RHC (performed in 25 out of 32 patients) confirmed PH in 10% (95% CI 3.4–16.5%) of all patients, with a prevalence of post-capillary PH of 6.25% (95% CI 0.95–11.55%) and pre-capillary PH of 3.75% (95% CI -0.4–7.9%). Patients with PH were older, had worse performance in 6MWTs, and more pronounced anaemia, haemolysis and renal dysfunction. Survival was shorter in patients with PH.
我们的研究强化了使用of echocardiography as a screening tool for PH in SCD and the mandatory role of RHC for proper diagnosis. Our findings confirmed the prognostic significance of PH in SCD as its association to pronounced haemolytic profile.
- Haemodynamics
- prevalence
- pulmonary hypertension
- right heart catheterisation
- sickle cell disease
- survival
Footnotes
For editorial comments seepage 3.
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Statement of Interest
None declared.
- ReceivedAugust 20, 2010.
- AcceptedMay 10, 2011.
- ©ERS 2012