Abstract
Recent studies have recognized 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-minute walk test (6MWT) and echocardiography. Patients with peak tricuspid regurgitant jet velocity(TRV) ≥2.5 m·s−1 were further evaluated through right heart catheterization (RHC) to assure the diagnosis of PH.
Our study evidenced a 40% prevalence of patients with elevated TRV at echocardiogram. RHC (performed in 25/32 patients) confirmed PH in 10% (95% CI 3.4 to 16.5) of all patients, with a prevalence of post-capillary PH of 6.25% (95%CI 0.95 to 11.55) and pre-capillary PH of 3.75% (95%CI −0.4 to 7.9%). Patients with PH were older, had worse performance at 6MWT and more pronounced anaemia, haemolysis and renal dysfunction. Survival was shorter in patients with PH.
Our study reinforced the use of echocardiogram as screening tool for PH in SCD but also the mandatory role of RHC for the proper diagnosis. Our findings confirmed the prognostic significance of PH in SCD as its association to pronounced hemolytic profile.
- Hemodynamics
- prevalence; pulmonary hypertension
- right heart catheterization
- sickle cell disease
- survival
- ERS
