Left ventricular remodeling in patients with sickle cell disease: determinants factors and impact on outcome.

Cardiovascular complications have been increasingly detected as a result of prolonged longevity of patients with sickle cell disease (SCD). Previous studies have focused especially on pulmonary hypertension and its consequences on the right-side heart chambers, whereas factors associated with morphological changes in left ventricle (LV) remain poorly understood. This study was designed to identify clinical, laboratorial, and echocardiographic parameters associated with LV remodeling and its impact on outcome in SCD. Ninety patients aged 28 ± 7 years and 20 age- and gender-balanced healthy subjects were enrolled. Laboratory tests, electrocardiogram, and an echocardiogram with tissue Doppler imaging were performed in all patients. Patients with SCD had larger left and right heart chambers dimensions, LV mass, and tricuspid regurgitation (TR) velocity compared to health controls with similar demographic features. Despite chambers enlargement, systolic function of both ventricles was preserved. The mitral inflow velocities were higher in the patients than in controls, whereas septal and lateral annular motion velocities were normal, suggesting normal ventricular relaxation. SCD patients who were on hydroxyurea therapy and/or hypertransfusion had higher hemoglobin concentrations, but similar echocardiographic findings in comparison to those without treatment. Systolic blood pressure, ferritin concentration, TR velocity, and parameters of diastolic function were independently associated with increased LV mass. In addition, the predictors of adverse events were ferritin concentration, lactate dehydrogenase levels, and TR velocity. LV remodeling in SCD patients seems to be influenced by a combination of factors including blood pressure, ferritin concentration, TR velocity, and parameters of LV diastolic function, and was not associated with adverse outcomes.

Assessment of ventricular function in adults with sickle cell disease: role of two-dimensional speckle-tracking strain.

BACKGROUND: Sickle cell disease (SCD) is a hemoglobinopathy that is common worldwide. It usually presents with cardiac involvement, although data on systolic function are somewhat controversial. The aim of this study was to investigate the value of speckle-tracking strain, a deformation index, in detecting ventricular dysfunction in SCD. METHODS: Ninety adult patients with SCD were compared with 20 healthy controls. Doppler echocardiography with Doppler tissue imaging was performed in all, and the left and right ventricles were analyzed by the use of two-dimensional speckle-tracking strain. RESULTS: The mean age of the patients with SCD was 26 years, and 43% were men. Left ventricular (LV) dimensions and mass were higher in patients with SCD, whereas LV ejection fraction did not differ from the controls. E and A waves, as well as E/e' ratio, were also higher in patients with SCD. Two-dimensional speckle-tracking strain of both ventricles in the patients with SCD was not different from that of controls. The factors independently associated with LV longitudinal strain were age (P = .009), oximetry (P = .001), lactate dehydrogenase (P = .014), LV ejection fraction (P < .001), and right ventricular systolic annular velocity (P = .010). CONCLUSIONS: Ventricular enlargement with normal ventricular function was a frequent finding in SCD. Two-dimensional speckle-tracking strain of both ventricles was similar in patients and controls, suggesting normal myocardial contractility in patients with SCD. LV global longitudinal strain was associated with age, intensity of hemolysis, and ventricular function.