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.