Cardiovascular complications have been increasingly detected in patients with sickle cell disease (SCD). Two-dimensional speckle-tracking echocardiography (STE) detects early myocardial changes in a number of pathophysiological processes, which may be useful in SCD. This study was designed to examine the value of STE in predicting clinical outcome in adult patients with SCD. A total of 219 patients, mean age 33 ± 12 years were prospectively enrolled. Several clinical, laboratory and echocardiographic variables including left ventricular global longitudinal strain (LVGLS) by STE were assessed. The endpoint was a composite of the following events: (1) all-cause mortality, (2) three or more acute painful episodes that require hospitalization in one year, (3) acute chest syndrome and (4) hospitalization due to disease complication. The majority of the patients had enlargement of LV and left atrial (LA) with preserved ejection fraction. During the mean follow-up of 30 months, 69 patients (32%) had reached the endpoint, including eight deaths (3.7%). No difference was observed in the parameters of diastolic function comparing the patients with and without events. LVGLS ranged from - 12.25 to - 25.44 (mean - 20.26 ± 2.5), with higher values in the patients who had events compared with those who did not. In the multivariable analysis, higher LVGLS values were associated with adverse events (adjusted OR 1.25; 95% CI 1.04-1.51; p = 0.021), independently of the TR maximal velocity and LV ejection fraction. In patients with SCD, higher LV global longitudinal strain was a predictor of adverse outcome, independently of age, TR velocity and LV function.
Keywords: Clinical outcome; Left ventricular longitudinal strain; Sickle cell disease; Speckle-tracking echocardiography.