Altered Left Ventricular Twist Is Associatedwith Clinical Severity in Adults and Adolescentswith Homozygous Sickle Cell Anemia

ABSTRACT

Sickle cell anemia (SCA) is associated with cardiac abnormalities and premature death. The aimsof this study were to identify early markers of cardiac dysfunction through ventricular strain and ventriculartwist and determine the relationships between these measures and other markers of cardiovascular risk.Methods: Forty patients with SCA (mean age, 23.5 6 9.3 years; 24 male patients) and 40 age- and sexmatchedhealthy individuals were compared. All subjects participated in structured interviews, and bloodsamples were collected. Standard echocardiography with subsequent offline evaluations using left ventricular(LV) and right ventricular systolic strain and rotational analyses of the left ventricle using two-dimensionalspeckle-tracking echocardiography were performed.Results: There were no differences in LV ejection fraction, global LV strain (longitudinal, circumferential, andradial), and global right ventricular longitudinal strain between patients and controls; however, LV twist wassignificantly lower in the patient group (mean, 7.4 6 1.2 vs 10.7 6 1.8 ; P < .0001). Several variables werestrongly related to LV twist, including the clinical severity index (r = 0.97, Z score = 6.05, P < .0001), E/e0 ratio(r = 0.78, P < .0001), LV end-diastolic volume index (r = 0.81, P < .0001), and pulmonary artery systolic pressure(r = 0.72, P < .0001).Conclusions: LV twist is altered in patients with SCA. There were strong correlations between left ventriculartwist and clinical severity index, E/e0 ratio, LV end-diastolic volume index, and pulmonary artery systolic pressure.These data suggest that decreased LV twist may indicate a subgroup of patients with SCA at greatercardiac risk.