Altered left ventricular twist is associated with clinical severity in adults and adolescents with homozygous sickle cell anemia.
BACKGROUND
Sickle cell anemia (SCA) is associated with cardiac abnormalities and premature death. The aims of this study were to identify early markers of cardiac dysfunction through ventricular strain and ventricular twist and determine the relationships between these measures and other markers of cardiovascular risk.


METHODS
Forty patients with SCA (mean age, 23.5 ± 9.3 years; 24 male patients) and 40 age- and sex-matched healthy individuals were compared. All subjects participated in structured interviews, and blood samples 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-dimensional speckle-tracking echocardiography were performed.


RESULTS
There were no differences in LV ejection fraction, global LV strain (longitudinal, circumferential, and radial), and global right ventricular longitudinal strain between patients and controls; however, LV twist was significantly lower in the patient group (mean, 7.4 ± 1.2° vs 10.7 ± 1.8°; P < .0001). Several variables were strongly related to LV twist, including the clinical severity index (ρ = -0.97, Z score = -6.05, P < .0001), E/e' 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 ventricular twist and clinical severity index, E/e' 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 greater cardiac risk.
Echocardiography Department, Dante Pazzanese Institute of Cardiology, São Paulo, Brazil. Electronic address: joaocbraga2002@yahoo.com.br.
Joao Carlos Moron Saes
JC
Braga
Cardiovascular Imaging Department, Dante Pazzanese Institute of Cardiology, São Paulo, Brazil.
Jorge Eduardo
JE
Assef
Internal Medicine Department, Marilia Medical School (Famema), Marilia, Brazil.
Paulo Henrique
PH
Waib
Invasive Cardiology Department, Dante Pazzanese Institute of Cardiology, São Paulo, Brazil.
Amanda Guerra de Moraes Rego
AG
de Sousa
Echocardiography Department, Dante Pazzanese Institute of Cardiology, São Paulo, Brazil.
Rodrigo Bellio
RB
de Mattos Barretto
Cardiology Department, Marilia Medical School (Famema), Marilia, Brazil.
Fábio Villaça
FV
Guimarães Filho
Cardiology Department, Marilia Medical School (Famema), Marilia, Brazil.
Alexandre
A
Rodrigues
Cardiology Department, Marilia Medical School (Famema), Marilia, Brazil.
Fernanda Di Tullio Trindade
FD
Vilela
Cardiology Department, Marilia Medical School (Famema), Marilia, Brazil.
Igor Ribeiro
IR
de Castro Bienert
Hematology Department, Marilia Medical School (Famema), Marilia, Brazil.
Doralice Marvulle
DM
Tan
Hematology Department, Marilia Medical School (Famema), Marilia, Brazil.
Danielen Cristina Mariano
DC
Peluccio
D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000175:diagnosis*; D004452:Echocardiography / Q000379:methods; D005260:Female; D006352:Heart Ventricles / Q000000981:diagnostic imaging; D006801:Humans; D008297:Male; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index; D014102:Torsion Abnormality / Q000000981:diagnostic imaging* / Q000209:etiology; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging* / Q000209:etiology*
Left ventricular twist; Sickle cell anemia; Speckle-tracking echocardiography