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Archives of Medical Science
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vol. 14
Clinical research

Left ventricular structural and functional changes evaluated by echocardiography and two-dimensional strain in patients with sickle cell disease

Ricardo Bedirian, Andrea Ribeiro Soares, Maria Christina Maioli, Jussara Fonseca Fernandes de Medeiros, Agnaldo José Lopes, Marcia Bueno Castier

Arch Med Sci 2018; 14, 3: 493–499
Online publish date: 2016/03/23
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Patients with sickle cell disease have increased left ventricular size, which is not usually accompanied by changes in systolic function indexes. We assessed echocardiographic abnormalities present in patients with sickle cell anemia (SCA) and compared echocardiographic parameters to other sickle cell diseases (OSCD).

Material and methods
A blind cross-sectional study with 60 patients with SCA and 16 patients with OSCD who underwent transthoracic echocardiography was performed.

Echocardiographic findings were: left atrial volume index 47.7 ±11.5 ml/m² in SCA group and 31.7 ±8.42 ml/m² in OSCD group (p < 0.001); left ventricular diastolic diameter index 3.47 ±0.37 cm/m² in SCA group and 2.97 ±0.41 cm/m² in OSCD group (p < 0.001); left ventricular systolic diameter index 2.12 ±0.31 cm/m² in SCA group and 1.86 ±0.28 cm/m² in OSCD group (p < 0.001). There were no differences in the left ventricular ejection fraction: 68.2 ±6.69% in SCA group and 67.1 ±6.21% in OSCD group (p = 0.527). The ratio between mitral E wave and mean mitral annulus e’ wave velocities was higher in the SCA group (7.72 ±1.54 vs. 6.70 ±1.65; p = 0.047). Mitral A wave correlated significantly with hemoglobin levels (r = –0.340; p = 0.032).

There was an increase of left ventricular and left atrial sizes in patients with SCA, compared to patients with OSCD, without changes in systolic or diastolic function in both groups. This could be due to the hyperkinetic state due to the more severe anemia in the SCA subjects.


sickle cell disease, hemoglobinopathies, left ventricle, systolic function, diastolic function

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