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

Abstract

IntroductionPatients 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 methodsA blind cross-sectional study with 60 patients with SCA and 16 patients with OSCD who underwent transthoracic echocardiography was performed.ResultsEchocardiographic 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).ConclusionsThere 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 and ventricular myocardial strain: A systematic review

“…was retrospective, and one (Mah et al.) was experimental 15,31–43,45,47 . Most studies obtained written consent and approval through an institutional review board or ethics committee.…”

“…Most studies obtained written consent and approval through an institutional review board or ethics committee. Eleven studies reported that patients were in steady state at the time of myocardial strain evaluation, defined as not being in any state of crisis or experiencing an acute event, generally for a minimum of 1 month 15,32,34–37,39–42,44,47 . The remaining six studies did not indicate timing of the patients’ last crisis in relation to myocardial strain evaluation (Table 3).…”

“…A growing number of studies have evaluated myocardial strain in individuals with SCD, but collective results have not yet been summarized. This systematic review is warranted to synthesize reported findings and remaining gaps in knowledge 15,31–47 …”

Evaluation of cardiac function in patients with sickle cell disease with left ventricular global longitudinal strain

“…Bedirian et al, [15] in a more recent research, found no difference in LV strain between a group of patients with SCD and a group of patients with other sickle cell diseases (OSCD). However, they found 79% of the patients with SCD to have abnormal strain, which is more than that we found but with a slightly higher cutoff for normal strain.…”

“…However, in this population, studies are rare and results are conflicting. [11][12][13][14][15][16] More recently, the GLS was found to be altered in a population of patients with β-thalassemia, [17,18] a hemoglobinopathy that can, in some respects, be compared with SCD.…”