Left ventricular remodeling in patients with sickle cell disease: determinants factors and impact on outcome

Abstract

Cardiovascular complications have been increasingly detected as a result of prolonged longevity of patients with sickle cell disease (SCD). Previous studies have focused especially on pulmonary hypertension and its consequences on the right-side heart chambers, whereas factors associated with morphological changes in left ventricle (LV) remain poorly understood. This study was designed to identify clinical, laboratorial, and echocardiographic parameters associated with LV remodeling and its impact on outcome in SCD. Ninety patients aged 28 ± 7 years and 20 age- and gender-balanced healthy subjects were enrolled. Laboratory tests, electrocardiogram, and an echocardiogram with tissue Doppler imaging were performed in all patients. Patients with SCD had larger left and right heart chambers dimensions, LV mass, and tricuspid regurgitation (TR) velocity compared to health controls with similar demographic features. Despite chambers enlargement, systolic function of both ventricles was preserved. The mitral inflow velocities were higher in the patients than in controls, whereas septal and lateral annular motion velocities were normal, suggesting normal ventricular relaxation. SCD patients who were on hydroxyurea therapy and/or hypertransfusion had higher hemoglobin concentrations, but similar echocardiographic findings in comparison to those without treatment. Systolic blood pressure, ferritin concentration, TR velocity, and parameters of diastolic function were independently associated with increased LV mass. In addition, the predictors of adverse events were ferritin concentration, lactate dehydrogenase levels, and TR velocity. LV remodeling in SCD patients seems to be influenced by a combination of factors including blood pressure, ferritin concentration, TR velocity, and parameters of LV diastolic function, and was not associated with adverse outcomes.

Computer Algorithm‐Based Hydroxyurea Dosing Facilitates Titration to Maximum Tolerated Dose in Sickle Cell Anemia

“…42 An adult study found no difference in systolic or diastolic function in patients receiving hydroxyurea or transfusions compared with untreated adults with SCD. 43 Our study did not find a significant difference in ejection fraction, tricuspid regurgitant velocity, or 6minute walk distance. Therefore, hydroxyurea dosing for 1 year was not associated with significant improvement in cardiopulmonary function.…”

“…Although 1 analysis reported no difference in measures of systolic function between children on and off hydroxyurea, the authors noted improved diastolic function in the hydroxyurea group 42 . An adult study found no difference in systolic or diastolic function in patients receiving hydroxyurea or transfusions compared with untreated adults with SCD 43 . Our study did not find a significant difference in ejection fraction, tricuspid regurgitant velocity, or 6‐minute walk distance.…”

Speckle tracking evaluation of right ventricular functions in children with sickle cell disease

“…Left ventricular dysfunction in patients with SCD has been reported in several studies. [ 10 ] The evaluation of right ventricular function is more challenging compared to the left ventricle. The speckle tracking method is more superior over ordinary tissue Doppler techniques because it is angle independent.…”

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

“…These discrepancies reflect the difficulty in finding a reliable index of function in patients with a chronic overload due to anemia. [3][4][5][6][7] Most methods for evaluating systolic function are highly dependent on volume. This is particularly true in evaluating the left ventricular ejection fraction (LVEF), which remains the most commonly used parameter for evaluating left ventricular systolic function in routine clinical practice.…”