Altered ventricular longitudinal strain in children with sickle cell disease: Role of TGF- and IL-18

Background: Cardiovascular involvement in sickle cell disease (SCD) has a great impact on patients' morbidity and mortality. Recently, interleukin-18 (IL-18) and transforming growth factor beta (TGF-beta) were suggested as potential biomarkers for sickle cell cardiomyopathy. Global longitudinal strain (GLS) is a reliable early parameter for estimation of deformed myocardium. This study evaluated the role of TGF-beta and IL-18 as risk indicators of altered strain in patients with SCD.Methods: Forty children with SCD (age >5 years) and 40 healthy children as controls, matched in age and sex, were enrolled in the study. All participants were subjected to clinical examination, complete blood count, serum ferritin, TGF-beta, IL-18, and assessment of cardiac function by echocardiography.Results: TGF-beta, IL-18, and lactic acid dehydrogenase (LDH) were significantly higher among cases (mean age: 10.6 +/- 3.5 years) when compared to controls (p < .001), at cutoff values 41.7 ng/mL, 128.9 pg/mL, and 340 unit, respectively. The LS of free wall of RV (FW-RV) was significantly lower among cases when compared to controls (-23.55% +/- 5.55% vs. -28.73% +/- 2.43%, p < .001). Free wall longitudinal strain of the right ventricle (FWLS-RV) was significantly correlated to IL-18 and LDH (p < .001), while GLS-RV was significantly correlated to TGF-beta. The GLS-LV was correlated to frequency of vaso-occlusive crises (VOCs) per year (p < .001). Diastolic function, E/A of LV, and RV were negatively correlated to the hemoglobin and serum ferritin levels.Conclusions: The TGF-beta, IL-18, and LDH along with frequent VOCs are correlated to altered LS, especially the right ventricle, and could serve as risk indicators for subclinical cardiomyopathy in children with SCD.