Clinical manifestations of cardiovascular abnormalities in patients with sickle cell (SC) anemia are well documented. Many variables were assessed in our study before and after administration of L-carnitine to randomly selected 37 SC disease (SCD) children for a period of 6 months. Variables such as weight, height, serum ferritin levels, units of blood transfused and the number of veno-occlusive crises all showed significant improvement after the 6 months of therapy with L-carnitine. Our study also showed that cardiac diastolic function and pulmonary hypertention are common in pediatric SCD patients. These two disorders showed some improvement after L-carnitine administration. Therefore, L-carnitine deserves a rigorous large-scale randomized clinical trial to evaluate its potential benefits as treatment for SCD patients with cardiac complications.
To evaluate the right ventricular (RV) function in relation to that of the left ventricle (LV) in patients with dilated cardiomyopathy (DCM). Echocardiographic examination was done using tissue Doppler imaging (TDI) and two-dimensional speckle tracking echocardiography (2D-STE) for 32 pediatric patients with DCM comparing them to another 32 normal matched controls. The global longitudinal strain (GLS) derived from 2D-STE was used to reflect the LV systolic function. Tricuspid annular plan systolic excursion (TAPSE) and the following RV TDI derived indexes: peak systolic velocity (S'), peak early diastolic velocity E', peak late diastolic velocity A', isovolumic acceleration (IVA) and myocardial performance index (MPI) were measured. RV had significant systolic and diastolic dysfunction; TAPSE, S' velocity, IVA, peak early diastolic velocity (E') and peak early diastolic velocity/peak late diastolic velocity (E'/A') ratio were significantly decreased while MPI was significantly prolonged compared to controls. Moreover, TAPSE, S', IVA, E', E'/A' and RV MPI were significantly correlated to LV GLS. For prediction of LV dysfunction among patients, the area under the receiver operating characteristic curve was 0.98 for RV MPI, 0.906 for RV IVA. For identifying severe LV dysfunction; RV MPI> 0.29 had 100% sensitivity and 93.7% specificity, while the RV IVA ≤ 3 had 84.4% sensitivity and 90.6% specificity. In pediatric patients with DCM the RV systolic and diastolic functions are affected beside the LV dysfunction. Non-conventional echocardiographic evaluation of RV function is recommended in among this cohort.
IntroductionTissue Doppler imaging (TDI) is a relatively new echocardiography method in children with chronic kidney disease (CKD). The aims of this study were to evaluate left ventricular (LV) diastolic function in pediatric CKD patients using conventional pulsed-wave Doppler echocardiography (cPWD) and TDI methods and correlate them with BNP levels.Material and methodsThirty children on regular hemodialysis (HD) were included. Left ventricular systolic and diastolic indices and BNP levels were measured immediately before and after HD.ResultsAfter HD, LV and left atrium diameters were significantly decreased (p < 0.001 and p < 0.001, respectively). Transmitral E velocity and E/A ratio decreased (p < 0.001 and p < 0.001, respectively). Also, there were significant decreases in the early diastolic velocity E’ of LV wall, septal wall, and anterior wall (p = 0.001, p = 0.004 and p < 0.001, respectively). Mean E/E’ ratio and ratios of septal and lateral walls were decreased significantly (p = 0.004, p = 0.002 and p = 0.017, respectively). BNP levels decreased significantly during HD when comparing concentrations before and after HD (p < 0.001). BNP strongly correlated with LV diastolic function indices (before HD: mean E/E’ (r = 0.401, p = 0.028), lateral E/E” (r = 0.291, p = 0.025), septal E’(r = –0.398, p = 0.029), lateral wall E’ (r = –0.452, p = 0.012) and mean E’ (r = –0.469, p = 0.009), after HD: mean E/E’ (r = 0.38, p = 0.038) and lateral E/E” (r = 0.474, p = 0.008) and lateral wall E’ (r = –0.270, p = 0.037)).ConclusionsThe cPWD and TDI-derived LV indices are influenced by HD. The diagnostic utility of BNP in the presence of deteriorating renal function may be compromised to some extent.
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