Evidence of diastolic Dysfunction and pulmonary hypertension in sickle cell anemia: possible role of L-carnitine treatment

ABSTRACT

The aim of this work was to assess cardiac status and pulmonary blood pressure in Egyptian sickle cell disease [SCD] pediatric patients. Possible role of L-carnitine in the amelioration of heart complications was also studied. This study was conducted on 37 pediatric patients with sickle cell disease, their mean age was 9.4 +/- 3.6 years. They were subjected to complete history taking, general examination, laboratory investigations [complete blood count and serum ferritin] and echocardiographic examination including measurement of cardiac chamber dimensions, systolic and diastolic functions of left ventricle and estimation of pulmonary artery systolic pressure [PASP]. The echocardiographic findings showed a significant increase in left atrial dimensions in SCD patients compared with the normal controls, which decreased after L-carnitine therapy but did not reach a significant difference. Other cardiac dimensions revealed no significant difference between the patients and control group. Systolic functions of left ventricle did not show any significant difference between patients and controls. Diastolic dysfunction was found in all patients. Diastolic parameters revealed a significant improvement by a decrease in E-wave peak velocity and E/A ratio after L-carnitine therapy. Pulmonary hypertension was found in 17 cases and showed a significant decrease after L-carnitine therapy. The clinical and laboratory re-evaluation of the patients after the period of therapy revealed a significant increase in weight and height of the patients together with a significant decrease in the frequency of blood transfusion and serum ferritin levels