[Sickle-cell cardiomegaly in children. Study of 13 cases]

Thirteen children, aged 18 months to 14 years and presenting with sickle cell anemia and cardiomegaly (Cardiothoracic ratio greater than or equal to 0.55) were investigated by echocardiography and radio-isotopic measurement of the cardiac output. Eight children presented with one or several criteria of anemic cardiopathy: left ventricular dilatation and hyperkinesia, increased cardiac index. On the contrary, 2 children presented with left ventricular dilatation and decreased contractility, without increase in the cardiac index; 3 children presented with echocardiographic findings similar to those of controls and a normal cardiac index, which is unusual in cases with chronic anemia. These results show that the cardiovascular system of children with sickle cell anemia does not always behave as one would expect in chronic anemia. They also suggest the possible early occurrence of cardiomyopathy, as already described in adults with sickle cell anemia.