[Cardiovascular manifestations of sickle-cell anemia in children]

Cardiovascular disorders are very frequent in children with homozygous sickle cell anaemia. They are due partly to the effects of chronic anaemia on the heart and vessels and partly to other factors, such as vaso-occlusive phenomena and desaturation of arterial oxygen, which alter the cardiac function. Because of reduced oxygen tension in arterial blood, the increase in resting cardiac output is more pronounced in sickle cell anaemia than in anaemias of other origins. Beside this "anaemic heart", myocardiopathies ascribed to multiple occlusions of small coronary vessels are well documented by pathological findings and by echocardiographic signs of left ventricular dysfunction. More rarely than adult children may develop chronic cor pulmonale due to large scale obstruction of the pulmonary vascular bed.