Cardiovascular Imaging

Sickle cell disease

hereditary defect in haemoglobin formation causing rigid and fragile red blood cells which results in chronic anaemia and blockage of small blood vessels in various organs. Patients homozygous for S haemoglobin become symptomatic after about 6 months of age. The cardiopulmonary system is frequently involved. The chronic anaemia causes a high output state with volume overload of the heart. Sickle cell crises may be associated with pulmonary infarctions resulting from thromboses in small pulmonary arteries. Pulmonary hypertension and cor pulmonale are rare complications. Acute myocardial infarction due to in situ thrombosis of coronary arteries is a rare complication. In most patients with sickle cell disease, cardiomegaly eventually ensues and a cardiomyopathy may occur. Echocardiography is usually employed to demonstrate left ventricular hypertrophy and dilatation and to monitor left ventricular function.

CBH