Myocardial infarction in a 7 1/2 year old child with familial hypercholesterolemia

We report on a 7 1/2 year old child with familial homozygous hypercholester olemia, insuccessfully treated by low cholesterol diet and cholesterol lowe ring drugs over a period of 4 1/2 years. As a complication of the third IDL apherese procedure, anemia had developed, followed by a myocardial infarct ion. Coronary arteriogram showed significant coronary artery disease with m ain stem stenosis and markedly impaired leftventricular contractility. Hypo tension and bradycardia at the end of the invasive study prompted urgent ao rtocoronary by pass operation. Despite successful insertion of a venous gra ft, the patient die postoperatively in a low cardiac output. Discussion:This case illustrates the high probability of familiar homozygou s hypercholesterolemia of having coronary artery disease at an early age wh ich can be complicated by myocardial infarction. It underlines the urgent n eed for prompt reduction of cholesterol and LDL levels with apheresis to pr event premature atherosclerotic death. In contrast to angiography two dimen sional echocardiography laks sufficient sensitivity to demonstrate stenotic lesion of the coronary arteries in children. Therefore, coronary angiograp hy should be performed, if stenosis lesions are suspected.