Aggressive management with LDL apheresis and percutaneous balloon dilation can successfully treat severe cardiac involvement in pediatric familial hypercholesterolemia.
May support combined LDL apheresis and angioplasty in rare severe pediatric cases; hypothesis-generating and requires prospective validation.
A 13-year-old boy with double heterozygosity for familial hypercholesterolaemia with a 90% left coronary artery main stem stenosis is reported. The patient's cholesterol levels were effectively controlled with weekly sessions of selective low-density lipoprotein cholesterol removal through immunoadsorption by use of an extracorporeal system. Left main coronary artery stenosis was successfully treated with percutaneous transluminal balloon dilation. At 30 months after the intervention and still under treatment with weekly sessions of low-density lipoprotein apheresis the patient is free of cardiac symptoms. He shows normal exercise capacity and normal myocardial perfusion. It is concluded that aggressive management is justified in such patients and may result in a near-normal quality of life.
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Pfammatter et al. (1996) studied this question.
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