Clinical outcome of patients with familial hypercholesterolemia and coronary artery disease undergoing partial ileal bypass surgery

Familial hypercholesterolemia is characterized by high serum levels of total cholesterol and LDL-cholesterol. It may be homozygous or heterozygous. In homozygous patients, LDL-cholesterol levels range from 500 to 1000 mg/dL and coronary artery disease is precocious, usually manifesting itself betwee...

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Bibliographic Details
Published in:Arquivos brasileiros de cardiologia 2000-07, Vol.75 (1), p.49-58
Main Authors: Issa, J S, Garrido, Jr, A, Giannini, S D, Forti, N, Diament, J, Pinotti, H W
Format: Article
Language:English
Subjects:
Adult
Anastomosis, Surgical
Anticholesteremic Agents - therapeutic use
Cecum - surgery
Coronary Disease - surgery
Female
Follow-Up Studies
Humans
Hyperlipoproteinemia Type II - surgery
Hyperlipoproteinemia Type II - therapy
Ileum - surgery
Male
Treatment Outcome
Xanthomatosis - surgery
Xanthomatosis - therapy
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Summary:Familial hypercholesterolemia is characterized by high serum levels of total cholesterol and LDL-cholesterol. It may be homozygous or heterozygous. In homozygous patients, LDL-cholesterol levels range from 500 to 1000 mg/dL and coronary artery disease is precocious, usually manifesting itself between the 2nd and 3rd decades of life. The diagnosis is often made by the presence of xanthoma tuberosum and tendinous xanthomas that appear between the 1st and 2nd decades of life. The use of high doses of statins or even unusual procedures (apheresis, partial ileal bypass surgery, liver transplantation, gene therapy), or both, is necessary for increasing survival and improving quality of life, because a reduction in cholesterol levels is essential for stabilizing the coronary artery disease and reducing xanthomas. We report our experience with 3 patients with xanthomatous familial hypercholesterolemia and coronary artery disease, who underwent partial ileal bypass surgery. Their follow-up over the years (approximately 8 years) showed a mean 30% reduction in total cholesterol, with a significant reduction in the xanthomas and stabilization of the coronary artery disease.
ISSN:0066-782X