Portacaval shunt in patients with familial hypercholesterolemia

Portacaval shunt was performed in ten patients with homozygous and two with heterozygous familial hypercholesterolemia (FH). Total serum cholesterol was lowered by 20% to 55.4% during follow-up periods of 14 months to almost 9 years, with commensurate decreases in LDL cholesterol. The effect on HDL...

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Bibliographic Details
Published in:Annals of surgery 1983-09, Vol.198 (3), p.273-283
Main Authors: STARZL, T. E, CHASE, H. P, BENSON, L. N, AHRENS, E. H. JR, MCNAMARA, D. J, BILHEIMER, D. W, SCHAEFER, E. J, REY, J, PORTER, K. A, STEIN, E, FRANCAVILLA, A
Format: Article
Language:English
Subjects:
Adolescent
Adult
Biological and medical sciences
Cardiovascular Diseases - complications
Child
Child, Preschool
Cholesterol - blood
Disorders of blood lipids. Hyperlipoproteinemia
Female
Follow-Up Studies
Growth Disorders - etiology
Hepatic Encephalopathy - etiology
Humans
Hyperlipoproteinemia Type II - blood
Hyperlipoproteinemia Type II - complications
Hyperlipoproteinemia Type II - surgery
Intelligence Tests
Kidney Function Tests
Liver Function Tests
Male
Medical sciences
Metabolic diseases
Middle Aged
Portacaval Shunt, Surgical - adverse effects
Triglycerides - blood
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Summary:Portacaval shunt was performed in ten patients with homozygous and two with heterozygous familial hypercholesterolemia (FH). Total serum cholesterol was lowered by 20% to 55.4% during follow-up periods of 14 months to almost 9 years, with commensurate decreases in LDL cholesterol. The effect on HDL cholesterol and triglyceride levels was variable. Tendinocutaneous xanthomas diminished or disappeared. Growth and development in children proceeded or accelerated. There was no detectable emotional or intellectual deterioration. Hepatic failure did not occur, although blood ammonia concentrations and serum alkaline phosphatase levels increased relative to preoperative values. Cardiac symptoms were often improved, but evidence of reversal of cardiovascular lesions was inconclusive. Three patients with pre-existing heart disease died of cardiac complications after 4 months, 18 1/2 months, and 30 months. Portacaval shunt has been effective therapy for patients with FH who were refractory or intolerant to medical treatment; it should be performed before the development of irreversible cardiovascular damage.
ISSN:0003-4932
1528-1140
DOI:10.1097/00000658-198309000-00004