Coronary artery bypass grafting in familial hypercholesterolemia.
Familial hypercholesterolemia is an autosomal dominant disorder caused by a mutation of the gene for the low-density lipoprotein receptor and is characterized by rapidly progressing coronary atherosclerosis. We assessed the long-term results of coronary artery bypass grafting performed during the past 13 years in 62 patients with heterozygous familial hypercholesterolemia, whose mean plasma total and low-density lipoprotein cholesterol level was 327 mg/dl, respectively. The patients had severe coronary atherosclerosis, with coronary stenosis index of 19.7, and the prevalence of extracoronary atherosclerotic lesions was 27%. Sixty-one patients underwent successful coronary artery bypass operation, with an average of 2.5 grafts, and the coronary stenosis index decreased to 7.1. After operation, all patients consumed a cholesterol-lowering diet and received drug therapy with pravastatin, probucol, or cholestyramine. Seven patients who were resistant to drug therapy were treated with plasma low-density lipoprotein apheresis. The cholesterol-lowering therapy reduced plasma total cholesterol level by 37%, low-density lipoprotein cholesterol level by 42%, and low-density lipoprotein/high-density lipoprotein cholesterol ratio by 37% (p < 0.001). During the follow-up period (mean, 52 months; range, 10 to 157 months), there was no cardiac death, but three patients died of malignant disease. The actuarial survival rate was 95% at 5 years and 89% at 12 years after operation. The actuarial freedom from recurrent angina was 90% at 5 years and 53% at 11 years after operation. Four patients underwent reoperation, an average of 8 years postoperatively, because of vein graft atherosclerosis. In spite of severe coronary atherosclerosis, these patients with familial hypercholesterolemia showed good long-term outcome after coronary artery bypass operation. The present findings suggest that aggressive use of arterial grafts, intensive cholesterol-lowering drug therapy, and low-density lipoprotein apheresis may be useful in patients with familial hypercholesterolemia.
Kawasuji
M
M
Department of Surgery, Kanazawa University School of Medicine, Japan.
Sakakibara
N
N
Takemura
H
H
Matsumoto
Y
Y
Mabuchi
H
H
Watanabe
Y
Y
D000206:Actuarial Analysis; D000924:Anticholesteremic Agents / Q000627:therapeutic use; D001781:Blood Component Removal; D002791:Cholesterol, Dietary / Q000008:administration & dosage; D001026:Coronary Artery Bypass; D003324:Coronary Artery Disease / Q000453:epidemiology / Q000209:etiology* / Q000601:surgery*; D003327:Coronary Disease / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D006579:Heterozygote; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000150:complications* / Q000453:epidemiology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D011182:Postoperative Care; D012086:Reoperation; D013997:Time Factors; D016896:Treatment Outcome
D000924:Anticholesteremic Agents; D002791:Cholesterol, Dietary