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 an d is characterized by rapidly progressing coronary atherosclerosis. We assessed the long-term results of coronary artery bypass grafting per formed during the past 13 years in 62 patients with heterozygous famil ial hypercholesterolemia, whose mean plasma total and low-density lipo protein cholesterol level was 327 mg/dl and 238 mg/dl, respectively. T he patients had severe coronary atherosclerosis, with coronary stenosi s index of 19.7, and the prevalence of extracoronary atherosclerotic l esions was 27%. Sixty-one patients underwent successful coronary arter y bypass operation, with an average of 2.5 grafts, and the coronary st enosis 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 dr ug therapy were treated with plasma low-density lipoprotein apheresis. The cholesterol-lowering therapy reduced plasma total cholesterol lev el by 37%, low-density lipoprotein cholesterol level by 42%, and low-d ensity 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 m alignant disease. The actuarial survival rate was 95% at 5 years and 8 9% at 12 years after operation. The actuarial freedom from recurrent a ngina was 90% at 5 years and 53% at 11 years after operation. Four pat ients underwent reoperation, an average of 8 years postoperatively, be cause of vein graft atherosclerosis. In spite of severe coronary ather osclerosis, these patients with familial hypercholesterolemia showed g ood long-term outcome after coronary artery bypass operation. The pres ent findings suggest that aggressive use of arterial grafts, intensive cholesterol-lowering drug therapy, and low-density lipoprotein aphere sis may be useful in patients with familial hypercholesterolemia.