Coronary artery bypass surgery with arterial grafts in familial hypercholesterolemia

Objective: Familial hypercholesterolemia is a dominantly inherited disorder caused by mutations at the locus for the low-density lipoprotein receptor and is frequently associated with premature coronary artery disease, This s tudy was performed to determine whether arterial grafting was associated wi th long-term benefits for patients with familial hypercholesterolemia. Methods: During the past 18 years, 101 patients with heterozygous familial hypercholesterolemia underwent primary coronary artery bypass grafting, wit h one hospital death. Group 1 patients (n = 31) received only saphenous vei n grafts. Group 2A patients (n = 47) received one internal thoracic artery graft and supplemental vein grafts, and group 2B patients (n = 23) had mult iple arterial grafts. After operation, all patients received diet therapy a nd intensive cholesterol-lowering drug therapy. Thirteen patients received low-density lipoprotein apheresis, Results: During a mean follow-up period of 95 months, 8 patients died, 9 un derwent reoperation, and 12 received catheter intervention. The overall sur vival was 82% (95% confidence limits, 65%-97%) at 18 years after operation. The survival in group 2 was higher than that found in group I (P = .01), T he overall freedom from major cardiac events (myocardial infarction, cardia c death, reoperation, and catheter intervention) was 57% (95% confidence li mits, 40%-74%) at 16 years after operation. The freedom from reoperation in group 2 was higher than that found in group I (P = .03), There was no diff erence in the survival or freedom from major cardiac events between groups 2A and 2B. Conclusion: Arterial grafting improved the long-term freedom from reoperati on in patients with familial hypercholesterolemia. Additional benefit of mu ltiple arterial grafting could not be identified.