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 study was performed to determine whether arterial grafting was associated with 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, with one hospital death. Group 1 patients (n = 31) received only saphenous vein grafts. Group 2A patients (n = 47) received one internal thoracic artery graft and supplemental vein grafts, and group 2B patients (n = 23) had multiple arterial grafts. After operation, all patients received diet therapy and 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 underwent reoperation, and 12 received catheter intervention. The overall survival was 82% (95% confidence limits, 65%-97%) at 18 years after operation. The survival in group 2 was higher than that found in group 1 (P =.01). The overall freedom from major cardiac events (myocardial infarction, cardiac death, reoperation, and catheter intervention) was 57% (95% confidence limits, 40%-74%) at 16 years after operation. The freedom from reoperation in group 2 was higher than that found in group 1 (P =.03). There was no difference in the survival or freedom from major cardiac events between groups 2A and 2B.


CONCLUSIONS
Arterial grafting improved the long-term freedom from reoperation in patients with familial hypercholesterolemia. Additional benefit of multiple arterial grafting could not be identified.
Kawasuji
M
M
Department of Surgery (I), Kanazawa University School of Medicine, Kanazawa, Japan.
Sakakibara
N
N
Fujii
S
S
Yasuda
T
T
Watanabe
Y
Y
D000328:Adult; D000368:Aged; D000924:Anticholesteremic Agents / Q000627:therapeutic use; D008076:Cholesterol, HDL / Q000097:blood; D008078:Cholesterol, LDL / Q000097:blood; D017023:Coronary Angiography; D001026:Coronary Artery Bypass / Q000379:methods*; D003327:Coronary Disease / Q000097:blood / Q000401:mortality / Q000601:surgery*; D005260:Female; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000097:blood / Q000401:mortality / Q000601:surgery*; D007387:Internal Mammary-Coronary Artery Anastomosis; D008297:Male; D008875:Middle Aged; D010956:Plasmapheresis; D012086:Reoperation; D012189:Retrospective Studies; D012501:Saphenous Vein / Q000637:transplantation*; D015996:Survival Rate; D014280:Triglycerides / Q000097:blood
D000924:Anticholesteremic Agents; D008076:Cholesterol, HDL; D008078:Cholesterol, LDL; D014280:Triglycerides