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Coronary artery bypass grafting in familial hypercholesterolemia
M Kawasuji1, N Sakakibara, H Takemura
1Department of Surgery, Kanazawa University School of Medicine, Japan.
Insights
Coronary artery bypass grafting offers good long-term survival for familial hypercholesterolemia patients. Aggressive cholesterol management, including diet, drugs, and apheresis, is crucial for successful outcomes.
Area of Science:
- Cardiology
- Genetics
- Vascular Surgery
Background:
- Familial hypercholesterolemia (FH) is an autosomal dominant disorder.
- FH causes severe, rapidly progressing coronary atherosclerosis.
- Low-density lipoprotein receptor gene mutations are the primary cause of FH.
Purpose of the Study:
- To assess long-term outcomes of coronary artery bypass grafting (CABG) in FH patients.
- To evaluate the effectiveness of cholesterol-lowering therapies post-CABG in FH.
- To determine survival and re-intervention rates after CABG in FH.
Main Methods:
- Retrospective analysis of 62 FH patients undergoing CABG over 13 years.
- Patients received cholesterol-lowering diet, drug therapy (pravastatin, probucol, cholestyramine), or LDL apheresis.
- Follow-up included assessment of cardiac events, survival, angina recurrence, and reoperations.
Main Results:
- Successful CABG in 61 patients, reducing coronary stenosis index from 19.7 to 7.1.
- Cholesterol-lowering therapy reduced total cholesterol by 37% and LDL by 42%.
- Actuarial survival was 95% at 5 years and 89% at 12 years; freedom from angina was 90% at 5 years.
Conclusions:
- CABG provides good long-term outcomes in FH patients despite severe atherosclerosis.
- Intensive cholesterol management (diet, drugs, apheresis) is vital post-CABG.
- Arterial grafts, aggressive lipid-lowering, and apheresis may improve results in FH patients.
Abstract:
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.