Carotid bifurcation atherosclerosis was demonstrated in 34 of 108 patients with familial hypercholesterolemia and coronary artery disease by B-scan, continuous-wave Doppler sonography, and intravenous digital subtraction anglography. An intensive combined therapy of diet, colestipol, and nicotinic acid was mounted to control the hypercholesterolemia of these patients. Their serial sonographies and digital subtraction anglography were evaluated independently by technical specialists who served as coinvestigators. The data obtained suggest that 1) extracranial arterial disease can develop concurrently with coronary artery disease in a significant proportion of patients with familial hypercholesterolemia, and 2) amaurosis fugax, transient ischemic attack, cerebral infarction, and myocardial infarction did not recur during 58-72 months of control of familial hypercholesterolemia in this series of patients. Increased susceptibility to premature coronary artery disease (CAD) has been well documented. 23 There is scant information about the prevalence of extracranial carotid arterial disease in patients with familial hypercholesterolemia, particularly in those patients with known CAD. Because ultrasonography and intravenous digital subtraction angiography (IV-DSA) 4 "* have created a safe and reliable means to evaluate extracranial arterial lesions, we used these techniques to determine the possible effect of lowering cholesterol and lipid on the carotid bifurcation pathology in patients with familial hypercholesterolemia. Our goal was to determine whether long-term control of hypercholesterolemia would reduce the excess risk for stroke in a series of familial hypercholesterolemic patients with extracranial carotid arterial disease and premature CAD. Received May 15, 1985; accepted February 6, 1987.
Subjects and Methods
Patient PopulationOne hundred eight unselected patients with familial hypercholesterolemia and CAD (angina and/or postmyocardial infarction), aged 39-62, were evaluated. Diagnosis of heterozygous familial hypercholesterolemia was established by the demonstration of persistent hypercholesterolemia (serum total cholesterol s 300 mg/dl, LDL cholesterol ^1 9 0 mg/dl, and triglyceridê 200 mg/dl); elevated LDL cholesterol that responded minimally to a diet low in cholesterol and saturated fat; demonstration of a similar plasma lipid-lipoprotein abnormality with or without clinical CAD in firstdegree family members; and characteristic clinical manifestations of familial hypercholesterolemia including tendinous xanthomas with or without corneal arcus.3 After obtaining informed consent, 34 patients in whom extracranial carotid arterial atherosclerosis had been demonstrated were enrolled in a diet-drug treatment program for long-term management of hypercholesterolemia. Women of childbearing age and patients with secondary hypercholesterolemia or who were receiving drugs or hormones known to exert significant effects on lipid metabolism were excluded from the study.
Laboratory DeterminationsDiagnosis of CAD was es...