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HEART ATTACK IN INDIVIDUALS WHO HAVE INHERITED HIGH BLOOD-CHOLESTEROL LEVELS
About three people in every thousand inherit a disorder in which cholesterol levels are high from birth. In some individuals this condition, familial hypercholesterolemia, is detected early in life, and most patients can be treated effectively to reduce the blood cholesterol. Affected people have irregular thickened tendons, due to cholesterol deposits; these are readily felt by the doctor. This leads to early diagnosis. The commonest sites are in the tendons at the back of the ankles, and in those on the backs of the hands. If the condition is not recognized and treated, affected men are at considerable risk of coronary disease; hence the importance of detecting the high cholesterol level as early as possible.
This condition has taught us a great deal about the hazards of having very high cholesterol levels. It is an example of how the development of coronary artery disease is accelerated by having a high blood-cholesterol level. The heart disease in most patients with familial hypercholesterolemia is due to typical coronary atherosclerosis; this was shown by Dr W. C. Roberts, a pathologist at the United States National Institutes of Health.
Because the condition is so uncommon it is not an important cause of heart attack in the community as a whole. But it does explain a proportion of the coronary disease occurring in very young people.
A thirty-five-year-old salesman was driving home one evening when he felt suddenly dizzy and faint. He pulled over to the kerb, collapsing over the steering wheel as he did so. Fortunately this was noticed by another motorist, and he was taken to hospital by ambulance within minutes.
A heart attack was diagnosed. He recovered quickly, but was left with angina. His coronary arteries were X-rayed, and all three branches were found to have severe atherosclerosis. He was operated upon; and lengths of vein were taken from his legs and were used to bypass the narrowed parts of his coronary arteries. The angina ceased.
Three months after the operation he was examined to seek any removable causes of his coronary disease. His blood pressure had never been high, nor had he ever smoked. He was lean and had been a regular squash player. An older brother had angina, and his father had died suddenly of a heart attack at the age of fifty-six.
His blood-cholesterol level was very high indeed. His brother was found to have an equally high level, and his four-year-old daughter also had a raised cholesterol level. Clearly the disorder was inherited.
He was treated with a diet low in saturated fat and cholesterol, and he eats certain foods rich in polyunsaturated fat. He also requires a drug to help keep his blood-cholesterol level normal. This is intended to reduce the risk of further damage to his arteries. His brother and daughter are receiving the same treatment.
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