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Although atherosclerotic disease of the coronary arteries is the most common cause of luminal narrowing and coronary heart disease, there are multiple nonatherosclerotic (congenital and acquired) causes of severe luminal narrowing and subsequent clinical coronary events (angina pectoris, acute myocardial infarction, and sudden death) (Table 55–1).

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Table 55–1. Nonatherosclerotic Causes of Coronary Artery Disease (Coronary Heart Disease)
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Various nonatherosclerotic coronary artery diseases can reduce or interrupt coronary blood flow by three mechanisms: (1) fixed luminal obstructions (internal narrowing), (2) encroachment of the lumen by disease of the arterial wall or adjacent tissues (external narrowing), or (3) both.1 Reduction in coronary arterial blood flow may also result from dynamic changes in the walls of an otherwise normal artery (spasm) or from a disproportion of myocardial oxygen supply and demand. In view of current trends toward rapid coronary artery reperfusion to salvage jeopardized myocardium during evolving acute myocardial infarction, the various nonatherosclerotic etiologies of coronary artery disease must be considered.

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Approximately 4% to 7% of all patients with acute myocardial infarction and nearly four times this percentage of patients younger than age 35 years do not have atherosclerotic coronary artery disease as demonstrated by coronary arteriography, at necropsy, or both.1-6 Because coronary angiography simply represents an image of one lumen, the specificity for etiology of the coronary luminal narrowing is extremely low. Review of necropsy studies1-3 suggests that approximately 95% of patients with fatal acute myocardial infarction have at least one major epicardial coronary artery with severe luminal narrowing or total occlusion (Fig. 55–1). The remaining 5% of patients apparently have normal major epicardial coronary arteries. Of ...

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