Abstract
Focal fibromuscular dysplasia of small coronary arteries is not so rare as it is unrecognized. Although sometimes occurring as an isolated abnormality, it more often accompanies a variety of other lesions including inflammation or infiltration. In this review based on personal study of over 1,000 human hearts, the 3 topics include a description of the morphologic characteristics of the lesion, a discussion of its functional consequences affecting coronary flow, and an iteration of theoretical explanations for its development. The typical lesion is focal in distribution, is comprised of both fibrous and smooth muscle elements, and the histologic organization is one of dysplastic array. Included among the subjects discussed in functional consequences are coronary spasm, coronary reserve, chest pain, electrical instability of the heart, and comments on the role of focal fibromuscular dysplasia of small coronary arteries in hypertension, myocardial hypertrophy and heart failure. Theories as to its development include primary faults of smooth muscle or collagen, and focal abnormalities of clotting or neurovascular relation, but it is likely that the cause is multifactorial.
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