James T N
World Health Organization Cardiovascular Center, Galveston, Texas.
Am J Cardiol. 1990 Apr 3;65(14):12G-22G. doi: 10.1016/0002-9149(90)90954-y.
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.
小冠状动脉局灶性纤维肌发育不良并非罕见，只是未被识别而已。虽然有时作为孤立异常出现，但它更常伴有包括炎症或浸润在内的多种其他病变。在这项基于对1000多颗人类心脏的个人研究的综述中，三个主题包括病变形态学特征的描述、其对冠状动脉血流影响的功能后果的讨论以及对其发展的理论解释的阐述。典型病变分布局灶，由纤维和平滑肌成分组成，组织学结构为发育异常排列。在功能后果讨论的主题中包括冠状动脉痉挛、冠状动脉储备、胸痛、心脏电不稳定，以及对小冠状动脉局灶性纤维肌发育不良在高血压、心肌肥厚和心力衰竭中作用的评论。关于其发展的理论包括平滑肌或胶原的原发性缺陷以及凝血或神经血管关系的局灶性异常，但病因可能是多因素的。
