Becker A E, van Mantgem J P
Am J Cardiol. 1975 Sep;36(3):315-21. doi: 10.1016/0002-9149(75)90482-8.
The coronary arteries of five hearts of patients known to have had Marfan's syndrome were studied microscopically. In four of the five cases the extramural coronary arteries showed structural changes consisting of accumulations of acid mucopolysaccharides and a concomitant disruption of the connective tissue elements. These changes closely resembled the changes that frequently occur in the aorta and that are generally described as "cystic medial necrosis". They are considered to be an important expression of wall weakening. In one case a laceration, probably traumatic, of a coronary artery led to formation of an "incomplete dissecting aneurysm." "Cystic medial necrosis" was found once in the proximal segment of the sinus nodal artery and once in the musculoelastic layer of a coronary artery that reached the atrioventricular node from the ventricular septum by perforating the anulus fibrosus. However, in contrast to findings in a previous report, the intramural coronary arteries were otherwise unaffected. The clinical significance of these data is as yet unknown particularly since these alterations are intramural and initially do not cause narrowing of the lumen. Nevertheless, the possibility of coronary arterial involvement by the metabolic disorder of Marfan's syndrome should always be considered during clinical assessment of this lesion.
对已知患有马方综合征的5例患者的心脏冠状动脉进行了显微镜检查。在5例中的4例中，壁外冠状动脉显示出结构变化，包括酸性粘多糖的积聚以及结缔组织成分的伴随破坏。这些变化与主动脉中经常出现的变化非常相似，通常被描述为“囊性中层坏死”。它们被认为是管壁变薄的重要表现。在1例中，冠状动脉的撕裂伤（可能是外伤性的）导致了“不完全夹层动脉瘤”的形成。在窦房结动脉的近端段以及一条通过穿破纤维环从室间隔到达房室结的冠状动脉的肌弹性层中各发现1次“囊性中层坏死”。然而，与之前一份报告的结果相反，壁内冠状动脉在其他方面未受影响。这些数据的临床意义尚不清楚，尤其是因为这些改变发生在壁内，最初不会导致管腔狭窄。尽管如此，在对该病变进行临床评估时，应始终考虑马方综合征代谢紊乱累及冠状动脉的可能性。
