Kennedy J H, Tedgui A, Mesnildrey P, Laborde F, Lavergne A, Piwnica A
Cardiovascular Surgery, Hôpital Lariboisiere, Paris, France.
Atherosclerosis. 1988 Sep;73(1):33-7. doi: 10.1016/0021-9150(88)90160-8.
The morphologic, biochemical, and mechanical abnormalities of connective tissue fibrous proteins in Marfan's syndrome have been well studied, and their role in cardiovascular complications is well accepted. Less is known, however, about the state of the amorphous components of the aortic connective tissue. In the course of a study of transmural transport in blood vessels, we have had the opportunity to study dystrophic aorta from two young men who survived elective surgery; both with aortic insufficiency (AI) histologically compatible with Marfan's syndrome. One had recurrent chronic dissecting aneurysm (RCDA) as well. The aorta of the first (but not the second) was histologically compatible with Marfan's syndrome. Fresh specimens of intact ascending aorta were incubated in Krebs solution, pH 7.4, containing 125I-labelled bovine serum albumin for 2 h at 37 degrees C. The samples were then frozen, and serially sectioned in the plane of the lumenal surface. The radioactivity of the 20-micron thick sections was then determined, and expressed as a tissue/labelled solution concentration ratio. Transmural profiles of these ratios revealed no difference between the aorta of the RCDA patient with non-specific aortic dystrophy, and that of a 70-year-old man undergoing aortocoronary bypass. However, in the patient with aortic histology compatible with Marfan's syndrome, the average media concentration ratio was 5-fold less (4% vs. 20%).
马凡氏综合征中结缔组织纤维蛋白的形态学、生化和力学异常已得到充分研究,其在心血管并发症中的作用也已得到广泛认可。然而,对于主动脉结缔组织无定形成分的状态,人们了解较少。在一项关于血管跨壁运输的研究过程中,我们有机会研究了两名择期手术后存活的年轻男性的营养不良性主动脉;两人均患有与马凡氏综合征组织学相符的主动脉瓣关闭不全(AI)。其中一人还患有复发性慢性夹层动脉瘤(RCDA)。第一名患者的主动脉在组织学上与马凡氏综合征相符(第二名不符)。将完整升主动脉的新鲜标本在含有125I标记牛血清白蛋白的pH 7.4的 Krebs溶液中于37℃孵育2小时。然后将样品冷冻,并在管腔表面平面上连续切片。接着测定20微米厚切片的放射性,并表示为组织/标记溶液浓度比。这些比值的跨壁分布显示,患有非特异性主动脉营养不良的RCDA患者的主动脉与一名接受主动脉冠状动脉搭桥手术的70岁男性的主动脉之间没有差异。然而,在主动脉组织学与马凡氏综合征相符的患者中,中膜平均浓度比低5倍(4%对20%)。