Mazur Piotr, Gawęda Bogusław, Natorska Joanna, Ząbczyk Michał, Undas Anetta, Sadowski Jerzy, Kopeć Grzegorz, Waligóra Marcin, Podolec Piotr, Kapelak Bogusław
Institute of Cardiology, Jagiellonian University Medical College, Kraków, Poland.
The John Paul II Hospital, Kraków, Poland.
J Thromb Thrombolysis. 2016 Aug;42(2):212-7. doi: 10.1007/s11239-016-1382-z.
Pulmonary endarterectomy (PEA) is a curative therapeutic approach in patients with chronic thromboembolic pulmonary hypertension (CTEPH). The location-dependent structural differences of thrombotic material found in pulmonary arteries in CTEPH are poorly investigated. We present the case of a 47-year-old woman with antiphospholipid syndrome, diabetes mellitus and abnormal fibrin phenotype, who underwent PEA for CTEPH. Intravascular material removed bilaterally during PEA (from lobar, segmental and sub-segmental arteries) has been studied using light and scanning electron microscopy (SEM). Light microscopy showed tighter fibrous network in the portions of intraluminal thrombotic material facing the vessel wall, which contained collagen and fibrin fibers, and abundant cells. Cells, evaluated by immunostaining, were present in the whole removed material. Tissue factor expression was also observed with the highest values in the portions of intravascular material facing the vessel wall. In the main pulmonary arteries, SEM images revealed thick fibers of fibrous proteins loosly meshed and few erythrocytes and platelets between them (both dysmorphic "wedged" and fresh cells were present). In the fibrotic layers, containing mainly collagen and fibrin, removed from the lobar/segmental pulmonary arteries we found a stepwise increase in fiber density with decreasing vessel calibre, followed by denser fibrous networks composed of thinner fibers. Elastic fibers in the lobar and segmental arteries were aligned along the blood flow vector. These findings demonstrate differences in the structure of endarterectomized PEA material dependent on the vessel calibre and might contribute to understanding of CTEPH pathophysiology.
肺动脉内膜剥脱术(PEA)是治疗慢性血栓栓塞性肺动脉高压(CTEPH)患者的一种根治性治疗方法。目前对CTEPH患者肺动脉中血栓物质的位置依赖性结构差异研究较少。我们报告了一例47岁患有抗磷脂综合征、糖尿病且纤维蛋白表型异常的女性患者,她因CTEPH接受了PEA治疗。已使用光学显微镜和扫描电子显微镜(SEM)对PEA期间双侧切除的血管内物质(来自叶、段和亚段动脉)进行了研究。光学显微镜显示,腔内血栓物质面向血管壁的部分存在更紧密的纤维网络,其中包含胶原蛋白和纤维蛋白纤维,以及大量细胞。通过免疫染色评估,细胞存在于整个切除的物质中。在血管内物质面向血管壁的部分也观察到组织因子表达,其值最高。在主肺动脉中,SEM图像显示纤维蛋白的粗纤维松散地交织在一起,其间有少量红细胞和血小板(既有畸形的“楔状”细胞,也有新鲜细胞)。在从叶/段肺动脉切除的主要包含胶原蛋白和纤维蛋白的纤维化层中,我们发现随着血管管径减小,纤维密度呈逐步增加,随后是由更细纤维组成的更致密的纤维网络。叶动脉和段动脉中的弹性纤维沿血流方向排列。这些发现表明,内膜剥脱的PEA物质结构因血管管径而异,可能有助于理解CTEPH的病理生理学。