在1993年的报告中Oser和bloor[[1]描述了具有可撞击慢性血栓栓塞性疾病患者的一系列微血管“肺高血压”病变。尽管得出的结论是,在任何一名患者中的这些发现都没有“排除阳性的血液动力学和临床结果”,但这些早期观察成为我们当前对肺动脉高压和正确心力衰竭进化的理解的基础。,有组织的有组织的血栓栓塞疾病[2那3]。尽管微循环在慢性血栓栓塞肺动脉高压(CTEPH)中的重要性和作用,但我们对组织病理学的理解仍然相当基本[4] and previously confined to descriptions of the precapillary pulmonary circulation [5.那6.]。因此,D的报告Orfmüller等等。[[7.] in the current journal is a timely re-examination of this subject. In their report, the authors included analysis of the native lungs from 16 select patients with CTEPH receiving lung transplantation and in one case through an autopsy. This combined series consisted both of CTEPH cases deemed inoperable (n=8) and CTEPH patients with residual pulmonary hypertension after pulmonary endarterectomy (PEA) (n=9). In addition to previously reported defects seen in the pulmonary arteries, they discovered abnormalities in the capillary bed (similar to defects seen in pulmonary capillary haemangiomatosis) as well as the post-capillary circulation with involvement of the pulmonary veins. The observed degree of venous fibrosis/remodelling and haemangiomatosis-like foci was reported to be of moderate intensity and less pronounced than those seen in pulmonary veno-occlusive disease. Nevertheless, this represents a unique histopathologic description of capillary and post-capillary defects in CTEPH, and widens our awareness of the spectrum of microvascular defects in this form of pulmonary hypertension.
The clinical significance of this report, however, is unclear and the authors were prudent to conclude that these observations required further investigation. It is widely regarded that PEA is the optimal treatment for patients with (CTEPH) [8那9.]。该外科手术涉及去除阻塞近端肺部血管的慢性有组织的血栓。并且无法解决即使是最有经验的豌豆外科医生的潜在伴随的微血管疾病。在大多数经历有利的肺血液动力学结局的患者中,尤其是在术前严重肺动脉高压的患者中,在这些情况下是否没有观察到的微血管变化相似?如果是这样,是否仅仅是微血管受累程度的问题,将那些在手术中表现良好的患者和不进行的患者分开?正如作者提到的那样,可能会开发出哪些成像或诊断工具,以定量微血管疾病的参与体内to better exclude those patients unlikely to benefit from an endarterectomy. Do these adaptive vascular changes “de-model” after a successful endarterectomy with the relief of the post-obstructive pressure gradient; as we know happens with the bronchial systemic vessels [10]?这些微血管发现中的一些,尤其是观察到毛细血管后静脉重塑和支气管对肺静脉分流的观察,对再灌注肺损伤和内膜切除术后肺出血的发展很重要吗?就像在CTEPH中对小血管病理学的初始描述一样,DOrfmüller等等。[[7.] raises yet more questions than it seems to answer, which of course is the hallmark of all important observations.
Footnotes
Conflict of interest: Disclosures can be found alongside the online version of this article atwww.qdcxjkg.com
- R.eceived2014年8月25日。
- 公认一种ugust 26, 2014.
- ©ERS 2014