Microvascular disease in chronic thromboernbolic pulmonary hypertension: a role for pulmonary veins and systemic vasculature
Microvascular disease in chronic thromboernbolic pulmonary hypertension: a role for pulmonary veins and systemic vasculature
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DOI：
10.1183/09031936.00169113
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发表时间：
2014-11-01
影响因子：
24.3
通讯作者：
Mercier, Olaf
中科院分区：
文献类型：
--
作者：
Dorfmueller, Peter;Guenther, Sven;Mercier, Olaf
有限数量的慢性血栓栓塞性肺动脉高压（CTEPH）手术患者对肺动脉内膜切除术（PEA）无效，并出现持续性肺动脉高压（PH）。我们回顾性评估了9例持续性PH患者（PEA无效组）和8例PEA无法到达的远端CTEPH移植患者（noPEA组）的肺组织学。将显微镜下观察到的特性与最近开发的仔猪CTEPH模型的组织学进行比较。收集并分析了inPEA和noPEA组患者的介入前临床/血液动力学数据和病史。两组的组织学检查均显示肺小动脉/小动脉、间隔静脉和间隔前小静脉明显重塑，包括局灶性毛细血管瘤病，以及支气管系统血管明显肥大和扩大。在我们的猪CTEPH模型中再现了大多数发现。墨水注射实验揭示了在所谓的小血管或微血管疾病，以及毛细血管后支气管肺动脉分流在人类和实验CTEPH丰富的小静脉参与。微血管疾病部分是由于毛细血管后重塑在人类和实验CTEPH，似乎与支气管肺静脉分流。需要进一步的研究来临床评估这一发现的功能重要性。
Limited numbers of operated patients with chronic thromboembolic pulmonary hypertension (CTEPH) are refractory to pulmonary endarterectomy (PEA) and experience persistent pulmonary hypertension (PH).We retrospectively assessed lung histology available from nine patients with persistent PH (ineffective PEA (inPEA) group) and from eight patients transplanted for distal CTEPH inaccessible by PEA (noPEA group). Microscopically observed peculiarities were compared with the histology of a recently developed CTEPH model in piglets. Pre-interventional clinical/haemodynamic data and medical history of patients from the inPEA and noPEA groups were collected and analysed.Conspicuous remodelling of small pulmonary arteries/arterioles, septal veins and pre-septal venules, including focal capillary haemangiomatosis, as well as pronounced hypertrophy and enlargement of bronchial systemic vessels, were the predominant pattern in histology from both groups. Most findings were reproduced in our porcine CTEPH model. Ink injection experiments unmasked abundant venular involvement in so-called small vessel or microvascular disease, as well as post-capillary bronchopulmonary shunting in human and experimental CTEPH.Microvascular disease is partly due to post-capillary remodelling in human and experimental CTEPH and appears to be related to bronchial-to-pulmonary venous shunting. Further studies are needed to clinically assess the functional importance of this finding.