Laboratory of Surgical Research and INSERM U999, Hôpital Marie-Lannelongue and Paris-Sud University, Le Plessis Robinson, France.
Eur Respir J. 2013 May;41(5):1200-6. doi: 10.1183/09031936.00101612. Epub 2013 Jan 11.
Chronic thromboembolic pulmonary hypertension (CTEPH) is a life-threatening disease due to pulmonary artery obstruction by persistent organised clots related to one or more episodes of acute pulmonary embolism. To date, the pathogenesis of CTEPH remains unexplained. Pulmonary endarterectomy removes obstruction from pulmonary vessels and can cure patients. However, some unreachable distal pulmonary obstruction and/or associated distal pulmonary vasculopathy could induce persistent pulmonary hypertension, the main postoperative complication. The pathophysiology of CTEPH is not fully understood and improving knowledge of this disease could improve our future surgical and medical management. Many attempts, conducted over several decades, have failed to reproduce this chronic disease in animals. However, several animal models have provided insights into the pathophysiology and pathogenesis of CTEPH. Here, we review all the animal models that have improved the comprehension of CTEPH and hold promise for further investigations. This short review analyses strengths and weaknesses of all animal models available to study the pathophysiology of CTEPH.
慢性血栓栓塞性肺动脉高压（CTEPH）是一种危及生命的疾病，其发病机制是肺动脉由于一次或多次急性肺栓塞而持续存在有组织的血栓导致阻塞。迄今为止，CTEPH 的发病机制仍未阐明。肺动脉内膜切除术可清除肺血管阻塞，从而治愈患者。然而，一些无法到达的远端肺阻塞和/或相关的远端肺血管病变可能导致持续性肺动脉高压，这是主要的术后并发症。CTEPH 的病理生理学尚未完全阐明，增加对这种疾病的认识可以改善我们未来的手术和医疗管理。几十年来，许多尝试都未能在动物身上重现这种慢性疾病。然而，一些动物模型为 CTEPH 的病理生理学和发病机制提供了一些认识。在这里，我们回顾了所有改善对 CTEPH 理解并有望进一步研究的动物模型。这篇简短的综述分析了所有可用于研究 CTEPH 病理生理学的动物模型的优缺点。
