Division of Pulmonary and Critical Care Medicine, University of California, San Diego, 9300 Campus Point Drive, La Jolla, CA 92037, USA.
Clin Chest Med. 2010 Dec;31(4):741-58. doi: 10.1016/j.ccm.2010.07.006.
Chronic thromboembolic pulmonary hypertension is one of the few forms of pulmonary hypertension that is surgically curable. It is likely underdiagnosed and must be considered in every patient presenting with pulmonary hypertension to avoid missing the opportunity to cure these patients. This article discusses the epidemiology, risk factors, natural history, diagnosis, and preoperative evaluation of patients with this disorder. Also covered are putative mechanisms for the conversion of acute emboli into fibrosed thrombembolic residua. Mechanical obstruction of the central pulmonary vasculature is rarely the sole cause of the pulmonary hypertension, and a discussion of the small vessel arteriopathy present in these patients is offered. Technical aspects of pulmonary endartectomy and the data supporting its role are discussed, as are the limited data on pulmonary arterial hypertension specific medical therapies for patients deemed noncandidates for the operation.
慢性血栓栓塞性肺动脉高压是少数几种可通过手术治愈的肺动脉高压类型之一。它可能被漏诊，因此在对肺动脉高压患者进行诊断时，必须考虑到这种疾病，以免错失治愈这些患者的机会。本文讨论了这种疾病的流行病学、危险因素、自然病程、诊断和术前评估。此外，还讨论了急性栓塞转变为纤维化血栓栓塞残留的推测机制。中央肺血管的机械阻塞很少是肺动脉高压的唯一原因，本文还讨论了这些患者存在的小血管血管病变。本文讨论了肺动脉内膜切除术的技术方面以及支持其作用的数据，以及对于那些被认为不适合手术的患者的肺动脉高压特定药物治疗的有限数据。
