Abstract：
Chronic thromboembolic pulmonary hypertension (CTEPH) is part of group 4 of the pulmonary hypertension (PH) classification and generally affects more than a third of patients referred to PH centers. It is a three-compartment disease involving proximal (lobar-to-segmental) and distal (subsegmental) pulmonary arteries that are obstructed by persistent fibrothrombotic material, and precapillary pulmonary arteries that can be affected as in pulmonary arterial hypertension. It is a rare complication of pulmonary embolism (PE), with an incidence of around 3% in PE survivors. The observed incidence of CTEPH in the general population is around six cases per million but could be three times higher than this, as estimated from PE incidence. However, a previous venous thromboembolic episode is not always documented. With advances in multimodality imaging and therapeutic management, survival for CTEPH has improved for both operable and inoperable patients. Advanced imaging with pulmonary angiography helps distinguish proximal from distal obstructive disease. However, right heart catheterization is of utmost importance to establish the diagnosis and hemodynamic severity of PH. The therapeutic strategy relies on a stepwise approach, starting with an operability assessment. Pulmonary endarterectomy (PEA), also known as pulmonary thromboendarterectomy, is the first-line treatment for operable patients. Growing experience and advances in surgical technique have enabled expansion of the distal limits of PEA and significant improvements in perioperative and mid- to long-term mortality. In patients who are inoperable or who have persistent/recurrent PH after PEA, medical therapy and/or balloon pulmonary angioplasty (BPA) are effective treatment options with favorable outcomes that are increasingly used. All treatment decisions should be made with a multidisciplinary team that includes a PEA surgeon, a BPA expert, and a chest radiologist.
摘要：
慢性血栓栓塞性肺动脉高压（CTEPH）是肺动脉高压（PH）分类第4组的一部分，通常影响到PH中心的三分之一以上的患者。这是一种三室疾病，涉及近端（肺叶到节段）和远端（亚节段）肺动脉，被持续性纤维化血栓形成物质阻塞，和肺动脉高压时可能受到影响的毛细血管前肺动脉。它是肺栓塞（PE）的罕见并发症，PE幸存者的发病率约为3％。在普通人群中观察到的CTEPH发病率约为每百万六例，但可能比这高出三倍，根据PE发病率估计。然而,以前的静脉血栓栓塞发作并不总是有记录.随着多模态成像和治疗管理的进步，对于可手术和不可手术的患者，CTEPH的生存率都有所提高。肺动脉造影的高级成像有助于区分近端和远端阻塞性疾病。然而,右心导管检查对于确定PH的诊断和血流动力学严重程度至关重要。治疗策略依赖于逐步的方法，从可操作性评估开始。肺内膜切除术（PEA），也被称为肺血栓内膜切除术，是可手术患者的一线治疗。不断增长的经验和手术技术的进步使PEA的远端界限得以扩大，并显着改善了围手术期和中长期死亡率。在PEA后不能手术或有持续性/复发性PH的患者中，药物治疗和/或球囊肺血管成形术(BPA)是具有良好预后的有效治疗选择,且应用日益广泛.所有的治疗决定都应该由一个多学科的团队，包括一个PEA外科医生，BPA专家,还有胸部放射科医生.
