Fernandes Timothy M, Poch David S, Auger William R
University of California-San Diego, School of Medicine, La Jolla, California.
Methodist Debakey Cardiovasc J. 2016 Oct-Dec;12(4):205-212. doi: 10.14797/mdcj-12-4-205.
Chronic thromboembolic pulmonary hypertension (CTEPH) is a potentially curable disease when treated with pulmonary thromboendarterectomy (PTE). However, even at experienced surgical centers, nearly one-third of patients with CTEPH will be deemed inoperable for reasons including distal disease, comorbidities, or out-of-proportion pulmonary hypertension. It is in these patients with inoperable CTEPH that pulmonary hypertension (PH)-targeted medical therapy and balloon pulmonary angioplasty have potential therapeutic value. Previous unblinded cohort trials have assessed PH-targeted medical therapy in various subpopulations of CTEPH patients using epoprostenol, treprostinil, sildenafil, bosentan, and iloprost, each demonstrating measurable pulmonary hemodynamic effects. However, riociguat, a soluble guanylate cyclase stimulator, is the first FDA-approved therapy for inoperable CTEPH to demonstrate both an improvement in functional capabilities (6-minute walk time) as well as significant gains in secondary pulmonary hemodynamic end points in a large placebo-controlled trial. Balloon pulmonary angioplasty is an interventional procedure using telescoping catheters placed in the pulmonary arteries, through which wires and balloons are used to mechanically disrupt chronic clot material and relieve pulmonary vascular obstruction. Contemporary case series from multiple centers worldwide have demonstrated pulmonary hemodynamic improvement with this approach. As a result of these advances, patients with inoperable CTEPH who had few options as recently as 5 years ago now have alternatives with emerging evidence of therapeutic efficacy.
慢性血栓栓塞性肺动脉高压（CTEPH）在接受肺动脉血栓内膜剥脱术（PTE）治疗时是一种潜在可治愈的疾病。然而，即使在经验丰富的手术中心，近三分之一的CTEPH患者会因包括远端病变、合并症或肺动脉高压不成比例等原因而被认为无法进行手术。正是在这些无法进行手术的CTEPH患者中，肺动脉高压（PH）靶向药物治疗和球囊肺动脉成形术具有潜在的治疗价值。以往的非盲队列试验使用依前列醇、曲前列尼尔、西地那非、波生坦和伊洛前列素，在CTEPH患者的不同亚组中评估了PH靶向药物治疗，每项试验都显示出可测量的肺血流动力学效应。然而，利奥西呱，一种可溶性鸟苷酸环化酶刺激剂，是美国食品药品监督管理局（FDA）批准的第一种用于无法进行手术的CTEPH的治疗药物，在一项大型安慰剂对照试验中，它既显示出功能能力（6分钟步行距离）的改善，也显示出肺血流动力学次要终点的显著改善。球囊肺动脉成形术是一种介入手术，使用放置在肺动脉中的可伸缩导管，通过这些导管，利用导线和球囊机械性地破坏慢性血栓物质并缓解肺血管阻塞。来自全球多个中心的当代病例系列研究表明，这种方法可改善肺血流动力学。由于这些进展，就在5年前还几乎没有选择的无法进行手术的CTEPH患者现在有了新的治疗选择，并且有越来越多的证据证明其治疗效果。
