Bresser Paul, Pepke-Zaba Joanna, Jaïs Xavier, Humbert Marc, Hoeper Marius M
Department of Pulmonology, Academic Medical Center, P.O. Box 22660, Amsterdam 1100 DD, The Netherlands.
Proc Am Thorac Soc. 2006 Sep;3(7):594-600. doi: 10.1513/pats.200605-115LR.
Pulmonary endarterectomy (PEA) is recommended as the treatment of choice for eligible patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, only a proportion of patients fulfill the criteria for surgical intervention. In addition, operated patients with CTEPH may experience a gradual hemodynamic and symptomatic decline related to a secondary hypertensive arteriopathy in the small precapillary pulmonary vessels. It has also been questioned what can be done to reduce risks from PEA surgery to improve outcome in "high risk" patients with CTEPH with substantial impairment of pulmonary hemodynamics before surgery. Such patients may benefit from preoperative reduction of pulmonary vascular resistance by means of medical therapy. Conventional medical treatments, such as anticoagulation, diuretics, digitalis, and chronic oxygen therapy, show low efficacy in the treatment of CTEPH as they do not affect underlying disease processes. Over the last decade, several novel therapies have been developed for pulmonary arterial hypertension (PAH), including prostacyclin analogs (epoprostenol, beraprost, iloprost), endothelin receptor antagonists (bosentan, sitaxsentan, ambrisentan), and phosphodiesterase-5 inhibitors (sildenafil). Evidence of efficacy in PAH, coupled with studies showing histopathologic similarities between CTEPH and PAH, provides a rationale to extend the use of some of these medications to the treatment of CTEPH. However, direct evidence from clinical trials in CTEPH is limited to date. This article reviews evidence supporting, and issues surrounding, the possible use of novel PAH medications in CTEPH.
对于符合条件的慢性血栓栓塞性肺动脉高压（CTEPH）患者，推荐行肺动脉内膜剥脱术（PEA）作为首选治疗方法。然而，只有一部分患者符合手术干预标准。此外，接受手术的CTEPH患者可能会因小的肺毛细血管前血管继发性高血压性动脉病而出现血流动力学和症状逐渐恶化的情况。对于术前肺血流动力学严重受损的“高危”CTEPH患者，如何降低PEA手术风险以改善预后也一直受到质疑。这类患者可能会从术前通过药物治疗降低肺血管阻力中获益。传统的药物治疗，如抗凝、利尿剂、洋地黄和长期氧疗，在CTEPH治疗中疗效较低，因为它们不影响潜在的疾病进程。在过去十年中，已开发出几种用于治疗肺动脉高压（PAH）的新型疗法，包括前列环素类似物（依前列醇、贝拉前列素、伊洛前列素）、内皮素受体拮抗剂（波生坦、西他生坦、安立生坦）和磷酸二酯酶-5抑制剂（西地那非）。PAH的疗效证据，以及显示CTEPH与PAH在组织病理学上相似性的研究，为将其中一些药物扩展用于CTEPH治疗提供了理论依据。然而，迄今为止，CTEPH临床试验的直接证据仍然有限。本文综述了支持在CTEPH中使用新型PAH药物的证据以及相关问题。
