Gerges Mario, Yacoub Magdi
Department of Internal Medicine II, Division of Cardiology, General Hospital Vienna, Medical University of Vienna, Vienna, Austria.
Aswan Heart Center, Aswan Governate, Egypt.
Glob Cardiol Sci Pract. 2020 Apr 30;2020(1):e202011. doi: 10.21542/gcsp.2020.11.
Chronic thromboembolic pulmonary hypertension (CTEPH) is one of the leading causes of severe pulmonary hypertension (PH). The disease is still underdiagnosed, and the true prevalence is unknown. CTEPH is characterized by intraluminal non-resolving thrombus organization and fibrous stenosis, or complete obliteration of pulmonary arteries, promoted by progressive remodeling of the pulmonary vasculature. One consequence of this is an increase in pulmonary vascular resistance and pressure, resulting in PH and progressive right heart failure, leading to death if left untreated. Endovascular disobliteration by pulmonary endarterectomy (PEA) is the preferred treatment for CTEPH patients. PEA surgery is the only technique that can potentially cure CTEPH disease, especially in patients with fresh or organized thrombi of the proximal branches of pulmonary arteries. However, not all patients are eligible for PEA surgery. Recent research has provided evidence suggesting balloon pulmonary angioplasty (BPA) and targeted medical therapy as additional promising available treatments options for inoperable CTEPH and recurrent/persistent PH after PEA surgery. Studies on BPA have shown it to improve pulmonary hemodynamics, symptoms, exercise capacity and RV function in inoperable CTEPH. Subsequently, BPA has developed into an essential component of the modern era of CTEPH treatment. Large randomized controlled trials have demonstrated varying significant improvements with targeted medical therapy in technically inoperable CTEPH patients. Thus, treatment of CTEPH requires a comprehensive multidisciplinary assessment, including an experienced PEA surgeon, PH specialist, BPA interventionist and CTEPH-trained radiologist at expert centers. In this comprehensive review, we address the latest developments in the fast-evolving field of CTEPH. These include advancements in imaging modalities and developments in operative and interventional techniques, which have widened the range of patients who may benefit from these procedures. The efficacy and safety of targeted medical therapies in CTEPH patients are also discussed. As the treatment options for CTEPH improve, hybrid management involving multiple treatments in the same patient may become a viable option in the near future.
慢性血栓栓塞性肺动脉高压（CTEPH）是重度肺动脉高压（PH）的主要病因之一。该疾病仍未得到充分诊断，其真实患病率尚不清楚。CTEPH的特征是管腔内血栓不溶解、机化以及纤维性狭窄，或肺动脉完全闭塞，这是由肺血管系统的进行性重塑所导致的。由此产生的一个后果是肺血管阻力和压力增加，导致肺动脉高压和进行性右心衰竭，如果不进行治疗会导致死亡。肺动脉内膜剥脱术（PEA）进行血管腔内再通是CTEPH患者的首选治疗方法。PEA手术是唯一有可能治愈CTEPH疾病的技术，特别是对于肺动脉近端分支有新鲜或机化血栓的患者。然而，并非所有患者都适合进行PEA手术。最近的研究提供了证据，表明球囊肺动脉成形术（BPA）和靶向药物治疗对于无法进行手术的CTEPH以及PEA手术后复发/持续的肺动脉高压是另外有前景的可用治疗选择。关于BPA的研究表明，它可改善无法进行手术的CTEPH患者的肺血流动力学、症状、运动能力和右心室功能。随后，BPA已发展成为CTEPH现代治疗时代的一个重要组成部分。大型随机对照试验表明，靶向药物治疗在技术上无法进行手术的CTEPH患者中取得了不同程度的显著改善。因此，CTEPH的治疗需要全面的多学科评估，包括在专家中心有经验的PEA外科医生、肺动脉高压专家、BPA介入医生和经过CTEPH培训的放射科医生。在这篇综述中，我们阐述了CTEPH快速发展领域的最新进展。这些进展包括成像模式的进步以及手术和介入技术的发展，这扩大了可能从这些手术中受益的患者范围。我们还讨论了CTEPH患者靶向药物治疗的疗效和安全性。随着CTEPH治疗选择的改善，同一患者采用多种治疗的联合管理在不久的将来可能成为一种可行的选择。
