Division of Cardiovascular and Thoracic Surgery, University of California, San Diego, La Jolla, CA, USA
Division of Pulmonary Circulation, Dept of Advanced Medicine for Pulmonary Hypertension, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.
Eur Respir Rev. 2017 Dec 20;26(146). doi: 10.1183/16000617.0105-2017. Print 2017 Dec 31.
For patients with chronic thromboembolic pulmonary hypertension (CTEPH), the current standard of care involves surgical removal of fibro-thrombotic obstructions by pulmonary endarterectomy. While this approach has excellent outcomes, significant proportions of patients are not eligible for surgery or suffer from persistent/recurrent pulmonary hypertension after the procedure. The availability of balloon pulmonary angioplasty and the approval of the first medical therapy for use in CTEPH have significantly improved the outlook for patients ineligible for pulmonary endarterectomy. In this comprehensive review, we discuss the latest developments in the rapidly evolving field of CTEPH. These include improvements in imaging modalities and advances in surgical and interventional techniques, which have broadened the range of patients who may benefit from such procedures. The efficacy and safety of targeted medical therapies in CTEPH patients are also discussed, particularly the encouraging data from the recent MERIT-1 trial, which demonstrated the beneficial impact of using macitentan to treat patients with inoperable CTEPH, including those on background therapy. As the treatment options for CTEPH improve, hybrid management involving more than one intervention in the same patient may become a viable option in the near future.
对于慢性血栓栓塞性肺动脉高压(CTEPH)患者,目前的标准治疗方法包括通过肺动脉内膜切除术切除纤维血栓阻塞物。虽然这种方法的效果很好,但相当一部分患者不适合手术,或者在手术后仍然存在持续性/复发性肺动脉高压。球囊肺动脉成形术的应用和首个 CTEPH 治疗药物的批准,显著改善了不适合肺动脉内膜切除术的患者的预后。在这篇全面的综述中,我们讨论了 CTEPH 这一快速发展领域的最新进展。这些进展包括成像方式的改进以及手术和介入技术的进步,这拓宽了可能从这些手术中获益的患者范围。我们还讨论了靶向药物治疗在 CTEPH 患者中的疗效和安全性,特别是最近 MERIT-1 试验的令人鼓舞的数据,该数据表明使用马西替坦治疗不能手术的 CTEPH 患者(包括那些正在接受背景治疗的患者)具有有益的影响。随着 CTEPH 的治疗选择的改善,在同一患者中进行多种干预的混合管理可能在不久的将来成为一种可行的选择。