Chang Sung-A, Yang Jeong Hoon, Jung Dong Seop, Kim Nick H
Division of Cardiology, Department of Internal Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Division of Thoracic and Cardiovascular Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Korean Circ J. 2025 May;55(5):365-381. doi: 10.4070/kcj.2024.0423. Epub 2025 Feb 10.
Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive form of pulmonary hypertension characterized by unresolved thromboembolic occlusion of pulmonary arteries, leading to increased pulmonary arterial pressure and right heart failure. This review examines recent advances in the pathophysiology, diagnosis, and management of CTEPH, focusing on expanding disease concepts and evolving therapeutic approaches. The incidence of CTEPH has been revised upward with improved diagnostic techniques revealing a higher prevalence than previously recognized. Advances in surgical and interventional therapies, particularly pulmonary endarterectomy and balloon pulmonary angioplasty, have significantly improved outcomes. Emerging medical therapies, including pulmonary vasodilators like riociguat, have offered new hope for inoperable cases. The understanding of CTEPH has broadened, leading to better diagnostic strategies and more comprehensive treatment options that significantly enhance patient outcomes. Multidisciplinary team approaches are crucial in managing the disease effectively.
慢性血栓栓塞性肺动脉高压(CTEPH)是一种进行性肺动脉高压形式,其特征是肺动脉血栓栓塞性阻塞未得到解决,导致肺动脉压力升高和右心衰竭。本综述探讨了CTEPH在病理生理学、诊断和管理方面的最新进展,重点关注不断扩展的疾病概念和不断发展的治疗方法。随着诊断技术的改进,CTEPH的发病率已向上修正,显示出比以前认识到的更高的患病率。手术和介入治疗的进展,特别是肺动脉内膜剥脱术和球囊肺动脉成形术,显著改善了治疗效果。包括利奥西呱等肺血管扩张剂在内的新兴药物治疗为无法手术的病例带来了新希望。对CTEPH的认识已经拓宽,从而产生了更好的诊断策略和更全面的治疗选择,显著提高了患者的治疗效果。多学科团队方法对于有效管理该疾病至关重要。