Education＆ScienceEducational Article
Effect of off-label targeted drugs on long-term survival in chronic thromboembolic pulmonary hypertension: Insights from a national multicentre prospective registry
July 2024
Authors: Wanying Xia, Yuling Qian, Yangyi Lin, Ruilin Quan, Yuanhua Yang, Zhenwen Yang, Hongyan Tian, Shengqing Li, Jieyan Shen, Yingqun Ji, Qing Gu, Huijun Han, Changming Xiong, Jianguo He
Comment by Mark Lavercombe: Pulmonary endarterectomy (PEA) is the preferred treatment for patients with chronic thromboembolic pulmonary hypertension, however there are patients for whom surgery is contraindicated or who have ongoing symptomatic pulmonary hypertension despite surgery. Therapeutic options include balloon pulmonary angioplasty and medical therapies including riociguat and treprostinil, although these agents were not available in China for the majority of the study period. This paper examines the use of phosphodiesterase 5 inhibitors (PDE5i) and endothelin receptor antagonists (ERA) in this group of patients using data from a national registry in China. Patients treated with PDE5i and/or ERA within three months of enrolment had a significantly improved survival compared with the control group.