Wang Aqian, Su Hongling, Duan Yichao, Jiang Kaiyu, Li Yu, Deng Mingjun, Long Xiaozhou, Wang Haijun, Zhang Min, Zhang Yan, Cao Yunshan
Department of Cardiology, Gansu Provincial Hospital, Lanzhou, China.
Department of Cardiology, Xianyang Central Hospital, Xianyang, China.
JACC Asia. 2022 May 17;2(3):218-234. doi: 10.1016/j.jacasi.2021.11.016. eCollection 2022 Jun.
Pulmonary hypertension (PH) is a progressive and severe disorder in pulmonary hemodynamics. PH can be fatal if not well managed. Fibrosing mediastinitis (FM) is a rare and benign fibroproliferative disease in the mediastinum, which may lead to pulmonary vessel compression and PH. PH caused by FM (PH-FM) is a pathologic condition belonging to group 5 in the World Health Organization PH classification. PH-FM has a poor prognosis because of a lack of effective therapeutic modalities and inappropriate diagnosis. With the development of percutaneous pulmonary vascular interventional therapy, the prognosis of PH-FM has been greatly improved in recent years. This article provides a comprehensive review on the epidemiology, pathophysiologic characteristics, clinical manifestations, diagnostic approaches, and treatment modalities of PH-FM based on data from published reports and our medical center with the goal of facilitating the diagnosis and treatment of this fatal disease.
肺动脉高压（PH）是一种肺血流动力学方面的进行性严重疾病。若管理不善，PH可能会致命。纤维性纵隔炎（FM）是纵隔内一种罕见的良性纤维增生性疾病，可导致肺血管受压及PH。由FM引起的PH（PH-FM）是世界卫生组织PH分类中第5组的一种病理状态。由于缺乏有效的治疗方式及诊断不当，PH-FM预后较差。随着经皮肺血管介入治疗的发展，近年来PH-FM的预后有了很大改善。本文基于已发表报告及我们医疗中心的数据，对PH-FM的流行病学、病理生理特征、临床表现、诊断方法及治疗方式进行全面综述，目的是促进对这种致命疾病的诊断和治疗。
