Kondo Takahisa, Okumura Naoki, Adachi Shiro, Murohara Toyoaki
Department of Advanced Medicine in Cardiopulmonary Disease, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Department of Cardiology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Nagoya J Med Sci. 2019 Feb;81(1):19-30. doi: 10.18999/nagjms.81.1.19.
Pulmonary hypertension (PH) is a hemodynamic state that is characterized by a resting mean pulmonary artery pressure ≧ 25 mmHg. The common forms of PH are pulmonary arterial hypertension (PAH), chronic thromboembolic pulmonary hypertension (CTEPH), PH caused by left-heart disease, and PH due to lung disease. Previously regarded as untreatable, the treatment of PAH has dramatically advanced since the introduction of the drug epoprostenol in 1999, with three-year survival rates improving from 30%-40% to over 85%. Drugs available for the specific treatment of PAH include endothelin-receptor antagonists, phosphodiesterase type 5 inhibitors, soluble guanylate cyclase stimulators, prostacyclin analogs, and prostacyclin-receptor agonists. In the past decade, management and treatment of CTEPH have also improved. While pulmonary endarterectomy used to be the only option for the treatment of CTEPH, newer treatments include a soluble guanylate cyclase stimulator, which has proven to be an efficacious targeted therapy. Other cases benefit from balloon pulmonary angioplasty.
肺动脉高压(PH)是一种血流动力学状态,其特征为静息平均肺动脉压≧25 mmHg。常见的PH类型包括动脉性肺动脉高压(PAH)、慢性血栓栓塞性肺动脉高压(CTEPH)、左心疾病所致的PH以及肺部疾病所致的PH。PAH曾被认为无法治疗,但自1999年引入依前列醇药物以来,PAH的治疗取得了显著进展,三年生存率从30%-40%提高到了85%以上。可用于PAH特异性治疗的药物包括内皮素受体拮抗剂、5型磷酸二酯酶抑制剂、可溶性鸟苷酸环化酶刺激剂、前列环素类似物和前列环素受体激动剂。在过去十年中,CTEPH的管理和治疗也有所改善。虽然肺动脉内膜剥脱术曾经是治疗CTEPH的唯一选择,但新的治疗方法包括一种可溶性鸟苷酸环化酶刺激剂,已被证明是一种有效的靶向治疗方法。其他病例则受益于球囊肺动脉成形术。