Unidade de Circulação Pulmonar - Divisão de Pneumologia - Incor - Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP - Brasil.
Instituto do Câncer da Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP - Brasil.
Arq Bras Cardiol. 2021 Oct;117(4):750-764. doi: 10.36660/abc.20200702.
In the last decades, important advances have been made in the treatment of pulmonary arterial hypertension (PAH), a severe, progressive, incurable, and potentially fatal disease. For an adequate therapy, correct hemodynamic diagnosis and etiology classification are fundamental. Many etiologies - rheumatic disease, portal hypertension, congenital heart diseases, schistosomiasis - require specific measures, in addition to drug therapy for PAH. The specific therapy for PAH is based on medications that act on three pathophysiological pathways - prostacyclin, endothelin, and nitric oxide pathways. These drugs have multiple presentations (oral, intravenous, subcutaneous, and inhaled) and have changed the history of PAH. This review presents an overview of drug therapy strategies and different forms and peculiarities of PAH.
在过去的几十年中，肺动脉高压（PAH）的治疗取得了重要进展，PAH 是一种严重、进行性、无法治愈且可能致命的疾病。为了进行适当的治疗，正确的血液动力学诊断和病因分类是基础。许多病因 - 风湿性疾病、门静脉高压、先天性心脏病、血吸虫病 - 需要除了针对 PAH 的药物治疗之外，还需要采取特定的措施。PAH 的特定治疗基于作用于三种病理生理途径的药物 - 前列环素、内皮素和一氧化氮途径。这些药物有多种剂型（口服、静脉内、皮下和吸入），并改变了 PAH 的历史。本综述介绍了药物治疗策略以及不同形式和特点的 PAH。
