肺动脉高压诊治现状

Current status of treatment for pulmonary hypertension

关键词:  肺动脉高压  靶向药物  慢性血栓栓塞性肺动脉高压  肺血管疾病

Key words:  pulmonary hypertension  target drugs  chronic thromboembolic pulmonary hypertension  pulmonary vascular diseases

摘要:

肺动脉高压（pulmonary hypertension, PH）是一类恶性肺血管疾病，其主要病理生理学特征为肺血管持续收缩及重构，导致肺血管阻力进行性增高，同时合并不同程度右心功能衰竭。超声心动图是疑诊PH首选无创筛查方法，右心导管检查是确诊PH的“金标准”。近10年，三大针对PH靶向治疗经典途径的药物： 内皮素受体拮抗剂、磷酸二酯酶5抑制剂、前列环素类药物陆续上市，联合治疗策略也被临床医师认可，PH患者预后明显改善。此外，第四大类慢性血栓栓塞性PH患者通过肺动脉内膜剥脱术或经皮肺动脉球囊扩张术，部分患者甚至获得“治愈”。随着PH研究机制的进展，不同作用途径的靶向药物陆续上市，越来越多不同专业的医生投入到肺血管疾病的诊治及研究工作中，推动PH领域的快速发展。

Abstract:

Pulmonary hypertension(PH), a progressive and fatal disease caused by a variety of disorders, is characterized by an increase in pulmonary vascular resistance, abnormal sustained pulmonary vasoconstriction and progressive structural remodeling of the pulmonary arteries, ultimately leading to right ventricular failure. Echocardiography is the first-line non-invasive diagnostic method for suspected PH, while right cardiac catheterization is the “gold standard” for the diagnosis of PH. Three classical PAH targeted therapy drugs including endothelin receptor antagonists, phosphodiesterase 5 inhibitors and prostacyclinsare are available in the market. Combination therapy strategies are increasingly recognized by physicians and the prognosis has gradually improved. Pulmonary endarterectomy and percutaneous balloon pulmonary angioplasty may significantly improve the survival of patients with chronic thromboembolic pulmonary hypertension. More and more specialists devote themselves to the fields of pulmonary vascular diseases and promote the treatment of PH in China.