摘要
特发性肺动脉高压（IPAH）是指原因不明的肺血管阻力增加引起持续性肺动脉压力升高，其病理改变为肺血管的收缩和重塑、原位血栓形成和血管内皮损伤。由于缺乏有效的治疗措施，病死率极高，预后差，明确诊断后平均存活时间为2～3年。近年来随着对IPAH的分子生物学水平研究的深入，一些新的治疗方法如前列环素及其衍生物、内皮素受体拮抗荆等不断应用于临床，使IPAH的生存率明显提高，生活质量明显改善。而一些被证明对IPAH具有潜在治疗意义的药物如肾上腺髓质素、西地那非、他汀类药物等仍在临床研究中，在不久的将来可能成为治疗IPAH的一线用药。
Idiopathic pulmonary artery hypertension （IPAH） means persistent hypertension in pulmonary artery with an increase of pulmonary resistance caused by unidentified factors. Constriction and remodelling of pulmonary artery,in situ thrombosis and endothelium in- jury are the major pathologic changes. Due to lack of effective therapy, mortality of IPAH is high with survival periods of only 2 to 3 years after it has been diagnosed. However,some novel medications have been developed to treat IPAH and improve its survival rate and the quality of life of patients, due to progress in research into molecular biologic mechanisms of these diseases, such as prostacyclin and its derivation, the antagonist of endothelin receptor. Other drugs,including adrenal medulla hormones, sildenafil and statins, are the subjects of clinical research, and may soon become first-line therapies.
出处
《心血管病学进展》
CAS
2007年第2期296-299,共4页
Advances in Cardiovascular Diseases
关键词
特发性肺动脉高压
药物治疗
进展
idiopathic pulmonary artery hypertension
medication
progress
