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肺动脉高压是一类表现为肺动脉压力和肺血管阻力进行性增高的疾病,通常导致右心功能衰竭并最终使患者死亡。肺动脉高压的临床症状没有特征性,对出现气短、晕厥、胸痛等症状而不能用常见心、肺疾病解释时,应想到肺动脉高压的可能,尽早进行超声心动图检查,缩短患者的确诊时间。准确评价心功能分级,完成6 m in步行距离试验的评价。确立右心导管检查诊断肺动脉高压,尤其是特发性肺动脉高压的价值。近年来,随着对肺动脉高压肺血管重构的细胞和分子基础的研究,出现了一系列新的治疗方法。但是传统疗法是治疗肺动脉高压的基石,特殊治疗应建立在规范传统治疗上,新的治疗药物(前列环素类似物、内皮素受体拮抗剂等)的引入,肺动脉高压的存活率有了很大的提高。口服波生坦和吸入性前列腺素作为特发性肺动脉高压的一线药。肺动脉高压发病基因的研究已取得进展,目前至少发现了一种基因与肺动脉高压的发病有关,相信在不久的将来,肺动脉高压的基因治疗将成为现实。
Pulmonary hypertension is a type of disease characterized by a progressive increase in pulmonary artery pressure and pulmonary vascular resistance, often leading to right heart failure and ultimately to patient death. The clinical symptoms of pulmonary hypertension are not characteristic, on the shortness of breath, syncope, chest pain and other symptoms can not be common heart, lung disease interpretation, should think of the possibility of pulmonary hypertension, echocardiography as soon as possible to shorten the patient’s diagnosis time. Accurate evaluation of cardiac function classification, to complete the 6 m in walking distance test evaluation. The establishment of right heart catheterization diagnosis of pulmonary hypertension, especially idiopathic pulmonary hypertension value. In recent years, with the research on the cellular and molecular basis of pulmonary vascular remodeling in pulmonary hypertension, a series of new treatments have emerged. However, the traditional therapy is the cornerstone of the treatment of pulmonary hypertension, special treatment should be based on the traditional treatment of traditional, new therapeutic drugs (prostacyclin analogues, endothelin receptor antagonists, etc.) the introduction of pulmonary hypertension have a very good survival rate Great improvement. Oral Bosentan and inhaled prostaglandin as first-line drugs for idiopathic pulmonary hypertension. Progress has been made in the study of the gene that causes pulmonary hypertension. At present, at least one gene has been found to be associated with the pathogenesis of pulmonary hypertension. It is believed that the gene therapy of pulmonary hypertension will become a reality in the near future.