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Jingdong book

肺动脉高压诊断与治疗2009

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肺动脉高压诊断与治疗2009

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多数心脏专科医师和呼吸专科医师见到的与左心充盈压升高相关的肺动脉高压要比肺动脉高压常见得多。任何导致左心充盈压升高的疾患,包括收缩功能不全、舒张功能不全和心脏瓣膜病,均可以导致肺动脉压升高。因此,治疗应当针对基础性左心疾病。偶尔,在对基础疾病进行最佳治疗后,如果肺毛细血管楔压正常或轻度升高,跨肺压差和肺血管阻力明显升高,患者的症状提示肺动脉高压一特异治疗可以有临床净获益,则可以考虑进行肺动脉高压的特异治疗。可以将这一组患者称为“不对称”肺动脉高压患者(高于根据其左心压力升高或肺疾病估测的压力)。专家们反对对非肺动脉高压性肺动脉高压进行治疗,除非临床研究资料显示这类患者可以从治疗中获益。这类患者进行肺动脉高压特异治疗的潜在不良反应包括液体潴留、肺水肿和通气灌注失衡。
Catalogue

1 病理与病因
1.1 组织学
1.2 右心室
1.3 肺动脉高压时的分子异常
1.4 肺动脉高压的遗传学
1.5 肺动脉高压时血液和内皮异常
1.6 前列环素和血栓素A2
1.7 内皮素
1.8 氧化氮
1.9 其他血管活性物质
1.10 炎症
1.11 肺动脉高压时的肺动脉平滑肌细胞

2 肺动脉高压的分类和流行病学(WHO1组)
2.1 特发性肺动脉高压
2.2 家族性肺动脉高压
2.3 与先天性心脏病相关的肺动脉高压
2.4 与结缔组织病相关的肺动脉高压
2.5 与人类免疫缺陷病毒感染相关的肺动脉高压
2.6 与门静脉高压相关的肺动脉高压
2.7 与药物和毒素相关的肺动脉高压
2.8 与血红蛋白病相关的肺动脉高压
2.9 与其他病因相关的肺动脉高压
2.10 与肺静脉阻塞或毛细血管相关的肺动脉高压

3 自然史和生存
3.1 肺动脉高压药物治疗:对存活的影响
3.2 影响存活和帮助评估预后的因素
3.3 功能分级
3.4 运动耐量
3.5 血流动力学
3.6 超声心动图检查
3.7 磁共振成像
3.8 生物标记物
3.9 建议小结

4 筛查与诊断性和血流动力学评估
4.1 肺动脉高压定义
4.2 诊断策略
4.3 超声心动图检查
4.4 运动超声心动图检查
4.5 肺动脉压增高诊断性评估中的新影像技术
4.6 有创血流动力学评估
4.7 右心导管术
4.8 最佳有创评估的组成
4.9 心导管术的安全性
4.10 肺动脉压的自发变异
4.11 肺动脉血流动力学的动态测量
4.12 急性扩血管试验
4.13 急性扩血管试验的药物
4.14 肺动脉高压急性扩血管试验有反应者的定义
4.15 肺动脉高压患者扩血管试验
4.16 小结

5 循证学治疗流程
5.1 一般措施
5.2 背景治疗
5.3 钙拮抗剂
5.4 前列腺素
5.5 依前列醇
5.6 曲罗尼尔
5.7 伊洛前列素
5.8 内皮素受体拮抗剂
5.9 波生坦
5.10 司他生坦
5.11 安贝生坦
5.12 磷酸二酯酶抑制剂
5.13 西地那非
5.14 他达拉非
5.15 联合治疗
5.16 肺动脉高压临床试验的局限性
5.17 费用问题
5.18 有创治疗
5.19 房间隔切开术
5.20 肺和心肺联合移植
5.21 肺动脉内膜血栓切除术
5.22 右心室辅助装置
5.23 治疗流程

6 实时再次评估:如何随访治疗的患者
6.1 专科医院护士在管理肺动脉高压患者中的作用

7 非肺动脉性肺动脉高压人群
7.1 WHO2组:肺静脉高压
7.2 WHO3组:低氧血症相关的肺动脉高压
7.3 WHO4组:血栓栓塞性肺动脉高压
7.4 建议小结

8 先天性心脏病相关的肺动脉高压

9 儿童肺动脉高压
9.1 新生儿持续性肺动脉高压
9.2 儿童肺动脉高压
10 肺动脉高压治疗中心
参考文献
Book Abstract

5循证学治疗流程
过去10年,肺动脉高压的治疗取得了较大进展,部分归功于对疾病基础病理生理学认知的进展以及拥有了靶向干预病理生理过程的药物。ACCP、欧洲心脏学会和参加2003年在意大利威尼斯召开的第三届世界肺动脉高压专题研讨会的一席专家们共同制定了肺动脉高压治疗原则。值得注意的是,这些法则所依据的临床资料主要来自于特发性肺动脉高压患者、结缔组织疾病和食欲抑制剂相关的肺动脉高压患者的研究。仅有少量试验入选了先天性心脏病和人类免疫缺陷病毒相关性肺动脉高压。因此,这些临床试验结果不能推及其他类型的肺动脉高压,包括左心疾病引起、低氧血症肺疾病或其他WH0分型的肺动脉高压。
肺动脉高压患者的治疗目的多种多样。改善患者最常见的症状一呼吸困难是最重要的。提高患者肺功能耐量也是治疗目的之一,可以通过6min行走试验或心肺运动试验对运动耐力进行客观评价。由于肺动脉高压时可存在严重血流动力学紊乱,因而降低肺动脉压和恢复心输出量至正常水平也是重要治疗目标。另一个重要目标是逆转或至少预防疾病的进展,即减少更多治疗、住院和肺移植的必要。最后,应力争改善存活。这一点尽管重要,但极少作为肺动脉高压治疗临床试验的终点指标,原因是患者数量少并且试验持续时间有限。
Introduction

《肺动脉高压的诊断与治疗一2009》是依据《ACCF/AHA肺动脉高压2009年专家共识文件》编写而成,后者分别发表在《美国心脏病学会杂志》(J Am Coll Cardiol,2009,53:1573~1619)和《循环》(Circulation,2009,119:2250~2294)上,相关网站(www.acc.org和www.my.american-heart.org)也已登载。
肺动脉高压是一种由于肺动脉循环血流受限导致肺血管阻力增加并且最终导致右心衰竭的综合征,是一个涉及多个学科的复杂疾患。临床医师对于肺动脉高压往往认识不足,对于其治疗常常更是束手无策。
《肺动脉高压的诊断与治疗一20099从肺动脉高压的病理与病因、肺动脉高压的分类和流行病学、自然史和生存、筛查与诊断性和血流动力学评估、循证学治疗流程、实时再次评估:如何随访治疗的患者、非肺动脉性肺动脉高压人群、先天性心脏病相关的肺动脉高压、儿童肺动脉高压和肺动脉高压治疗中心总共10个方面对肺动脉高压的诊断与治疗进行了全面阐述,反映了全世界目前在该领域的研究现状。
我们在繁重的临床工作之余编译本书,期望能为从事相关领域工作的临床医师提供帮助。由于水平所限,错误难免,敬请指正。必要时,烦请读者参阅原文。

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