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你可能喜欢eisenmenger’s syndrome-艾森曼格氏综合症-知来数据
&注：没有中文版本，只有英文版本
Abstract:</b
In general, most authors advocate nonintervention in Eisenmenger&#8217;s syndrome, but an awareness of potential palliative measures
to avoid destabilizing a delicately balanced physiology is needed as well. This approach has failed to alter long-term outcome,
however. Survival for patients with Eisenmenger&#8217;s syndrome has not improved substantially in the past several decades. Quality
of life is universally altered by the presence of cyanosis, exercise intolerance, and the comorbid conditions associated with
erythrocytosis. We therefore believe that the use of novel alternatives, as they become available, is warranted and that these
alternatives are likely to be best evaluated in multicenter collaborative trials.
The approach to the patient with pulmonary vascular obstructive disease (PVOD) should begin with maximization of palliative
therapy and should, as compliance and teaching are ensured, proceed to the use of therapies designed to reverse the underlying
proliferative changes in the pulmonary vasculature. Frequent checking of potential supplemental oxygen responsiveness and
use of inhaled oxygen as needed to maximize systemic arterial saturation should be considered, although evidence of the value
of home oxygen use is currently lacking. We favor systemic anticoagulation to a target international normalized ratio of 2.0
to 2.5. There are currently no published data supporting this practice in patients with PVOD, but we believe that as in patients
with primary pulmonary hypertension, benefit is likely to outweigh risk. In the setting of a meticulous outpatient anticoagulation
service, we have witnessed acceptably low bleeding event rates. A controlled clinical trial is warranted.
Selective pulmonary vasodilators and antiproliferative agents hold significant promise in altering the natural history of
PVOD associated with intracardiac shunting. The risk of paradoxic embolism and the theoretical worsening of rightto-left shunting
compound the already high risk of systemically ad neither, to date, has been limiting in our patients.
Studies of infused or newer subcutaneous and inhaled formulations are under way, and preliminary experience suggests real
benefit&#8212;improved hemodynamics, improved exercise tolerance, and increased systemic arterial saturation&#8212;in this group of patients.
Lung transplantation still trades a disease for another set of problems associated with long-term immunosuppression and chronic
graft rejection in patients with previous sternotomy and thoracotomy and with a high acute surgical risk. Population studies
of mortality and morbidity in patients with PVOD associated with congenital heart disease who receive transplants do not seem
to suggest significant improvement with this therapy.
In the future, the management of Eisenmenger&#8217;s syndrome will probably include a multipharmacologic approach that targets several
factors in the inflammatory cascade leading to vascular proliferation, perhaps offered in concert with novel surgical or transcatheter
strategies designed to limit intracardiac shunting and, if desired, provide complete repair of intracardiac defects.
翻译后摘要:</b
- 一般情况下，多数作者主张不干预艾森曼格氏综合症，但潜在的纾缓措施，以避免让一个微妙的平衡生理的认识同样也需要。这种方法并没有改变长期的结果，但是。艾森曼格氏综合症患者的生存没有显着改善，在过去的几十年。普遍的存在，紫绀，运动耐受力，并与红细胞增多症并存的条件改变的生活质量。因此，我们认为，使用新型的替代品，因为它们变得可用，是必要的，这些替代品可能是最好的多中心合作试验评价。 - 该方法对病人的肺血管阻塞性疾病（PVOD）应以最大化的姑息性治疗，并应确保合规性和教学，继续使用的疗法，旨在扭转的基础肺血管增殖性改变。应考虑潜在补充氧气吸入氧气，需要最大限度地提高全身动脉饱和的响应速度和使用的频繁检查，虽然目前尚缺乏证据的家庭使用氧气的价值。我们赞成全身抗凝目标国际标准化比值为2.0?2.5。目前没有公布的数据支持这种做法与PVOD患者，但我们相信，在原发性肺动脉高压患者，好处是可能会大于风险。在细致的门诊抗凝服务的设置中，我们目睹了出血事件的发生率在可接受的低水平。一项临床对照试验是必要的。 - 选择性肺血管扩张剂和抗增殖药物的重大承诺改变PVOD的自然史与心内分流。既不paradoxic栓塞的风险和理论恶化的rightto左分流化合物全身给予治疗已经很高的风险;，迄今为止，已在我们的患者的限制。注入或新的皮下和吸入制剂的研究正在进行中，初步的经验表明，真正的好处，改善血流动力学，改善运动能力，增加全身动脉饱和度在这组患者。 - 肺移植还是换另一套相关的问题与以前的胸骨开胸手术风险较高的急性患者长期应用免疫抑制剂，慢性移植物排斥反应的一种疾病。人口研究与先天性心脏疾病，不接受器官移植似乎表明这种疗法的显着改善，与PVOD患者的死亡率和发病率。 - 在未来，艾森曼格氏综合症的管理可能会包括一个multipharmacologic的方法，针对几个因素在血管增生导致的炎症级联反应，也许在演唱会提供新的手术或经导管战略旨在限制心内分流，如果需要的话，提供完整的心内缺损修复。
Author:</b
Jacques I. BMichael J. L;zberg
Source:</b
Current Treatment Options in Cardiovascular Medicine
Volume-OnPage:</b
Volume 1, Number 4&&355-362
[1] V. Phupong, Pornthip Ultchaswadi, Chuenkamon Charakorn, Kongkwan Prammanee, Suthisinee PSo
[2] P. J. Cole*, M. H. Cross And M. Dresner
[3] Jonathan I. Osorio, John M. Watkins, Charlie SJoseph M. Jenrette
[4] Shigeki Sakuraba, Shuya Kiyama, Ryoichi Ochiai, Shinichi Yamamoto, Tatsuya Yamada, Saori H
[5] Anne L Aspler Carly Bolshin Suzanne D Vernon Gordon Broderick
[6] Hideo Wada
[7] Janice ZNancy Esterly
[8] Elena Katzap, Maria-Louise Barilla-LGalina Marder
[9] Mark J. Hilsenroth, John Menaker, Eric J. Peters And Aaron L. Pincus
[10] Raymond Vanholder, R. De Smet, P. Vogeleere, C. HSeverin Ringoir
[1] 7,553,829
[3] 6,410,339
[5] 8,389,468
[6] 6,703,198
[7] 5,006,517
[8] 6,095,994
[10] 6,107,025
&&|&&外文文献&&|&&
QQ:5032928艾森曼格氏综合症
患者性别：男
患者年龄：17详细病情及咨询目的：我是一个先天性心脏病患者，我现在已经17岁了，9岁的时候曾去过北京的阜外医院，当时检查为房间隔缺损，伴有肺动脉高压，无法手术。时至今日，我感觉自己好没用，边最基本的走路都会觉得很累。这么多年了，倒没有吃过什么药，也没有过什么并发症，不做过力运动，与正常人一样，没有人会看出我有这种病。我现在身高173cm,体重50公斤。因为身体情况，已辍学4年，我个人感觉心理上与身体上已承受巨大痛苦！！！本次发病及持续的时间：没有发病现象，只是出力过重时间过长，会很累目前一般情况：现在身体基本正常，血压、脉搏、精神、体力、食欲、睡眠及大小便都很正常病史：无以往的诊断和治疗经过及效果：以前诊断为房间隔缺损，伴有肺动脉高压。没有治疗过辅助检查：无
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