ACCF/AHA 2009 关于肺动脉高压的专家建议
Title: ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension: A Report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents
Topic: Heart Failure/Transplant
Date Posted: 3/30/2009 10:00:00 AM
Author: McLaughlin VV, Archer SL, Badesch DB, et al.
Citation: J Am Coll Cardiol 2009;Mar 30:[Epub ahead of print].
Clinical Trial: No
Perspective: The following are 10 points to remember about the expert consensus document on pulmonary hypertension.
1. Pulmonary arterial hypertension (PAH) is a syndrome resulting from restricted flow through the pulmonary arterial circulation, resulting in increased pulmonary vascular resistance (PVR) and ultimately in right heart failure. Multiple pathogenic pathways are responsible for modifying smooth muscle and endothelial cells and adventitia, and the imbalance in the vasoconstrictor/vasodilator milieu.
2. The prevalence of PAH is 15 per million in the French registry. Idiopathic PAH (IPAH) is the most common type of PAH and is more common in women. Familial PAH often results from a mutation in the bone morphogenic protein receptor-2 and is inherited as an autosomal dominant disease with incomplete penetrance and genetic anticipation. PAH is also associated with congenital heart disease (CHD), connective tissue diseases, drugs and toxins, human immunodeficiency virus, portal hypertension, hemoglobinopathies, and myeloproliferative disorders. CHD occurs in 8 per 1,000 live births, and PAH will develop in 30% of those with nonrepaired CHD.
3. The 1-year mortality in PAH is about 15% on modern therapy. Predictors of a poor prognosis include: advanced functional class, poor exercise capacity, high right atrial pressure, significant right ventricular (RV) dysfunction, RV failure, low cardiac index, elevated brain natriuretic peptide, and the scleroderma spectrum of diseases.
4. Patients at sufficient risk for the development of PAH to warrant periodic screening include those with a family history of IPAH, scleroderma spectrum of diseases, and portal hypertension who are undergoing evaluation for liver transplantation. The most appropriate study to obtain in patients suspected of having PH is an echocardiogram.
5. The diagnosis of PAH requires confirmation with a complete right heart catheterization. The hemodynamic definition of PAH is a mean pulmonary artery pressure >25 mm Hg; a pulmonary capillary wedge pressure (PCW), left atrial pressure, or left ventricular end-diastolic pressure ≤15 mm Hg; and a PVR >3 Wood units. Acute vasodilator testing should be performed in experienced centers on IPAH patients to screen eligibility for long-term calcium channel blocker (CC therapy. Exceptions include those with overt right heart failure or hemodynamic instability.
6. General treatment measures include diet, exercise, appropriate vaccinations, and avoidance of pregnancy. Warfarin anticoagulation is recommended in all patients with IPAH. Diuretics are used for symptomatic management of RV volume overload. Oxygen is recommended to maintain oxygen saturation >90%. Acute responders to vasodilator testing treated with CCB should be followed closely for both the safety and the efficacy of this therapy.
7. Continuous intravenous epoprostenol improves exercise capacity, hemodynamics, and survival in IPAH and is the preferred treatment option for the most critically ill patients. Treprostinil, a prostanoid, may be delivered via either continuous intravenous or subcutaneous infusion. Iloprost is a prostanoid delivered by an adaptive aerosolized device 6 times daily.
8. The endothelin receptor antagonists (ETAs) are oral therapies that improve exercise capacity in PAH. Liver function tests must be monitored indefinitely on a monthly basis. Phosphodiesterase (PDE)-5 inhibitors also improve exercise capacity and hemodynamics in PAH. Patients with poor prognostic indexes should be initiated on parenteral therapy, while patients with class II or early III symptoms can commence therapy with either ETAs or PDE-5 inhibitors. Initial trials suggest that combination therapy may be useful. Lung transplantation is an option for selected patients who progress despite optimal medical management.
9. Due to the complex nature of the disease and its treatments, PAH patients must be closely followed by experienced physicians and nurse clinicians. In general, office visits should be more frequent for patients with advanced symptoms, right heart failure, and advanced hemodynamics and those on parenteral or combination therapy. Most experts obtain an assessment of functional class and exercise capacity, such as a 6-minute walk or graded treadmill test, with each office visit.
10. Any disorder that elevates left heart filling pressures, including systolic dysfunction, diastolic dysfunction, and valvular heart disease, can result in elevated pulmonary artery pressures. In rare instances, PAH-specific therapy may be considered if the underlying cause has been optimally treated, the PCW is normal or minimally elevated, and the transpulmonary gradient and PVR are significantly elevated. The latter is known as 揹isproportionate?PH or greater than expected on the basis of the elevated left heart pressure or lung disease. The potential adverse effects of PAH-specific therapies in such patients include worsening fluid retention, pulmonary edema, and ventilation perfusion mismatch. Melvyn Rubenfire, M.D., F.A.C.C.
2009年美国心脏病学会基金会（ACCF）/美国心脏病学会（AHA）有关肺动脉高压所达成的共识文件：一份关于美国心脏病学会基金会（ACCF）专家组在临床专家共识文件方面的报道。
专题：心衰/移植
发布日期：2009年3月30号上午10点
作者：McLaughlin VV，Archer SL, Badesch DB, 等
引文：美国心脏病协会杂志 2009；3月30日［Epub 提前打印］
临床试验：否
观点：以下10点有关肺动脉高压的专家共识文件需牢记。
1.肺动脉高压（PAH）是一种综合症即由于限制了通过肺动脉循环的血流，从而导致肺血管阻力增加（PVR），最终形成右心衰。多种致病途径中主要是由于血管平滑肌、内皮细胞以及血管外膜的改变引起，还有就是血管收缩/血管舒张环境的失衡。
2.在法国根据登记在册的情况来看每一百万人中有15个患有肺动脉高压。原发性肺动脉高压（IPAH）是最常见的类型且尤其多见于妇女。家族性PAH通常是由于成骨蛋白受体2的变异引起，而且遗传了带有不完全外显率和遗传预期的常染色体显性疾病。另外，PAH还与冠心病（CHD），结缔组织病，药物和毒素，人免疫缺陷病毒，门脉高压，血红蛋白病，以及骨髓增殖性疾病有关。每1000个新生儿就有8个罹患CHD，而且在患有不可修复的CHD病人当中有30%将会出现PAH。
3. 通过现代治疗PAH一年的死亡率是15%。评估不良预后的因素包括：早期的功能分级，缺乏运动能力，右房压高，显著的右心室不全，右心衰，低心指数，脑利钠肽升高和硬皮病谱疾病。
4. 对于有足够风险发展为PAH的病人要确保定期检查，当然也包括有家族史的原发性肺动脉高压（IPAH）患者，硬皮病谱疾病以及处于肝移植评估阶段的门脉高压患者。最适当的研究就是通过超声心电图来检查可疑病人。
5. PAH的诊断需要完善的右心导管插入来证实。PAH的血液动力学界定是平均肺动脉压＞25 mm Hg；肺毛细血管楔压（PCW），左心房压，或左心室舒张末期压≤15 mm Hg;并且PVR >3 个伍德单位。快速血管扩张试验应该在有经验的实验中心做，对象是长期服用钙离子拮抗剂治疗的IPAH患者。有明显的右心衰或血流动力学不稳定的患者除外。
6. 一般治疗措施包括饮食，锻炼，适当的预防接种，还有就是避免怀孕。对所以患有IPAH的患者推荐使用华法林抗凝治疗。利尿剂适用于右室压力负荷过大伴有症状出现者。对于氧饱和度>90%患者建议氧疗。使用钙离子拮抗剂治疗后，对血管扩张试验反应敏感者则应该密切观察这种治疗的安全性和疗效。
7. 持续静脉输注依前列醇以提高运动能力，血流动力学，还可提高IPAH患者的生存期，而对于急危重病人依前列醇则是首选的治疗方法。曲罗尼尔，一种类前列腺素制剂，可以通过持续静脉输注给药，也可以通过皮下注射。伊洛前列素通过适当的雾化装置吸入，每天6次。
8. 血管内皮受体抑制剂（ETAs）可以口服治疗以提高PAH的运动能力。 肝功能的监测应该每月不定时进行。磷酸二酯酶抑制剂5（PDE）也能提高PAH患者的运动能力和血流动力学。对于有不良预后指数的病人应该首先可以通过静脉给药治疗，同时对于疾病分级二级的患者或有三级症状者一开始就着手应用ETAs或PDE-5阻滞剂。早期试验提示两者联合应用治疗也许也是有效的。肺移植对于有进展的患者也是一种选择，尽管有完善的医学管理。
9. 由于疾病及其治疗的复杂性，PAH患者必须与有经验的医生和临床护理医生保持密切的联系。对于有晚期症状，右心衰，晚期血流动力学改变和静脉给药治疗或综合治疗的患者要增加诊室就医的频率。绝大多数专家采用一些方法来评估功能分级和运动能力，比如每次诊室就医可以做6分钟行走法或平板试验分级。
10. 任何紊乱都会引起左心充盈压升高，包括心脏收缩功能障碍，心脏舒张功能障碍，还有瓣膜性心脏病，都能导致肺动脉压的升高。对于罕见的病例，可能要考虑PAH的特殊治疗，对根本原因看是否已经过合理的处理，PCW是正常的或最低限度的升高，以及跨肺梯度和PVR显著升高。后者被成为是相称？PH或远大于基于左室压升高或肺疾病引起的肺动脉高压。PAH特殊治疗的潜在不良反应包括恶性液体潴留，肺水肿，以及通气灌注不良。美国心脏病学院士，Melvyn Rubenfire博士。
引自丁香园http://www.dxy.cn/bbs/post/view?bid=116&id=14286882&sty=1&tpg=1&age=0
感谢原文作者及丁香园docgao dr_duran
[此贴子已经被作者于2009-4-5 22:41:23编辑过]
