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1、,肺心病的肺康复治疗,肺康复的概述,肺心病/肺动脉高压的肺康复,1,2,目录,CONTENTS,01,肺康复的概述,PART ONE,肺康复的历史,1970年以前（肺康复的历史阶段）历史上关于治疗性呼吸训练的记载可以追溯到1781年。1940-1950年，在美国和其他国家已经开始了对肺结核急性期后、神经肌肉疾病导致呼吸肌麻痹、急性脊髓灰质炎急性期后的病人的呼吸康复。,肺康复的历史,1970年以后（肺康复的现代阶段）1970年以后，肺康复在欧美广泛开展，因此称为肺康复的现代阶段。1975年，美国胸科学会（ATS）第一次提出肺康复的定义。随着检查手段的不断完善和设备的逐步更新使得评价技术不断进步，
2、对于呼吸系统疾病的认识也逐步更新，90年代以来循证医学又为我们带来更为科学的临床指导性的证据，肺康复在循证医学基础上得到不断的改进。,肺康复的历史,1981年，ATS正式发表了“肺康复的立场声明”1994年，美国国家卫生研究院发表了“肺康复研究展望”1997年，美国胸科医师学院（ACCP）和美国心血管和肺康复学会（AACVPR）共同发表了“肺康复-ACCP/AACVPR联合循证指南”1999年，ATS发表了“肺康复-1999”2006年，ATS和ERS（欧洲呼吸学会）共同发表了“肺康复的立场声明”2007年，更新了“肺康复-ACCP/AACVPR联合循证指南”2013年，ATS和ERS更新了“
3、肺康复的立场声明”,参考文献：1.Ries AL,et al.Pulmonary Rehabilitation:Joint ACCP/AACVPR Evidence-Based Clinical Practice Guidelines.Chest.2007 May;131(5 Suppl):4S-42S2.Spruit MA,et al.An official American Thoracic Society/European Respiratory Society statement:key concepts and advances in pulmonary rehabilitation
4、.Am J Respir Crit Care Med.2013 Oct 15;188(8):e13-64.,肺康复的定义-2006,肺康复是一种循证的、多学科的、针对有症状和日常活动减少的慢性呼吸疾病患者的综合干预措施。在患者个体化治疗中加入肺康复，旨在通过稳定或逆转疾病的系统损害来减轻症状、优化功能状态、增加参与度、减少卫生保健费用。全面的肺康复项目包括患者评估、运动训练、健康教育和心理社会支持。这个定义重点关注肺康复的3个重要特征：多学科、个体化、关注生理和社会功能,-ATS/ERS 肺康复声明2006,肺康复的定义,尽管旧的肺康复定义被广泛接受并使用，但是专家组成员认为有必要拟订新的肺康
5、复定义。这个决定是基于近期肺康复的研究进展的理解。部分综合性的肺康复项目基于多年的临床经验和专家意见，而不是循证医学。目前认为对慢性呼吸性疾病患者来说，肺康复不仅仅是多学科的方法，而更倾向于是一种跨学科的干预措施。旧的定义强调了稳定或逆转疾病症状的重要性，而没有特别关注行为干预。,-ATS/ERS 肺康复声明2013,肺康复的定义-2013,肺康复是在患者的个体化治疗后进行的、基于患者全面评估结果的一种综合干预措施，其治疗包括但不限于运动训练、健康教育和行为干预，旨在改善慢性呼吸疾病患者的生理及心理状态以及提高利于健康的行为的长期依从性。,-ATS/ERS 肺康复声明2013,康复锻炼与药物对
6、运动耐量的影响,参考文献：3.Casaburi et al.Improvement in Exercise Tolerance With the Combination of Tiotropium and Pulmonary Rehabilitation in Patients With COPD*Chest 2005;127:809-17,肺康复在慢阻肺治疗中的重要性,肺康复在整体医疗中的地位,-ATS/ERS 肺康复声明2013,肺康复的治疗目标,最大程度降低症状最大程度增加运动能力提高自理能力增加日常活动的参与能力改善健康相关的生命质量促进改善健康的长期行为改变,肺康复的效果,2.肺康复
7、可改善COPD患者的呼吸困难（推荐级别1A）3.肺康复可改善COPD患者的HRQOL（健康相关的生命质量）（推荐级别1A）,ACCP/AACVPR肺康复指南 2007,肺康复的效果,4.肺康复可减少COPD患者的住院天数及其他医疗手段的应用（推荐级别2B）7.COPD患者应用综合性肺康复方案可获得心理社会方面的益处（推荐级别2B）如减压、改善认知功能、减少焦虑和抑郁症状、提高患者对疾病的积极结果的感受。,ACCP/AACVPR肺康复指南 2007,肺康复的适用范围,-ATS/ERS 肺康复声明2013,肺康复的内容,全面评估建立长期治疗与随访计划运动训练吸气肌训练健康教育营养支持社会心理支持等
8、,运动训练,运动训练是肺康复的基础“运动训练是综合性肺康复方案的基石（推荐级别为1A级），最具有循证医学证据，是肺康复方案中不可缺少的内容，其他方法均应建立在运动疗法的基础之上。”运动训练包括,耐力训练间歇性训练阻力/力量训练,上肢训练柔韧性训练神经肌肉电刺激,-ATS/ERS 肺康复声明2013,运动训练的方案设置,原则：超负荷、循序渐进方案内容频率（Frequency）强度（Intensity)时间（Time）类型（Type）,-ATS/ERS 肺康复声明2013,运动训练的设计与流程,综合的患者评估和优化治疗极量运动耐受试验,确定最大运动耐量评估运动受限的原因-通气受限-气体交换受限-心
9、功能受限-外周肌肉功能障碍-呼吸肌功能障碍,-ATS/ERS 肺康复声明2013,神经肌肉电刺激,适用于严重通气和/或心功能限制的患者，包括因疾病急性加重或呼吸衰竭入院者。小巧、相对便宜、便携式的电刺激同样适合家庭使用，这种方法使得对于重度残疾而无法离家的患者，或者需要家庭机械通气及缺乏传统肺康复项目的患者可以获益。,-ATS/ERS 肺康复声明2013,神经肌肉电刺激,被动式运动训练不会引起呼吸困难心血管循环需求较小绕过了传统运动中个体的认知、能动性以及生理状态方面等可能会影响运动训练效果的因素,股四头肌电刺激,-ATS/ERS 肺康复声明2013,吸气肌训练（IMT）,新指南中列举的6项研
10、究结果均证明，呼吸肌训练可以改善患者的呼吸肌功能，增强其运动能力，减轻呼吸困难的症状，但都是单中心、小样本研究，因此专家组建议:现有证据不支持常规应用吸气肌训练对进行药物治疗后仍有呼吸肌力量减弱或呼吸困难的患者进行吸气肌训练。进行大规模、多中心的随机对照研究，用足够的统计学数据支持常规应用吸气肌训练。,-ATS/ERS 肺康复声明2013,健康教育,17.健康教育应该作为肺康复的一部分。健康教育应该包括关于协作性自主管理以及慢阻肺加重期的预防和处理。推荐等级 1B。,ACCP/AACVPR肺康复指南 2007,社会心理支持,18.极少有研究支持将心理学干预作为单一的治疗手段。推荐等级 2C。1
11、9.虽然因缺乏科学的证据不能做出推荐，目前的临床实践以及专家意见支持将心理干预措施作为针对慢阻肺患者的全面肺康复的一部分。,ACCP/AACVPR肺康复指南 2007,营养支持,23.目前尚无充分证据支持在慢阻肺患者肺康复中常规进行营养补充。无具体建议。,ACCP/AACVPR肺康复指南 2007,呼吸支持技术,20.严重的活动性低氧血症患者进行肺康复期间应辅助氧疗。推荐等级 1C21.无活动性低氧血症的患者高强度运动时进行氧疗可能改善患者的运动耐量。推荐等级 2C22.严重COPD患者运动时辅助进行无创通气治疗可额外适度获益。推荐等级 2B,ACCP/AACVPR肺康复指南 2007,02,
12、肺心病/肺动脉高压的肺康复,PART TWO,实用内科学（第14版）,慢性肺源性心脏病采用膈式呼吸及缩唇呼吸等康复锻炼，以改善肺脏通气。对缓解期的患者进行康复治疗及开展家庭病床工作能明显降低急性期的发作。,参考文献：4.陈灏珠,林果为,王吉耀.实用内科学(第14版）.人民卫生出版社.2014,Pulmonary Hypertension in COPD:Epidemiology,Significance,and Management Pulmonary Vascular Disease:The Global Perspective,研究表明，肺康复可改善肺动脉高压和COPD患者的功能能力。药物
13、治疗最大化时，可能肺康复对于COPD相关的PH患者也是有益的。目前尚无针对COPD相关的PH患者进行肺康复的具体建议。,参考文献：5.Minai OA,et al.Pulmonary Hypertension in COPD:Epidemiology,Significance,and Management Pulmonary Vascular Disease:The Global Perspective.Chest.2010 Jun;137(6 Suppl):39S-51S.,2015 ESC/ERS Guidelines for the diagnosis and treatment of
14、pulmonary hypertension,This recommendation is limited by gaps in the knowledge about the optimal method of exercise rehabilitation and the intensity and duration of the training.In addition,the characteristics of the supervision and the mechanisms for the improvement of symptoms,exercise and functio
15、nal capacity are unclear,as are the possible effects on prognosis.In addition,patients should be treated with the best standard of pharmacological treatment and in stable clinical condition before embarking on a supervised rehabilitation programme.,参考文献：6.Galie N,et al.2015 ESC/ERS Guidelines for th
16、e diagnosis and treatment of pulmonary hypertension.Rev Esp Cardiol(Engl Ed).2016 Feb;69(2):177,Guidelines for Pulmonary Rehabilitation Programs 4E,参考文献：AACVPR.Guidelines for Pulmonary Rehabilitation Programs(4th Revised edition).Human Kinetics Publishers,2010,An official American Thoracic Society/E
17、uropean Respiratory Society statement:key concepts and advances in pulmonary rehabilitation,The initial prescription is generally formulated on the basis of an exercise test such as cardiopulmonary exercise testing or 6-minute walk test along with evaluation of exertional symptoms.The optimal exerci
18、se training program remains currently unknown.Light or moderate aerobic,and light resistive training are recommended forms of exercise.Slow,incremental exercise protocols at low intensity and short duration are often used initially.It would be prudent to avoid interval training because of the associ
19、ated rapid changes in pulmonary hemodynamics and risk of syncope.On the basis of symptoms and heart rate/oxygenation response,the intensity and duration of exercise may be advanced as tolerated.However,the target level for exercise training is generally kept at a submaximal level.currently no restri
20、ctions for upper or lower extremity strengthening exercises Range of motion exercises and flexibility training can also be performed safely by these individuals.,Similar recommendation as Guidelines for Pulmonary Rehabilitation Programs 4E,Special Considerations,Although light-intensity resistance e
21、xercise may be included,this is generally performed only when the patient can comply with appropriate breathing patterns to avoid the Valsalva-type maneuver.Care must be taken to maintain SaO288%during exercise and supplemental O2 should be available.,参考文献：2.Spruit MA,et al.An official American Thor
22、acic Society/European Respiratory Society statement:key concepts and advances in pulmonary rehabilitation.Am J Respir Crit Care Med.2013 Oct 15;188(8):e13-64.,参考文献：7.Candemir İ,et al.Pulmonary rehabilitation efficiency in COPD patients with pulmonary hypertension.Tuberk Toraks.2015 Sep;63(3):178-84.
23、,Pulmonary rehabilitation efficiency in COPD patients with pulmonary hypertension,Pulmonary rehabilitation and exercise in pulmonary arterial hypertension:An underutilized intervention,ConclusionFrom the trials that have been conducted,the evidence supports the benefits and safety of exercise traini
24、ng and rehabilitation programs in the pulmonary hypertension populations.Initial exercise training and pulmonary rehabilitation programs were assessed in a stable,treated,systolic heart failure,and COPD and showed improved functional capacity and reduced exercise intolerance,symptoms of dyspnea,and
25、fatigue.Trials conducted in a pulmonary hypertension population showed improvement in exercise endurance and muscle strength as well as quality of life with the implementation of exercise training and cardiopulmonary rehabilitation as part of their medical care without a good safety profile.In conju
26、nction with pharmacotherapy all patients with confirmed pulmonary arterial hypertension should be treated with a pulmonary rehabilitation and exercise training program.,参考文献：8.Sahni S,et al.Pulmonary rehabilitation and exercise in pulmonary arterial hypertension:An underutilized intervention.J Exerc
27、 Rehabil.2015 Apr 30;11(2):74-9.,参考文献：9.Marra AM,et al.Principles of rehabilitation and reactivation:pulmonary hypertension.Respiration.2015;89(4):265-73.,PA=Pulmonary artery;RV=right ventricular;SMC=smooth muscle cell,Principles of rehabilitation and reactivation:pulmonary hypertension,参考文献：9.Marra
28、 AM,et al.Principles of rehabilitation and reactivation:pulmonary hypertension.Respiration.2015;89(4):265-73.,Inspiratory muscle training in pulmonary arterial hypertension,参考文献：10.Saglam M,et al.Inspiratory muscle training in pulmonary arterial hypertension.J Cardiopulm Rehabil Prev.2015 May-Jun;35
29、(3):198-206.,Figure 1.(A)Comparison of maximum inspiratory pressure(MIP)between inspiratory muscle training(IMT)and control groups.(B)Comparison of maximum expiratory pressure(MEP)between IMT and control groups.,呼吸肌是呼吸动力，其中膈肌是最重要的呼吸肌。膈肌移动1cm,肺通气量增加约350ml占静息呼吸的75%-80%但耗氧量占比20%膈肌发生废用性萎缩的速度是其他骨骼肌的8倍,参考
30、文献：1 Thomason DB,Biggs RB,Booth FW.Protein metabolism and beta-myosin Heavy chain mRNA in unweighted soleus muscle.Am J Physio l.1989,257:300-305.,膈肌 呼吸动力之源,参考文献：11.DeRuisseau KC,et al.Diaphragm Unloading via Controlled Mechanical Ventilation Alters the Gene Expression Profile.Am J Respir Crit Care
31、Med.2005 Nov 15;172(10):1267-75,Diaphragm muscle fiber weakness in pulmonary hypertension,In rats with PH,twitch and maximal tetanic force generation of diaphragm strips were significantly lower,and the force-frequency relation was shifted to the right(i.e.,impaired relative force generation)compare
32、d with control subjects.Diaphragm fiber CSA was significantly smaller in rats with PH compared with controls,and was associated with increased expression of E3-ligases MAFbx and MuRF-1.In line with the rat data,studies on patients with PH revealed significantly reduced CSA and impaired contractility
33、 of diaphragm muscle fibers compared with control subjects,with no changes in quadriceps muscle.,参考文献：12.de Man FS,et al.Diaphragm muscle fiber weakness in pulmonary hypertension.Am J Respir Crit Care Med.2011 May 15;183(10):1411-8,Figure 2A.Typical example of diaphragm muscle sections of controls a
34、nd rats with PH stained for slow(antibody brd-5)and fast muscle fibers(antibody sc-71).,Figure 2B.Cross-sectional area of diaphragm muscle fibers from rats with PH was significantly reduced,irrespective of fiber type.,Control,n=5;PH,n=5.*P0.05,*P 0.01,*P 0.001.CON=control.,The CSA of both slow and f
35、ast diaphragm fibers are significantly smaller in rats with PH than in controls.The SuHx model showed a comparable magnitude of diaphragm muscle fiber atrophy.In contrast,no atrophy was observed in a model for PAB.No changes in the CSA of EDL muscle fibers were observed between rats with PH and cont
36、rols(slow fibers,1,33470 vs.1,36673 um2;fast fibers,3,079164 vs.2,857 134 um2;control vs.PH,respectively).These findings indicate specific atrophy of diaphragm muscle fibers in PH.,Figure 7.Selective diaphragm fiber atrophy in patients with PH,Selective diaphragm muscle atrophy was observed in six p
37、atients with PH compared with six control subjects.(A)Left:Typical examples of diaphragm muscle fiber cross-sections of a control subject and a patient with PH.Right:Average cross-sectional area of diaphragm muscle fibers for six control subjects and six patients with PH.(B)In contrast to the diaphr
38、agm,quadriceps muscle fiber cross-sectional area was preserved.Left:Typical examples of quadriceps fiber cross-sections of a control subject and a patient with PH.Right:Average cross-sectional area of quadriceps muscle fibers from four control subjects and four patients with PH.Data presented as mea
39、n 6 SEM.,A marked reduction in the CSA of diaphragm muscle fibers from patients with PH was observed(Figure 7A),indicating severe diaphragm fiber atrophy in patients with PH.,In contrast,CSA of quadriceps muscle in patients with PH was on average increased compared with control subjects;however,this
40、 did not reach statistical significance(Figure 7B),Figure 8.Diaphragm muscle fiber weakness in patients with PH,The maximal forcegenerating capacity of diaphragm muscle fibers was severely impaired(50%reduction)(Figure 8)in patients with PH compared with control subjects.,Force-velocity relationship
41、 from sample control(open circles)and PH mice(closed circles).B)Maximal shortening velocity(Vmax)determined from extrapolation of shortening velocity to zero force using Hill equation.Force-power relationship of sample control and PH mice.Peak Power from all mice in each group.,Figure 3.Pulmonary hy
42、pertension impairs isotonic contractile properties of isolated diaphragm bundles.,An important and novel observation was that PH impaired isotonic contractile properties in the diaphragm.Specifically,maximal shortening velocity(Vmax)was 40%slower and peak power was 63%lower in PH than control(P0.05;
43、),despite a 138%increase in curvature of the force velocity relationship.The differences in the force-velocity characteristics remained afternormalizing for Po(Fig.3A).,参考文献：13.Ahn B,et al.Diaphragm atrophy and contractile dysfunction in a murine model of pulmonary hypertension.PLoS One.2013 Apr 22;
44、8(4):e62702.,Diaphragm atrophy and contractile dysfunction in a murine model of pulmonary hypertension,Ondines Curse:hemodynamic response to diaphragm pacing(electrophrenic respiration),There was a fall in mean pulmonary artery pressure on diaphragm pacing,from 30 mm.Hg at rest to 25 mm.Hg(p0.01).Th
45、e decrease was most prominent in cases with pulmonary hypertension.Administration of 100%oxygen by inhalation did not significantly affect mean pulmonary artery pressure,and oxygen inhalation plus pacing did not decrease mean pulmonary artery pressure further than did pacing alone.,参考文献：14.Langou RA
46、,et al.Ondines Curse:hemodynamic response to diaphragm pacing(electrophrenic respiration).Am Heart J.1978 Mar;95(3):295-300.,Reversal of pulmonary hypertension after diaphragm pacing in an adult patient with congenital central hypoventilation syndrome,参考文献：15.Morlot-Panzini C,et al.Reversal of pulmo
47、nary hypertension after diaphragm pacing in an adult patient with congenital central hypoventilation syndrome.Int J Artif Organs.2013 Jun 25;36(6):434-8.,Comparison of inspiratory muscle training(IMT),15%had a score of 10,suggestive of symptoms of depressions,40%had mild to moderate symptoms(PHQ-8 s
48、core 4-9),and 45%had no depression symptoms(PHQ-8 score 0-3).Study further investigated effects on cognition and intellectual function in patient and found profound impairment in motor ability(57%),executive dysfunction(15%),and impaired attention in 13%.Though 70%of patients received medical therap
49、y for pulmonary hypertension,only 24%received any therapy for mental illness.While pulmonary rehabilitation programs can have a positive impact on patients,physicians should also utilize cognitive behavioral therapy(CBT)as part of regular care.,参考文献：16.Verma S,et al.Depression in pulmonary arterial
50、hypertension and interstitial lung diseases.N Am J Med Sci.2014 Jun;6(6):240-9.,Depression in pulmonary arterial hypertension and interstitial lung diseases,Depression in pulmonary arterial hypertension:Anundertreated comorbidity,The best treatment for depression in PAH patients is not known.lack of
