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1、肺动脉高压的诊断和临床分类pulmonary hypertension(PH),诊 断 步 骤,I.clinical suspicion PH(screening)risk factors,family history,symptoms,signs II.detection of PHECG,chest radiograph,经胸多 普 勒超 声 心动图(TTE)III.PH clinical class identificationIV.PAH evaluation(type,function capacity,右 心 导 管检查 haemodynamics),筛选：,危险 因 子结缔组织疾
2、病(系 统 性 硬 化 症，系统性红斑狼疮，类风湿性关节炎等)减肥药(Aminorex,Fenfluramine,Defluramine)女性HIV感 染门脉高压先天性左向右分流(房缺,室缺,动脉导管未闭),早期发现，早期治疗，提高疗效。非创伤性、危险性低高敏感性和特异性。筛选方法:病史、体检、经胸超声心动图,Genetic Screening for Mutation,mutations in the bone morphogenetic protein receptor II(BMPR2)gene.Bone morphogenetic proteins(BMPs)originally id
3、entified as molecules that induce bone and cartilage formation,BMPs are also critical regulators of mammalian development.BMPs play an important role in the maintenance of normal pulmonary vascular physiology.,症 状及体 征,症 状：breathlessness 呼 吸 困 难、fatigue,weakness,心 悸、心 前 区 疼 痛 和 晕 厥体 征：肺 动 脉 瓣 第 二 音(P
4、2)亢 进三 尖 瓣 区 心 音 较 心 尖 部 明 显 增 强或 出 现收 缩 期 杂 音颈 静 脉 怒 张、肝 肿 大、肝 颈 反 流 征 阳 性、下 肢 浮 肿（右 心 功 能 不 全),运动耐量试验,评价功能状况及疗效six-minute walk test(6MWT)WHO/NYHA functional statusBorg dyspnoea scorecardiopulmonary exercise test:treadmil(usually Naughton protocol)or cycle ergometrypeak VO2,功能分级,New York Heart Asso
5、ciation(NYHA)or WHO Functional Classification一级：可进行正常体力活动二级：体力活动轻度受限三级:体力活动明显受限四级：不能无症状地进行任何体力活动 休息时仍感呼吸困难和/或 乏力,X 线 胸 片,中心肺动脉扩张而外周分支纤细，两者形成明显对比 右 下 肺 动 脉 扩 张，横 径15mm;右 下 肺 动 脉 横 径 与 气 管 横 径 比 值1.07;肺 动 脉 段 突 出3mm.,经胸多普勒超声心动图(Transthoracic Doppler-echocardiography,TTE),pulmonary artery systolic pres
6、sure(PASP),right ventricural systolic pressure(RVSP)三 尖 瓣 返 流速率(systolic tricuspid regurigitant velocity,TRV,v):RVSP=4V2+RAPmild PH:PASP 36-50(40-50 mmHg),corresponds to tricuspid regurgitant velocity 2.8-3.4(3.0-3.5)m/sec(assuming a normal right atrial pressure of 5 mmHg),经胸多普勒超声心动图(2),右心室射血时间左、右心室
7、形态 和功能右心室容积right ventricula index of myocardial performancetiming of mid-systolic deceleration of right ventricular ejectionrecognise left heart disease(Clinical class 2),肺 功 能,Decreased DLco(40-80%predicted)and reduction of lung volumes 19系统性硬化症患者有单纯肺弥散功能障碍 如果DLco低于预计值55或FVC 预计值/DLco 预计值高于1.4，则 很 可
8、能 进展 为 肺动脉高压。(Steen VD Arthritis Rheum.1992;35:765-770)(Sacks DG.J Rheumatol 1996;23:639-642),Ventilation and perfusion(V/Q)lung scanpulmonary angiography,CTEPH(Clinical class 4),Contrast-enhanced MRI or CT,Exclude CTEPH测量右心室、右心房和肺动脉形态和右心室功能。推荐应用高分辨率(high-resolution)CT评估肺间质ILD、检出有无肺静脉阻塞性疾患(pulmonary
9、 venocclusive disease)。,右 心 导 管检查,推荐应用于所有疑诊为肺动脉高压的患者测量右房压、右室收缩期和舒张末期压、肺动脉收缩期、舒张期和平均压、肺毛细血管嵌压、体循环和肺动脉血氧饱和度以及心输出量。证实肺动脉高压是否存在及严重程度,进行血管舒张试验，进行分型诊断，指导治疗。,血管舒张试验,所有患者在首次进行右心导管检查时应当应用短效血管舒张剂进行血管舒张试验，以评定他们对于血管舒张剂的反应。推荐静脉应用前列环素(epoprotenol)或吸入一氧化氮或静脉应用腺苷(adenosine)。患者对血管舒张试验有反应者可能会对口服钙通道阻滞剂有较好疗效。平均肺动脉压降低至少
10、10mmHg to reach mPAP=40 mmHg,心输出量无变化或增高者被认为有反应。,Clinical Classification of Pulmonary Hypertension,Evian,France 1998Venice,Italy 2003,Definition,Mean pulmonary artery pressure(mPAP)25 mmHg at restor 30 mmHg with exercisePCWP=15 mmHg for PAHPH previous classified into 2 categoriesprimary PH(PPH)second
11、ary PH,Revised Clinical Classification of Pulmonary Hypertension(Evian 1998;Venice 2003),Simonneau,JACC 2004;43:10S,Pulmonary arterial hypertension(PAH),1.1.Idiopathic(IPAH)1.2.Familial(FPAH)1.3.Associated with(APAH):1.3.1.Collagen vascular disease1.3.2.Congenital systemic-to-pulmonary shunts*1.3.3.
12、Portal hypertension,Pulmonary arterial hypertension(PAH),1.3.4.Human immunodeficiency virus(HIV)infection1.3.5.Drugs and toxins1.3.6.Other(thyroid disorders,glycogen storage disease,Gaucher disease,hereditary hemorrhagic telangiectasia,hemoglobinopathies,myeloproliferative disorders,splenectomy),Pul
13、monary arterial hypertension(PAH),1.4.Asssociated with significant venous or capillary involvement1.4.1.Pulmonary veno-occlusive disease(PVOD)1.4.2.Pulmonary capillary hemangiomatosis(PCH)1.5.Persistent pulmonary hypertension of the newborn,Pulmonary hypertension with left heart disease,2.1.Left-sid
14、ed atrial or ventricular heart disease2.2.Left-sided valvular heart disease,Pulmonary hypertension associated with lung diseases and/or hypoxemia,3.1.Chronic obstructive pulmonary disease3.2.Interstitial lung disease3.3.Sleep-disordered breathing3.4.Alveolar hypoventilation disorders3.5.Chronic expo
15、sure to high altitude3.6.Developmental abnormalities,Pulmonary hypertension due to chronic thrombotic and/or embolic disease,4.1.Thromboembolic obstruction of proximal pulmonary arteries4.2.Thromboembolic obstrction of distal pulmonary arteries4.3.Non-thrombolic pulmonary embolism(tumar,parasiters,f
16、oreign material),5.Miscellaneous,Sarcoidosis,histiocytosis X,lymphangiomatosis,compression of pulmonary vessels(adenopathy,tumar,fibrosing mediastinitis),主要参考文献,Simonneau G,et al.Clinical Classification of pulmonary hypertension.J Am Colle Cardio 2004;43:5s-12sWHO Evian Venice PH meeting陆慰萱，王辰主编。肺循环病学。北京，人民卫生出版社，2007,
