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1、肺动脉高压的诊断和临床分类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 PH ECG,chest radiograph,经胸经胸多多 普普 勒超勒超 声声 心动图心动图(TTE)III.PH clinical class identification IV.PAH evaluation(type,function capacity,右右 心心 导导 管检查管检查
2、haemodynamics)筛选：筛选：危险 因 子结缔组织疾病(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 morphogenet
3、ic proteins(BMPs)originally identified 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.症症 状状及体及体 征征症症 状：状：breathlessness 呼 吸 困 难、fatigue,weakness,心
4、悸、心 前 区 疼 痛 和 晕 厥体体 征：征：肺 动 脉 瓣 第 二 音(P2)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 ergo
5、metry peak VO2,功能分级 New York Heart Association(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-echocardiog
6、raphy,TTE)pulmonary artery systolic pressure(PASP),right ventricural systolic pressure(RVSP)三 尖 瓣 返 流速率(systolic tricuspid regurigitant velocity,TRV,v):RVSP=4V2+RAP mild 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 press
7、ure of 5 mmHg)经胸多普勒超声心动图 (2)右心室射血时间左、右心室形态 和功能右心室容积right ventricula index of myocardial performancetiming of mid-systolic deceleration of right ventricular ejection recognise 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
8、果DLco低于预计值55或FVC 预计值/DLco 预计值高于1.4，则 很 可能 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 CTExclude 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-resolut
9、ion)CT评估肺间质ILD、检出有无肺静脉阻塞性疾患(pulmonary 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
10、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10mmHg to reach mPAP 25 mmHg at rest or 30 mmHg with exercise PCWP=15 mmHg for PAH PH previous classified into 2 categories primary PH(PPH)secondary PHRevised Clinical Classification of Pulmonary Hypertension(Evian 1998;Venice 2003)Pulmonary arterial hypertension(P
11、AH)1.1.Idiopathic(IPAH)1.2.Familial(FPAH)1.3.Associated with(APAH):1.3.1.Collagen vascular disease 1.3.2.Congenital systemic-to-pulmonary shunts*1.3.3.Portal hypertensionPulmonary arterial hypertension(PAH)1.3.4.Human immunodeficiency virus(HIV)infection 1.3.5.Drugs and toxins 1.3.6.Other(thyroid di
12、sorders,glycogen storage disease,Gaucher disease,hereditary hemorrhagic telangiectasia,hemoglobinopathies,myeloproliferative disorders,splenectomy)Pulmonary arterial hypertension(PAH)1.4.Asssociated with significant venous or capillary involvement 1.4.1.Pulmonary veno-occlusive disease(PVOD)1.4.2.Pu
13、lmonary capillary hemangiomatosis(PCH)1.5.Persistent pulmonary hypertension of the newbornPulmonary hypertension with left heart disease 2.1.Left-sided atrial or ventricular heart disease 2.2.Left-sided valvular heart diseasePulmonary hypertension associated with lung diseases and/or hypoxemia 3.1.C
14、hronic obstructive pulmonary disease 3.2.Interstitial lung disease 3.3.Sleep-disordered breathing 3.4.Alveolar hypoventilation disorders 3.5.Chronic exposure to high altitude 3.6.Developmental abnormalitiesPulmonary hypertension due to chronic thrombotic and/or embolic disease 4.1.Thromboembolic obs
15、truction of proximal pulmonary arteries 4.2.Thromboembolic obstrction of distal pulmonary arteries 4.3.Non-thrombolic pulmonary embolism(tumar,parasiters,foreign 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-12s WHO 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
