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1、The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First慢性肺源性心慢性肺源性心脏脏病病浙医二院滨江院区呼吸科浙医二院肺癌诊治中心丁礼仁The Needs of The Patients and The Customers
2、Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First主要内容主要内容概述概述病因病因发病机制及病理生理发病机制及病理生理表现表现诊断与鉴别诊断与鉴别处理原则处理原则The Needs of The Patients and The Customers Come FirstThe Needs of The P
3、atients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First体静脉体静脉右心房右心房右心室右心室肺动脉肺动脉肺肺肺静脉肺静脉左心左心房房左心室左心室主动主动脉脉血液气体交换右心室右心房概概 述述The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The C
4、ustomers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First定义定义由肺组织、肺血管或胸廓的慢性病变引起肺组织结构和（或）功能异常，产生肺血管阻力增加，肺动脉压力增高，使右心室扩张或（和）肥厚，伴或不伴右心功能衰竭的心脏病，并排除先天性心脏病和左心病变引起者。The Needs of The Patients and The Customers Come FirstThe Needs of The Pa
5、tients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstPage 5流行病学流行病学7070年代，年代，14y14y，患病率患病率4.84.89090年代，年代，15y15y，患病率患病率6.76.7agednessyoungsmokeno smoke哥抽的不哥抽的不是烟，是是烟，是命。命。The Needs of The Patients and The Custo
6、mers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstPage 6northsouth（地域性）（地域性）流行病学流行病学countycityThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients
7、 and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First主要内容主要内容概述概述病因病因发病机制及病理生理发病机制及病理生理表现表现诊断与鉴别诊断与鉴别处理原则处理原则The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come Firs
8、tThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First病因学病因学1.支气管、肺疾病：支气管、肺疾病：COPD（80%-90%）；）；The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers
9、 Come FirstThe Needs of The Patients and The Customers Come FirstPage 9病因学病因学2.胸廓运动障碍性疾病；胸廓运动障碍性疾病；（1）胸廓或脊椎畸形）胸廓或脊椎畸形（2）神经肌肉疾患）神经肌肉疾患The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe N
10、eeds of The Patients and The Customers Come First病因学病因学3.肺血管疾病；肺血管疾病；4.其他：睡眠呼吸暂停低通气综合征。其他：睡眠呼吸暂停低通气综合征。The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The C
11、ustomers Come First主要内容主要内容概述概述病因病因发病机制及病理生理发病机制及病理生理表现表现诊断与鉴别诊断与鉴别处理原则处理原则The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First发病机制发病机制肺
12、动脉高压的形成心脏病变和心力衰竭其它重要脏器损害The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First肺动脉高压肺动脉高压标准标准由多种原因或未知原因引起的肺动脉压异常升高的一种病理生理状态，血液动力学诊断标准为：在海平面
13、、静息状态下，右心导管测量平均肺动脉压（mean pulmonary artery pressure,mPAP)25mmHg。The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First肺动脉高压肺动脉高压与呼吸系统疾病或缺氧相
14、关的肺高压与呼吸系统疾病或缺氧相关的肺高压1.特发性2.遗传性 骨形成蛋白受体基因(BMPR2)突变 活化素受体样激酶(ALK-1)，转化生长因子-受体(endoglin)基因突变3.药物和毒物诱导4.相关因素所致 结缔组织病 HIV 感染 门脉高压 先天性心脏病 血吸虫病 慢性溶血性贫血5.新生儿持续性肺高压 1.肺静脉闭塞病和/或肺毛细血管瘤1.慢性阻塞性肺疾病2.间质性肺疾病3.其他同时存在限制性和阻塞性通气功能障碍的肺疾病4.睡眠呼吸障碍5.肺泡低通气综合征6.慢性高原病7.肺泡毛细血管发育不良慢性血栓栓塞性肺高压慢性血栓栓塞性肺高压不明原因或多种因素所致肺高压不明原因或多种因素所致肺
15、高压1.血液系统疾病：骨髓增生性疾病，脾切除2.全身性疾病：结节病，肺朗罕氏组织细胞增多症，淋巴管肌瘤病，多发性神经纤维瘤，血管炎3.代谢性疾病：糖原累积病，高雪氏病，甲状腺疾病4.其他：肿瘤性阻塞，纤维性纵隔炎，长期透析的慢性肾衰竭左心疾病相关肺高压左心疾病相关肺高压1.收缩功能障碍2.舒张功能障碍3.心脏瓣膜疾病ESC;ERS;ISHLT,Gali N,et al.European Heart Journal(2009)30,24932537 2008肺肺动动脉脉高高压压分分类类The Needs of The Patients and The Customers Come FirstTh
16、e Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First肺动脉高压的形成肺动脉高压的形成肺血管阻力增加的功能性因素肺血管阻力增加的功能性因素肺血管阻力增加的解剖因素血液粘稠度增加及血容量增多The Needs of The Patients and The Customers Come FirstThe Needs of The Pa
17、tients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First肺动脉高压功能性因素肺动脉高压功能性因素1.缺氧（最重要因素）缺氧（最重要因素）2.高碳酸血症高碳酸血症3.呼吸性酸中毒呼吸性酸中毒肺血管收肺血管收缩、痉挛缩、痉挛肺动脉高压肺动脉高压The Needs of The Patients and The Customers Come FirstThe Needs o
18、f The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First缺氧性肺血管收缩缺氧性肺血管收缩（1）体液因素作用；）体液因素作用；细细胞胞释释放血管活性物放血管活性物质质，缩缩血管物血管物质质增加增加（2）直接作用于血管平滑肌；）直接作用于血管平滑肌；细细胞内胞内Ca2+肌肉肌肉兴奋兴奋-收收缩缩偶偶联联效效应应增增强强（3）交感神）交感神经经作用。作用。交
19、感神交感神经兴奋经兴奋肺血管肺血管受体受体血管收血管收缩缩The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First高碳酸血症高碳酸血症呼吸性酸中毒呼吸性酸中毒H+血管对缺氧血管对缺氧的收缩敏感的收缩敏感性增强性增强肺动脉压升
20、高肺动脉压升高其他功能性因素The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First肺动脉高压的形成肺动脉高压的形成肺血管阻力增加的功能性因素肺血管阻力增加的解剖因素肺血管阻力增加的解剖因素血液粘稠度增加及血容量增多The
21、Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First肺动脉高压肺动脉高压解剖学因素解剖学因素血管血管炎炎管壁增厚、管管壁增厚、管腔狭窄、纤维腔狭窄、纤维化、闭塞化、闭塞血管阻血管阻力力a.肺小动脉肺小动脉肺动脉肺动脉高压高压No
22、rmal small Normal small pulmonary arterypulmonary arteryProliferative pulmonary Proliferative pulmonary vascular diseasevascular diseaseThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstTh
23、e Needs of The Patients and The Customers Come First肺动脉高压肺动脉高压解剖学因素解剖学因素b.肺毛肺毛细细血管血管肺气肿肺气肿肺泡内肺泡内压压肺泡毛细肺泡毛细血管受压血管受压管腔狭管腔狭窄、闭窄、闭塞塞肺泡壁肺泡壁破裂破裂毛细血管毛细血管网毁损网毁损肺循环肺循环阻力阻力70%The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients a
24、nd The Customers Come FirstThe Needs of The Patients and The Customers Come Firstc.肺血管重塑肺血管重塑缺氧缺氧肺血管肺血管收缩收缩管壁张管壁张力力肺内产生多肺内产生多种生长因子种生长因子平滑肌细胞、平滑肌细胞、弹力纤维、胶弹力纤维、胶原纤维增生原纤维增生肺血管管壁增厚、硬化，管腔狭窄肺血管管壁增厚、硬化，管腔狭窄肺动脉高压肺动脉高压解剖学因素解剖学因素The Needs of The Patients and The Customers Come FirstThe Needs of The Patients a
25、nd The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First肺动脉高压肺动脉高压解剖学因素解剖学因素d.血栓形成血栓形成多发性肺微多发性肺微小动脉原位小动脉原位血栓血栓肺血管肺血管阻力阻力肺动脉肺动脉高压高压The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Cus
26、tomers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First小结肺气肿慢性炎症血管炎肺泡内压肺泡壁破裂肺泡毛细血管受压毛细血管网毁损肺血管重塑管腔狭窄、闭塞肺血管阻力缺氧肺动脉高压多发肺小动脉栓塞The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come Firs
27、tThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First肺动脉高压的形成肺动脉高压的形成肺血管阻力增加的功能性因素肺血管阻力增加的解剖因素血液粘稠度增加及血容量增多血液粘稠度增加及血容量增多The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The
28、 Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First1.缺氧缺氧刺激肾小管旁间质细胞刺激肾小管旁间质细胞epo释释 放放RBC血黏度血黏度 缺氧缺氧醛固酮醛固酮水、钠潴留水、钠潴留血容量血容量 肾小动脉收缩肾小动脉收缩肾血流量肾血流量2.血黏度增加血黏度增加 血容量增多血容量增多The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Custom
29、ers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First病因病因缺氧缺氧高碳酸血症高碳酸血症呼酸呼酸血容量增加血容量增加血粘度增加血粘度增加血流阻血流阻力增加力增加肺循肺循环阻力增加阻力增加肺动脉高压肺动脉高压右心室扩张右心室扩张,肥大肥大/右心衰右心衰慢性肺心病慢性肺心病慢支累及慢支累及临近小近小A;肺气肺气肿，肺泡，肺泡压增增加，加，压迫毛迫毛细血管血管;肺泡破裂，毛肺泡破裂，毛细血血管床减管床减损;肺血
30、管收肺血管收缩及重构及重构;促红素促红素醛固酮增加醛固酮增加肾动脉收缩肾动脉收缩PGE.PGFTX,LTs5-HTAT2.PAFEDRF/EDCF肺心病发病机制示意图肺心病发病机制示意图The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers C
31、ome First心脏病变和心力衰竭心脏病变和心力衰竭肺动脉高压使右心室负荷增加慢性缺氧，呼吸系统反复感染酸碱及电解质失衡引起心律失常The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First多脏器损害多脏器损害肝肾功能受损害
32、水、电解质及酸碱失衡消化道出血播散性血管内凝血休克The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First主要内容主要内容概述概述病因病因发病机制及病理生理发病机制及病理生理表现表现诊断与鉴别诊断与鉴别处理原则处理原则The
33、 Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First一、肺、心功能代偿期（包括缓解期）一、肺、心功能代偿期（包括缓解期）症状症状：慢阻肺的表现(咳嗽、咳痰、气促、活动后闷气、呼吸困难、乏力、劳动耐力下降)体征体征：肺气肿征+肺
34、动脉高压+右室肥大 P2亢进分裂亢进分裂剑下心音心尖部 剑下心脏搏动三尖瓣收缩期杂音临床表现临床表现The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First临床表现临床表现二、肺、心功能失代偿期（包括急性加重期）二、肺、心功
35、能失代偿期（包括急性加重期）代偿期症状体征代偿期症状体征+呼吸衰竭呼吸衰竭心力衰竭心力衰竭缺氧和二氧化碳潴留引起的一系列表现（PaO2 50mmHg）颈静脉怒张肝颈静脉回流征阳性肝肿大压痛下肢水肿静脉压胸腹水The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The
36、Customers Come First症状：咳嗽、咳痰、气促，活动后可有心悸、呼吸困难、乏力和劳动耐力下降。急性感染可使上述症状加重。少有胸痛或咯血。体征：可有不同程度的发绀和肺气肿体征。偶有干、湿性啰音，心音遥远，P2A2，三尖瓣区可出现收缩期杂音或剑突下心脏搏动增强，提示有右心室肥厚。部分患者因肺气肿使胸内压升高，阻碍腔静脉回流，可有颈静脉充盈。此期肝界下移是膈下降所致。肺、心功能代偿期肺、心功能代偿期The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Custom
37、ers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First肺心肺心功能失代偿期功能失代偿期呼吸衰竭1.症状：呼吸困难加重，夜间为甚，常有头痛、失眠、食欲下降，但白天嗜睡，甚至出现表情淡漠、神志恍惚、谵妄等肺性脑病的表现。2.体征：明显发绀，球结膜充血、水肿，严重时可有视网膜血管扩张、视乳头水肿等颅内压升高的表现。腱反射减弱或消失，出现病理反射。因高碳酸血症可出现周围血管扩张的表现，如皮肤潮红、多汗。The Ne
38、eds of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First右心衰竭1.症状：气促更明显，心悸、食欲不振、腹胀、恶心等。2.体征：发绀更明显，颈静脉怒张，心率增快，可出现心律失常，剑突下可闻及收缩期杂音，甚至出现舒张期杂音。肝大且有压痛，肝
39、颈静脉回流征阳性，下肢水肿，重者可有腹水。少数患者可出现肺水肿及全心衰竭的体征。肺、心功能失代偿期肺、心功能失代偿期The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First辅助检查辅助检查X线检查心电图检查超声心动图血气分析
40、血化验其他：痰微生物，肺功能The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First右下肺右下肺动脉干脉干扩张，其横径其横径 15mm横径与气管横径横径与气管横径之比之比值 1.07肺肺动脉段明脉段明显突出突出或其高度或其高
41、度 3mm 右心室增大征右心室增大征胸片胸片The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First电轴右偏，重度顺钟向转位，Rv1+Sv5 1.05mV，肺型P波心电图The Needs of The Patients a
42、nd The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First 超声检查超声检查 右肺动脉内径18mm,或肺动脉干20mm 右室内径20mm 右室流出道30mmThe Needs of The Patients and The Customers Come FirstThe Need
43、s of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First诊断与鉴别诊断诊断与鉴别诊断基础疾病：COPD，支扩，胸廓畸形等肺动脉高压或右心肥大/衰竭的表现体征：P2A2、颈静脉怒张、肝大压痛、肝颈静脉反流征阳性、下肢水肿及体静脉压升高等辅助检查：CXR、ECGThe Needs of The Patients and The Customers C
44、ome FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First鉴别诊断鉴别诊断风湿性关节炎、风湿性关节炎、心肌炎病史，其心肌炎病史，其他瓣膜常有病变。他瓣膜常有病变。常有两病共存。冠常有两病共存。冠心病有典型的心绞心病有典型的心绞痛、心肌梗死病史痛、心肌梗死病史或心电图表现，体或心电图表现，体检、胸片、心电图、检、胸片
45、、心电图、UCG检查呈左心室检查呈左心室肥厚为主的征象。肥厚为主的征象。多为全心增大，无慢性呼吸道病史，多为全心增大，无慢性呼吸道病史，无肺动脉高压的无肺动脉高压的X线表现。线表现。The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Com
46、e First急性加重期急性加重期治疗治疗积极控制感染通畅呼吸道，改善呼吸功能纠正缺氧和二氧化碳潴留控制呼吸和心力衰竭积极处理并发症其它治疗The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come First治疗治疗急性加重期急性加重期
47、1.控制感染 经验用药，院外感染以G+菌占多数，院内感染以G菌占多数 根据痰培养及药敏选择有效抗生素常用的有青霉素常用的有青霉素类、氨基糖、氨基糖甙类、氟氟喹诺酮类、头孢菌素菌素类等抗菌等抗菌药The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Custome
48、rs Come First 治疗治疗急性加重期急性加重期2.通畅呼吸道，纠正缺氧和二氧化碳潴留排痰、支气管舒张剂雾化吸入湿化痰液排痰、支气管舒张剂雾化吸入湿化痰液气管插管或气管切开建立人工气道气管插管或气管切开建立人工气道氧疗氧疗应用呼吸兴奋剂应用呼吸兴奋剂呼吸机的使用呼吸机的使用 The Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come F
49、irstThe Needs of The Patients and The Customers Come First1）清除口咽部分泌物，）清除口咽部分泌物，预防返流至气管；防返流至气管；2）鼓励患者咳痰；）鼓励患者咳痰；3）应用祛痰用祛痰药物及痰液溶解物及痰液溶解剂；4）应用解用解痉、平喘、平喘药物；物；5）吸氧）吸氧6）必要）必要时气管切开和使用机械通气气管切开和使用机械通气。2.2.通畅呼吸道，纠正缺氧和二氧化碳潴留通畅呼吸道，纠正缺氧和二氧化碳潴留 治疗治疗急性加重期急性加重期The Needs of The Patients and The Customers Come FirstT
50、he Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstThe Needs of The Patients and The Customers Come FirstPage 461利尿药利尿药电解质、痰黏电解质、痰黏电解质、痰黏电解质、痰黏2正性肌力药正性肌力药耐受低、效较耐受低、效较耐受低、效较耐受低、效较差、小剂量差、小剂量差、小剂量差、小剂量3血管扩张药血管扩张药顽固性心衰试用顽固性心衰试用顽固性心衰试用顽固性心衰试用控制控制心力衰