重症肺动脉高压致急性右心功能不全.ppt

《重症肺动脉高压致急性右心功能不全.ppt》由会员分享，可在线阅读，更多相关《重症肺动脉高压致急性右心功能不全.ppt（71页珍藏版）》请在淘文阁 - 分享文档赚钱的网站上搜索。

1、重症肺动脉高压致急性右心功能不全中南大学湘雅医院 余再新大连 2011729患者基本情况患者：性别：男年龄：48岁 婚况：已婚入院日期：2011年06月30日主诉：发热7天现病史n n患者于患者于7 7天前开始无明天前开始无明显诱显诱因出因出现发热现发热，体温，体温骤骤然升高在然升高在3939摄摄氏度以上，最高达氏度以上，最高达4141摄摄氏度。持氏度。持续续数小数小时时后能自行后能自行缓缓解，解，以夜以夜间间明明显显，并，并阵发阵发性咳嗽，以干咳性咳嗽，以干咳为为主，无痰，无咯血，主，无痰，无咯血，伴畏寒、盗汗，盗汗以午后及夜伴畏寒、盗汗，盗汗以午后及夜间间明明显显，无寒，无寒战战，无明，无

2、明显显胸痛、胸胸痛、胸闷闷，无，无恶恶心、呕吐，无关心、呕吐，无关节节疼痛，感活疼痛，感活动动后气促，后气促，经经休息后能逐休息后能逐渐缓渐缓解，无夜解，无夜间阵发间阵发性呼吸困性呼吸困难难及端坐呼吸，及端坐呼吸，遂就遂就诊诊于广州新海医院，行胸片于广州新海医院，行胸片检查检查示示右右侧侧液气胸液气胸，经输经输液治液治疗疗（具体不（具体不详详）症状好）症状好转转不明不明显显，为为求求进进一步一步诊诊治故治故来我院就来我院就诊诊，收入我科。患者起病以来，精神食欲欠佳，收入我科。患者起病以来，精神食欲欠佳，大小便尚可，睡眠欠佳。体重大小便尚可，睡眠欠佳。体重较较前无明前无明显变显变化。化。n n既

3、往史：患者于患者于5 5年前开始多于活年前开始多于活动动后出后出现现气促，气促，经经休息后能休息后能缓缓解，曾至本院解，曾至本院诊诊断断为为“慢性血栓栓塞性肺慢性血栓栓塞性肺动动脉高脉高压压”，长长期口服期口服“华华法林片法林片 2.5mg 2.5mg”1 1次次/日，未日，未规则规则监测监测INRINR。否。否认认“高血高血压压”病史，否病史，否认认“肝炎肝炎”“”“结结核核”等等传传染染病史，无重大外病史，无重大外伤伤史，无史，无输输血史，否血史，否认药认药物物过过敏史，敏史，预预防接种史不防接种史不详详。n n个人史、婚育史、家族史无特殊。既往史：体格检查T 39.9 T 39.9 P

4、130 P 130次次/分分 R 22 R 22次次/分分BP130/70mmHgBP130/70mmHg发发育正常，神志育正常，神志尚清，体尚清，体查查合作，合作，急性重病容，半坐位急性重病容，半坐位。全身皮肤、巩膜无黄染，。全身皮肤、巩膜无黄染，皮肤无出血点，皮肤皮肤无出血点，皮肤弹弹性可。性可。全身浅表淋巴全身浅表淋巴结结无无肿肿大大。头颅头颅五官五官大小无畸形，大小无畸形，结结膜无充血，双瞳孔等大等膜无充血，双瞳孔等大等圆圆，对对光反射灵敏。外光反射灵敏。外耳道无流耳道无流脓脓。鼻翼无煽。鼻翼无煽动动，口唇无，口唇无发绀发绀，咽无充血，扁桃体不大。，咽无充血，扁桃体不大。颈软颈软，颈颈

5、静脉充盈静脉充盈，颈动颈动脉搏脉搏动动可，未可，未闻闻及明及明显显血管血管杂杂音。气管音。气管居中，甲状腺不大。胸廓居中，甲状腺不大。胸廓对对称，称，右右侧语颤侧语颤减弱，右上肺叩减弱，右上肺叩诊诊呈呈过过清音，右下肺叩清音，右下肺叩诊诊呈呈实实音，左肺叩音，左肺叩诊诊呈清音，右肺呼吸音呈清音，右肺呼吸音较较左左侧侧低，双下肺可低，双下肺可闻闻少少许许湿湿罗罗音音，无胸膜摩擦音。腹平，无胸膜摩擦音。腹平软软，未，未见见胃胃肠肠型及蠕型及蠕动动波，无波，无压压痛，无反跳痛，肝脾肋下未痛，无反跳痛，肝脾肋下未扪扪及，移及，移动动性性浊浊音音阴性，双阴性，双侧肾侧肾区无叩区无叩击击，肠鸣肠鸣音音4

6、4次次/分。肛分。肛门门外生殖器未外生殖器未见见明明显显异常。脊柱四肢无畸形，双下肢无水异常。脊柱四肢无畸形，双下肢无水肿肿，生理反射可，病理征未，生理反射可，病理征未引出。引出。体格检查 专专科科检查检查：心前区无异常隆起，心尖搏动位于第5肋间左锁骨中线外1cm处，胸骨左缘4-5肋间可见心脏搏动，无抬举性心尖搏动，触无震颤，无心包摩擦感，心界向左扩大，心率130次/分，心律齐，P2A2，胸骨左缘3-4肋间可闻及舒张期奔马律，无杂音，无心包摩擦音。周围血管征阴性。4749505657辅助检查 右心右心右心右心导导导导管管管管检查检查检查检查（2010-06-242010-06-24）示：）示：

7、）示：）示：PCWP 10 PCWP 10 mmHg,mPAP 65mmHg,CO 3.2 L/min,mmHg,mPAP 65mmHg,CO 3.2 L/min,肺肺肺肺动动动动脉阻脉阻脉阻脉阻力力力力 17.2 Wood 17.2 Wood血常血常规规相关相关检查检查ESR(7.13):ESR(7.13):67mm/h.67mm/h.NT-proBNPNT-proBNP：2087pg/ml2087pg/ml，结结结结核抗体：核抗体：核抗体：核抗体：IgGIgG弱阳性。弱阳性。弱阳性。弱阳性。胸水常胸水常胸水常胸水常规规规规：黄色黄色黄色黄色浑浊浑浊浑浊浑浊，李凡它，李凡它，李凡它，李凡它试

8、验试验试验试验阳性，阳性，阳性，阳性，细细细细胞胞胞胞总总总总数数数数1920*106/l1920*106/l。胸水生化：胸水生化：胸水生化：胸水生化：腺苷脱腺苷脱腺苷脱腺苷脱氢氢氢氢酶酶酶酶53u/l53u/l，乳酸脱，乳酸脱，乳酸脱，乳酸脱氢氢氢氢酶酶酶酶1260.7u/l1260.7u/lD-D-二聚体二聚体二聚体二聚体 3.18mg/l 3.18mg/l血、胸水、骨髓培养均无菌生血、胸水、骨髓培养均无菌生血、胸水、骨髓培养均无菌生血、胸水、骨髓培养均无菌生长长长长，多次胸水病，多次胸水病，多次胸水病，多次胸水病检检检检未未未未找到癌找到癌找到癌找到癌细细细细胞，肥达氏反胞，肥达氏反胞，

9、肥达氏反胞，肥达氏反应应应应，甲功三，甲功三，甲功三，甲功三项项项项，肌，肌，肌，肌钙钙钙钙蛋白，蛋白，蛋白，蛋白，血清血清血清血清肿肿肿肿瘤相关物瘤相关物瘤相关物瘤相关物质质质质，C-12C-12，PPDPPD，ENA+ENA+狼狼狼狼疮疮疮疮全全全全套套套套+免疫全套，免疫全套，免疫全套，免疫全套，肾肾肾肾病全套，抗心磷脂抗体均未病全套，抗心磷脂抗体均未病全套，抗心磷脂抗体均未病全套，抗心磷脂抗体均未见见见见明明明明显显显显异常。异常。异常。异常。血气血气606162636465716667686970727353547475辅辅助助检查检查心心心心脏脏脏脏彩超（彩超（彩超（彩超（2011

10、-07-062011-07-06）：）：）：）：床旁心床旁心脏脏彩超示：彩超示：左房左房（32mm32mm）左室（左室（54mm54mm）右房右房（48*55mm48*55mm）右室（右室（33mm33mm，4CV52mm4CV52mm）主主动动脉直径脉直径30mm30mm，肺，肺动动脉脉34mm34mm，下腔静脉，下腔静脉 14mm 14mm，EF 62%EF 62%。右室壁增厚（前壁右室壁增厚（前壁10mm10mm），三尖瓣患），三尖瓣患扩张扩张，开放可，开放可，关关闭闭裂隙。三尖瓣返流速裂隙。三尖瓣返流速4.50m/s4.50m/s。提示：提示：肺肺动动脉高脉高压压 右房、右室大。右房、

11、右室大。三尖瓣中度返流、二尖瓣三尖瓣中度返流、二尖瓣轻轻度返流度返流 心包少量心包少量积积液（后心包液（后心包5mm5mm）入院诊断 1、发热查因：肺部感染结核肿瘤 2、慢性血栓栓塞性肺动脉高压 心脏扩大 心功能级 3、自发性气胸治疗方案1、抗感染：、抗感染：氨曲南（6.30-7.12）左氧氟沙星(7.2-7.11）万古霉素（7.12-7-25）莫西沙星（7.11-7.25）2、降肺、降肺动动脉高脉高压压：西地那非0.02 3次/日,法舒地尔、前列地尔,阿托伐他汀。3、抗凝：、抗凝：华法林片 2.5mg 1次/日（6.30-7.4）；3.125mg 1次/日（7.4-7.11）；3.438mg

12、1次/日（7月4至今）治疗方案4.抗抗结结核核 异烟肼 0.3 1次/日 利福喷丁 0.45 2次/周 乙胺丁醇 0.75 1次/日5、胸穿置管引流、胸穿置管引流（7.1-7.20）共引流出约2500ml淡黄色液体。6、护护肝降肝降酶酶：还原性谷胱肝肽、肝泰乐7、强强心：心：西地兰、多巴酚丁胺急性右心衰急性右心衰n n7 7月月2323日，患者下床解大便后突日，患者下床解大便后突发发呼吸困呼吸困难难，胸，胸闷闷，濒濒死感，大汗淋漓死感，大汗淋漓n n查查：Bp 136/78mmHg,R36Bp 136/78mmHg,R36次分次分 端坐呼吸，唇端坐呼吸，唇紫紫绀绀，双肺湿，双肺湿罗罗音无明音无

13、明显显增加，增加，心率心率163163次次/分，分，心律心律齐齐，P2A2P2A2，胸骨左，胸骨左缘缘3-43-4肋肋间间可可闻闻及舒及舒张张期奔期奔马马律。律。n n急急查查血气分析，血气分析，电电解解质质，D D二聚体，二聚体，ECGECG387677处理n n无无创创呼吸机呼吸机辅辅助呼吸助呼吸n n静脉滴注法舒地静脉滴注法舒地尔尔n n静脉注射西地静脉注射西地兰兰 0.4mg 0.4mgn n静脉注射静脉注射呋呋塞米塞米20mg20mgn n静脉滴注多巴酚丁胺静脉滴注多巴酚丁胺 5ug/kg/min 5ug/kg/min415859目前目前诊诊断断n n1、发热查因：结核肿瘤n n2、

14、慢性血栓栓塞性肺动脉高压n n 心脏扩大n n 心功能级n n3、自发性气胸n n4、白塞氏病？n n5、病毒性肝炎 慢性 乙型概 念右心功能衰竭(Right heart failure)是指任何原因导致的心血管结构或功能异常，损害右心室充盈或射血功能的临床综合征，且右心功能衰竭的病残率和死亡率均高于左心衰竭。目前对于右心功能和右心功能衰竭的研究远远落后于左心功能和左心功能衰竭。5Effect of Ventricular Contractionn nRight ventricular Contraction is sequential,starting with Right ventricu

15、lar Contraction is sequential,starting with the inlet and ending at the infundibulum.the inlet and ending at the infundibulum.Inward movement of the free wall(bellows effect)Inward movement of the free wall(bellows effect)Contraction of the longitudinal fibers,which shortens Contraction of the longi

16、tudinal fibers,which shortens the long axis.the long axis.Traction of the free wall caused by contraction of the Traction of the free wall caused by contraction of the LV.(wringing effect)LV.(wringing effect)n Shortening of the RV is greater longitudinally than radially.In contrast to the LV,twistin

17、g and rotational movements do not contribute significantly to RV contraction.n nThis leads to near complete emptying of the This leads to near complete emptying of the RVreduced RVEDPOptimal Venous return.RVreduced RVEDPOptimal Venous return.56n nRight Ventricular PerfusionRight Ventricular Perfusio

18、n In a right dominant system;In a right dominant system;Lateral Wall-Marginal branches of RCALateral Wall-Marginal branches of RCA Posterior and Inferoseptal-PDAPosterior and Inferoseptal-PDA Anterior and Anteroseptal-Branches of LADAnterior and Anteroseptal-Branches of LADn nRCA is relatively resis

19、tant to ischemia compared to the RCA is relatively resistant to ischemia compared to the LV.LV.Lower oxygen consumptionLower oxygen consumption More extensive collateral systemMore extensive collateral system The ability to increase its O2 extraction during increase demand.The ability to increase it

20、s O2 extraction during increase demand.n nNormally proximal RCA flow occurs in both diastole and Normally proximal RCA flow occurs in both diastole and Systole.Unlike LCA flow to the LV.This is secondary to Systole.Unlike LCA flow to the LV.This is secondary to the transmural pressure generated duri

21、ng systole.the transmural pressure generated during systole.67Aortic Pressure and Coronary Blood Flow7The response of the RV and LV to experimental increase in afterload.Pathophysiology of failing RVPiazza,G.et al.Chest 2005;128:1836-18522008年美国Dana point 分类18Autonomic nervous systemIn a dog model o

22、f RV failure caused by pulmonary artery banding,demonstrated a decrease in beta-adrenergic receptor density in the stressed RV.Interestingly,the reduced beta-adrenergic receptor density was not limited to the failing ventricle but also occurred in the LV.In patients with PAH,elevated catecholamine l

23、evels were associated with higher pulmonary vascular resistance and lower cardiac index.1819 Renin-angiotensin-aldosterone systemRV pressure overload led to a loss of responsiveness to the inotropic effects of angiotensin II and to an uncoupling of angiotensin 1 receptors to downstream signaling pat

24、hways.In patients with corpulmonale,activation of the renin-angiotensin-aldosterone system also may contribute to fluid retention and ventricular remodeling1920Endothelin systemEndothelin system activation may be important feature of pulmonary vascular disease and right HF.In a monocrotaline-induced

25、 PAH rat model,an increase in both vascular and RV gene expression of endothelin-1 and endothelin receptors was demonstrated.2027BNP B-type natriuretic peptide levels may increase in RV pressure-or volume-overload states such as PH,cor pulmonale,PE,and selected CHD.Elevated B-type natriuretic peptid

26、e levels also are associated with an increased risk of mortality in patients with idiopathic PAH.2728Cytokine activationActivation of cytokines may play an important role in patients with RV failure.In patients with selected forms of CHD and RV dysfunction,elevated levels of tumor necrosis factor an

27、d endotoxin were associated with more symptomatic disease(lower functional class or more edema).28Interactions between ventricular remodeling,neurohormonal and cytokine activation,and gene expression in the setting of RV failure.Vicious cycle of auto-aggravationManagementControl of trigerring factor

28、sSupportive treatment:Optimization of preloadImproving contractilityPulmonary vasodilatorsSpecific therapies addressing the cause of RVFTreatment of triggering factors(acute on chronic)ArrhytmiasInfectionsPulmonary embolismThyrod dysfunctionOptimization of preloadFrank-Starling relationship between

29、preload and stroke volume:preload dependance(A)and preload independance(B)Fluid therapyDiureticsFrequent volume overloadAt a point of Frank-Starling curve where there is no more reserve on contractilityVentricular interdependanceDiuretics to be consideredSometimes with continuous high dose infusionP

30、rospective,controlled,randomized,animal study 22 dogs underwent transient PA constriction(90mn)Dobutamine 5 and 10 g/kg/mn,norepinephrine 0.1 to 0.5 g/kg/mnA transient increase in PA pressure persistently worsens PA hemodynamics,RV contractility,RV-PA coupling,and cardiac output.Dobutamine restores

31、RV-PA coupling and cardiac output better than norepinephrine because of its more pronounced inotropic effectDobutamine1 adrenergic stimulation CI PVR at 5 g/kg/mnAt higher dose HR without subsequent in PVRExperimental models Dobutamine Norepinephrine to improve right-ventricular pulmonary artery cou

32、plingImproves CI,PVR and PaO2/FiO2 in combination with Inhaled nitric oxydeNorepinephrine1 and 1 adrenergic stimulationIncreases mPAP and PVRBut marked improvement in COUseful in combination with Dobutamine for hypotensive patientsCauses less tachycardia than other inotropesSecond choice after Dobutamine in normotensive patientsPulmonary vasodilators肺动脉血管扩张剂 目前临床上应用的血管扩张剂有：n n 前列环素n n 吸入NOn n 内皮素受体拮抗剂n n 5型磷酸二酯酶抑制剂n n Rho激酶抑制剂。Haddad F,MD;Circulation.2008;117:1717-1731Thank you for attention!