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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,患者于7天前开始无明显诱因出现发热，体温骤然升高在39摄氏度以上，最高达41摄氏度。持续数小时后能自行缓解，以夜间明显，并阵发性咳嗽，以干咳为主，无痰，无咯血，伴畏寒、盗汗，盗汗以午后及夜间明显，无寒战，无明显胸痛、胸闷，无恶心、呕吐，无关节疼痛，感活动后气促，经休息后能逐渐缓解，无夜间阵发性呼吸困难及端坐呼吸，遂就诊于广州新海医院，行胸片检查示右侧液气胸，经输液治疗（具体不详）症状好转不明显，为求进一步诊治
2、故来我院就诊，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。体重较前无明显变化。,既往史：患者于5年前开始多于活动后出现气促，经休息后能缓解，曾至本院诊断为“慢性血栓栓塞性肺动脉高压”，长期口服“华法林片 2.5mg”1次/日，未规则监测INR。否认“高血压”病史，否认“肝炎”“结核”等传染病史，无重大外伤史，无输血史，否认药物过敏史，预防接种史不详。个人史、婚育史、家族史无特殊。,既往史：,体格检查,T 39.9 P 130次/分 R 22次/分BP130/70mmHg发育正常，神志尚清，体查合作，急性重病容，半坐位。全身皮肤、巩膜无黄染，皮肤无出血点，皮肤弹性可。全身浅表淋巴
3、结无肿大。头颅五官大小无畸形，结膜无充血，双瞳孔等大等圆，对光反射灵敏。外耳道无流脓。鼻翼无煽动，口唇无发绀，咽无充血，扁桃体不大。颈软，颈静脉充盈，颈动脉搏动可，未闻及明显血管杂音。气管居中，甲状腺不大。胸廓对称，右侧语颤减弱，右上肺叩诊呈过清音，右下肺叩诊呈实音，左肺叩诊呈清音，右肺呼吸音较左侧低，双下肺可闻少许湿罗音，无胸膜摩擦音。腹平软，未见胃肠型及蠕动波，无压痛，无反跳痛，肝脾肋下未扪及，移动性浊音阴性，双侧肾区无叩击，肠鸣音4次/分。肛门外生殖器未见明显异常。脊柱四肢无畸形，双下肢无水肿，生理反射可，病理征未引出。,体格检查,专科检查：心前区无异常隆起，心尖搏动位于第5肋间左锁骨中
4、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。,47,49,50,56,57,辅助检查,右心导管检查（2010-06-24）示：PCWP 10 mmHg,mPAP 65mmHg,CO 3.2 L/min,肺动脉阻力 17.2 Wood,血常规,相关检查,ESR(7.13):67mm/h.NT-proBNP：2087pg/ml，结核抗体：IgG弱阳性。胸水常规：黄色浑浊，李凡它试验阳性，细胞总数1920*106/l。胸水生化：腺苷
5、脱氢酶53u/l，乳酸脱氢酶1260.7u/lD-二聚体 3.18mg/l血、胸水、骨髓培养均无菌生长，多次胸水病检未找到癌细胞，肥达氏反应，甲功三项，肌钙蛋白，血清肿瘤相关物质，C-12，PPD，ENA+狼疮全套+免疫全套，肾病全套，抗心磷脂抗体均未见明显异常。,血气,60,61,62,63,64,65,71,66,67,68,69,70,72,73,53,54,74,75,辅助检查,心脏彩超（2011-07-06）：床旁心脏彩超示：左房（32mm）左室（54mm）右房（48*55mm）右室（33mm，4CV52mm）主动脉直径30mm，肺动脉34mm，下腔静脉 14mm，EF 62%。右室
6、壁增厚（前壁10mm），三尖瓣患扩张，开放可，关闭裂隙。三尖瓣返流速4.50m/s。提示：肺动脉高压 右房、右室大。三尖瓣中度返流、二尖瓣轻度返流 心包少量积液（后心包5mm）,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：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、降肺动脉高压：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：华法林片 2.5mg 1次/日（6.30-7.4）；3.125mg 1次/日（7.4-
7、7.11）；3.438mg1次/日（7月4至今）,治疗方案,4.抗结核 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（7.1-7.20）共引流出约2500ml淡黄色液体。6、护肝降酶：还原性谷胱肝肽、肝泰乐7、强心：西地兰、多巴酚丁胺,38,急性右心衰,7月23日，患者下床解大便后突发呼吸困难，胸闷，濒死感，大汗淋漓查：Bp 136/78mmHg,R36次分 端坐呼吸，唇紫绀，双肺湿罗音无明显增加，心率163次/分，心律齐，P2A2，胸骨左缘3-4肋间可闻及舒张期奔马律。急查血气分析，电解质，D二聚体，ECG,76,77,41,处理,无创
8、呼吸机辅助呼吸静脉滴注法舒地尔静脉注射西地兰 0.4mg静脉注射呋塞米20mg静脉滴注多巴酚丁胺 5ug/kg/min,58,59,目前诊断,1、发热查因：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4、白塞氏病？5、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。,5,Effect of Ventricular Contraction,R
9、ight ventricular Contraction is sequential,starting with the inlet and ending at the infundibulum.Inward movement of the free wall(bellows effect)Contraction of the longitudinal fibers,which shortens the long axis.Traction of the free wall caused by contraction of the LV.(wringing effect)Shortening
10、of the RV is greater longitudinally than radially.In contrast to the LV,twisting and rotational movements do not contribute significantly to RV contraction.This leads to near complete emptying of the RVreduced RVEDPOptimal Venous return.,5,6,Right Ventricular PerfusionIn a right dominant system;Late
11、ral Wall-Marginal branches of RCAPosterior and Inferoseptal-PDAAnterior and Anteroseptal-Branches of LADRCA is relatively resistant to ischemia compared to the LV.Lower oxygen consumptionMore extensive collateral systemThe ability to increase its O2 extraction during increase demand.Normally proxima
12、l RCA flow occurs in both diastole and Systole.Unlike LCA flow to the LV.This is secondary to the transmural pressure generated during systole.,6,7,Aortic Pressure and Coronary Blood Flow,7,The response of the RV and LV to experimental increase in afterload.,Pathophysiology of failing RV,2008年美国Dana
13、 point 分类,18,Autonomic nervous system,In a dog model of 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 i
14、n the LV.In patients with PAH,elevated catecholamine levels were associated with higher pulmonary vascular resistance and lower cardiac index.,18,19,Renin-angiotensin-aldosterone system,RV pressure overload led to a loss of responsiveness to the inotropic effects of angiotensin II and to an uncoupli
15、ng of angiotensin 1 receptors to downstream signaling pathways.In patients with corpulmonale,activation of the renin-angiotensin-aldosterone system also may contribute to fluid retention and ventricular remodeling,19,20,Endothelin system,Endothelin system activation may be important feature of pulmo
16、nary vascular disease and right HF.In a monocrotaline-induced PAH rat model,an increase in both vascular and RV gene expression of endothelin-1 and endothelin receptors was demonstrated.,20,27,BNP,B-type natriuretic peptide levels may increase in RV pressure-or volume-overload states such as PH,cor
17、pulmonale,PE,and selected CHD.Elevated B-type natriuretic peptide levels also are associated with an increased risk of mortality in patients with idiopathic PAH.,27,28,Cytokine activation,Activation of cytokines may play an important role in patients with RV failure.In patients with selected forms o
18、f CHD and RV dysfunction,elevated levels of tumor necrosis factor and endotoxin were associated with more symptomatic disease(lower functional class or more edema).,28,Interactions between ventricular remodeling,neurohormonal and cytokine activation,and gene expression in the setting of RV failure.,
19、Vicious cycle of auto-aggravation,Management,Control of trigerring factorsSupportive treatment:Optimization of preloadImproving contractilityPulmonary vasodilatorsSpecific therapies addressing the cause of RVF,Treatment of triggering factors(acute on chronic),ArrhytmiasInfectionsPulmonary embolismTh
20、yrod dysfunction,Optimization of preload,Frank-Starling relationship between preload and stroke volume:preload dependance(A)and preload independance(B),Fluid therapy,Diuretics,Frequent volume overloadAt a point of Frank-Starling curve where there is no more reserve on contractilityVentricular interd
21、ependanceDiuretics to be consideredSometimes with continuous high dose infusion,Prospective,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 hem
22、odynamics,RV contractility,RV-PA coupling,and cardiac output.Dobutamine restores RV-PA coupling and cardiac output better than norepinephrine because of its more pronounced inotropic effect,Dobutamine,1 adrenergic stimulation CI PVR at 5 g/kg/mnAt higher dose HR without subsequent in PVRExperimental
23、 models Dobutamine Norepinephrine to improve right-ventricular pulmonary artery couplingImproves CI,PVR and PaO2/FiO2 in combination with Inhaled nitric oxyde,Norepinephrine,1 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 patients,Pulmonary vasodilators,肺动脉血管扩张剂,目前临床上应用的血管扩张剂有：前列环素 吸入NO 内皮素受体拮抗剂 5型磷酸二酯酶抑制剂 Rho激酶抑制剂。,Haddad F,MD;Circulation.2008;117:1717-1731,Thank you for attention!,
