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1、Valvular Heart Disease,心脏瓣膜病,心脏瓣膜病 是指心瓣膜及瓣下装置由于炎症、变性、粘连、缺血性坏死、创伤、老化或钙质沉着及先天性发育异常等原因，使单个或多个瓣膜发生急性或慢性的狭窄或关闭不全，导致前向血流障碍和/或返流的一组疾病。临床上最常受累的为二尖瓣，其次为主动脉瓣。风湿性心脏病简称风心病，仍是我国主要的心脏病，是风湿性炎症过程所致瓣叶损害。根据人群发病率调查已有下降趋势。,Mitral valve disease,二尖瓣疾病,Mitral stenosis(MS),二尖瓣狭窄,Etiology and Pathology（病因和病理）,Rheumatic hear
2、t disease(风湿性心脏病)Congenital malformation(先天性畸形)Senile mitral annulus and subvalvular calcification(老年人二尖瓣环及环下区钙化),Pathophysiology(病理生理),The cross-sectional area of the mitral valve orifice(瓣环口面积）Normal adults 4-6cmMild MS 2cmModerate MS 1.5cmSevere MS 1.0cm,The effect on LA and cardiac output of MS
3、Mild MS：LA压力轻度升高，心排血量正常Severe MS：跨瓣压差增大(20mmHg)LA压力升高（25mmHg)；休息时心排血量正常或减少The effect on the pulmonary circulation and respiration of elevated left atrium pressure,LA PVP、PCP lung congestion/pulmonary edema pulmonary artery intima hyperplasia and thickening（肺动脉内膜增生肥厚）PAP（肺动脉压升高）Right heart failure（右心
4、衰竭）,Remarks(备注),PAP：肺动脉压PCP：肺毛细血管压PVP肺静脉压,Clinical situation(临床表现),一、Symptom(中度狭窄始出现症状)Exertion dyspnea(劳力性呼吸困难)Hemoptysis(咯血)支气管静脉压破裂出血 肺梗死 肺水肿,Hoarseness（声嘶）Cough(咳嗽）LA增大压迫左主支气管，支气粘膜淤血水肿，易致感染 扩大的LA、肺A压迫喉返N,Clinical situation,二、Physical Sign(体征)Mitral facies(二尖瓣面容)S1，可闻及OS(开瓣音)Cardiac apex DM(心尖区舒张
5、期杂音），often accompanying diastolic thrill(舒张震颤）RV，P2 excessive(亢进)，Relative SM of TI(相对性三尖瓣关闭不全收缩期杂音),Laboratory examination(实验室检查),XRay二尖瓣型心：左房右室大，主动脉结缩小，肺动脉扩张，肺淤血ECG P0.12s,RV1,电轴右偏，心房纤颤，粗f波 Echocardiogram(超声心动图)：是确诊、定量MS的可靠方法,M型：二尖瓣前后叶同向运动 二维：狭窄瓣膜形态结构，瓣口面积，房室大小 连续多普勒：测定血流速度、跨瓣压差Cardiac catheteriza
6、tion(心导管术)测定肺毛细血管压和左室压，确定跨瓣压差，明确狭窄程度,Diagnosis and Differential diagnosis(诊断和鉴别),Diagnosis心尖区DMLA扩大及实验室检查可诊断，超声有确诊价值Differential diagnosis二尖瓣口血流增加Austin Flint杂音左房粘液瘤：随体位改变的DM,Complication（并发症）,一、Atrial fibrillation(心房纤颤)见于5%以上的患者；房颤使心排量下降20%，常是体力活动明显受限的开始二、Acute pulmonary edema(急性肺水肿)为重度MS最严重的并发症及致死
7、原因三、Embolism(栓塞)80%有房颤、大左房（D55mm)；2/3为脑栓塞，也可有周围及内脏栓塞,Complication（并发症）,四、Right heart failure(右心衰竭)五、Pulmonary infection(肺部感染),Prognosis(预后),无症状者可存活多年，一旦有症状至致残平均7.4年死亡原因多为上述并发症,Therapy(治疗),General therapy(一般治疗)：预防风湿热及感染性心内膜炎Hemoptysis(咯血)：减低肺静脉压力Atrial fibrillation：快速心室率时应用洋地黄Right heart failure：以利尿为
8、主,Therapy(治疗),Acute pulmonary edema：处理同急性左心衰；注意二尖瓣狭窄时用正性肌力药物不好，仅当房颤快速心室率时应用Mechanic therapeutics(机械治疗)MS：经皮球囊二尖前瓣成型术；外科手术,Mitral incompetence:MI,二尖瓣关闭不全,Etiology and Pathology（病因病理）,During systole,competence(关闭)of mitral valve depend on the integrity of mitral structure and function(including leftle
9、ts of valve,mitral annulus(瓣环),tendinous cords(腱索),papillary muscle(乳头肌)and LV.Every abnormality may lead to MI.,一、Chronic MI,Rheumatic heart disease:The leftlets of mitral valve fibrose,thicken,shorten and often accompany MS and aortic valve diseaseMitral valve prolapse(二尖瓣脱垂)CHD：Chronic ischemia(缺
10、血)or infarction(梗死)lead to fibrosis and functional disorder of papillary muscle,一、Chronic MI,Calcification of mitral ring and subvalvular(二尖瓣环及环下区钙化)Infective endocsrditis Rupture of chordae tendineae(unknown cause)LV enlarged significantly(左室显著扩大)Else,二、Acute MI,Rupture of chordae tendineae(腱索断裂)En
11、docarditis leads to the leftlets of valve destruction(心内膜炎致瓣叶毁损)Acute myocardial infarction(急性心肌梗死)Trauma results in rupture of the mitral valve component(创伤使二尖瓣器破裂)Rupture of prosthetic valve(人工瓣膜开裂),Pathophysiology,MI LVEDV LV hypertrophy LVEDP,LALV failure Pulmonary congestion PAP Right heart fai
12、lure,Clinical situation(临床表现),一、Symptom轻度MI可终身无症状，严重MI心排血量减少，感乏力、呼吸困难二、Physical SignHeaving apex impulse(抬举性心尖搏动)Cardiac sound：S1（重度MI），S2分裂，闻及S3Cardiac murmur：从S1后立即开始，与S2同时终止的SM，可伴收缩期震颤，向左腋、左肩胛下区传导；乳头肌功能不全、腱索断裂的杂音似海鸥鸣叫样,Laboratory examination,XRayECGEchocardiogram二维超声：可显示二尖瓣的形态结构，提供心室大小，明确病因彩超连续多普
13、勒：可用于二尖瓣心房侧探及收缩期射流，半定量返流量,Diagnosis and Differential diagnosis,心尖区SM心房、心室增大，诊断MI可成立，确诊有赖于超声心动图应与以下情况相鉴别：Tricuspid incompetence（三尖瓣关闭不全）：胸左缘4、5肋间SM，可传至心尖区，杂音吸气时增强，伴颈静脉收缩期搏动，RVVSD（室间隔缺损）,Systolic ejection murmur in left border of sternum生理性杂音功能性杂音主、肺动脉根部扩张左或右室流出道梗阻,Atrial fibrillation Infective endoca
14、rditis Embolism Heart failure,Complication,Prognosis,急性严重返流者，若不及时手术，极难存活慢性MI无症状期长，一旦发生左心衰竭，预后不良,Therapy,Medical therapy(内科治疗)Prevent endocarditis and rheumatic feverPatients who are asymptomatic and having normal cardiac function neednt therapy but regular follow-up(定期随访).Complication are cured in p
15、atients with complication.,Surgical treatment Prosthetic valve replacement为主要手术方法，趋向早期手术有症状者应在LVEF0.5，平均肺动脉压 20mmHg之前手术产生左室功能不全、LVEF 0.30.5、年龄55岁、LVEDD80mm，已不置换瓣 Valvuloplasty of mitral valve(二尖瓣整复术)优点：不需长期抗凝，LV功能恢复较好,Aortic Valve Disease,主动脉瓣疾病,Aortic stenosis(AS),主动脉瓣狭窄,Etiology and Pathology,Rheu
16、matic heart disease：风湿性炎症所致瓣膜交界处融合、瓣叶纤维化、钙化，引起瓣叶狭窄畸形，多伴AI及二尖瓣损害Congenital bicuspid valve(先天性二叶瓣)Senile calcific(degenerative)AS(退行性老年钙化性主动脉瓣狭窄)：65岁老年人AS的常见原因，瓣叶主动脉面钙化结节限制瓣叶活动,Pathophysiology,The cross-sectional area of the aortic valve orifice(瓣环口面积）Normal adult 3.0cmThe area 1.0cm,LVSP,transvalve p
17、ressure gradient manifest(跨瓣压差明显),ASAfter loadingLV hypertrophyLVEDP,LA amplification PAP PCP Lung congestion and edema,Myocardial ischemia Myocardial contractility Heart failure,Clinical situation,SymptomAS Triple syndrom(AS三联症)Dyspnoea(呼吸困难):Lung congestionAngina(心绞痛)Causing by Synocope(晕厥)cardiac
18、 output,Clinical situation,Physical signCardiac sound:S1 is normal,S2 is paradoxical splitting(逆分裂),S4 may be heardCardiac murmur:：SM is heard on the second intercostal space(肋间)of right border of sternum,and radiate to cervical part(颈部),left inferior border of sternum and cardiac apex accompanying
19、thrill Cardiac dilatation(心脏扩大)，SBP and pulse pressure decrease,Laboratory examination,X-Ray：心影可正常或稍大，晚期见肺淤血ECG：可有左室肥厚劳累征，及各种心律失常Echocardiogram：为确定、定量AS的重要方法Cardiac catheterization：可根据左室-主动脉压差计算瓣口面积,Diagnosis and Differential diagnosis,Diagnosis典型的收缩期杂音，易于诊断；多瓣膜病变提示风心病单纯AS：根据年龄，应考虑单叶瓣、二叶瓣膜及老年退行性变，确诊
20、有赖于超声心动图Differential diagnosisAS应与左室流出道梗阻性疾病鉴别,Complication,Arrhythmia：10%可发生房颤、室性心律失常、房室传导阻滞，可至猝死、晕厥Infective endocarditisEmbolismHeart failure：发生左心衰后，病情迅速恶化Gastrointestinal hemorrhage(胃肠道出血)：15-20%胃肠道血管发育不良,Prognosis,一旦出现症状，平均寿命仅三年。死亡原因为：左心衰、猝死人工瓣膜置换术后，远期存活率优于内科治疗,Medicine therapy,Principal object
21、ive：确定狭窄发生度、观察病情进展，争取手术机会择期手术Methods预防感染性心内膜炎、风湿热AS不能耐受房颤，一旦出现即时转复处理心衰,PBAP:Percutaneous balloon aortic valvuloplasty(经皮球囊主动脉瓣成形术),适用于高龄患者、不宜换瓣及妊娠等情况，作为姑息治疗,Aortic incompetence,主动脉瓣关闭不全,Etiology and pathology,一、Chronic AI（一）：Aortic valve diseaseRheumatic heart disease:占2/3，由于瓣叶纤维化、增厚缩短，影响闭合，常合并AS及二尖
22、瓣损害Infective endocarditis:为单纯AI的常见病因Congenital malformation:先天性二叶瓣、室间隔缺损伴一叶瓣脱垂、先天性主动脉瓣穿孔Aortic valve mucinous degeneration(主动脉瓣粘液样变性):可致主动脉瓣脱垂,Etiology and pathology,（二）：Aorta root dilatation：瓣环扩大，瓣叶关闭不全Syphilitic aortitis(梅毒性主动脉炎)：主动脉炎致主动脉根部扩张,30%呈AIMarfars syndrome：为遗传性结缔组织病，升主动脉呈梭形扩张，常伴二尖瓣脱垂Sever
23、e hypertension or atherosclerosisIdiopathic dilatation of ascending aorta(特发性升主动脉扩张),Etiology and pathology,二、Acute AIInfective endocarditisTraumaDissection of aorta(主动脉夹层分离):夹层血肿使主动脉瓣环扩大，或瓣叶、瓣环被夹层血肿撕裂，多见于马凡氏综合征、高血压或妊娠 Rupture of prosthetic valve(人工瓣膜破裂),Pathophysiology,Chronic aortic regurgitationL
24、VEDVSBP of LV after many years DBP of aortaangina LVEDP Pulse pressure LV dilating and hypertrophy Peripheral vascular sign LAP、PVP Left heart failure,Remarks(备注),SBP：收缩压DBP：舒张压PVP：肺静脉压LAP：左房压 LVEDP：左室舒张末压Peripheral vascular sign：周围血管征,Clinical situation,Physical Sign:SP,DP,PP 1.Peripheral vascular
25、signWater-hammer pulse(水冲脉)Pistol shot sound(枪击音)De musset signs(点头运动）Duroziez signs(杜氏双重杂音)Capillary pulse(毛细血管搏动)Carotid artery pulse(颈动脉搏动),Clinical situation,Physical Sign2.Apical impluse displaced to left and down(心尖搏动向左下移位)3.Cardiac sound：S1、S2减弱，可闻及S34.Cardiac murmur：舒张早期杂音，吹风性，呼气末期易闻及，于左胸第三肋
26、间明显。重度返流者，心尖区可闻及舒张早期隆隆样杂音（Austin Flint杂音）,Laboratory examination,X-Ray：急性者心脏大小正常；有肺淤血、肺水肿者，心胸比值增大，LV、LA增大，升主动脉扩张及左心衰、肺淤血ECG：LV肥厚劳损Echocardiogram,Diagnosis and Differential diagnosis,Diagnosis 典型杂音周围血管征Differential diagnosis与Graham Stell 杂音的区别：见于严重肺动脉高压及肺动脉扩张的肺动脉瓣关闭不全Austin Flint 杂音与MS杂音鉴别,Complicati
27、on,Infective endocarditisVentricular arrhythmia(室性心律失常)Heart failure,Therapy,对于急性AI，外科治疗为根本措施Medicine therapy mainly include：预防感染性心内膜炎、风湿热无症状的轻、中度AI者定期随访，限制体力活动心衰时强心、利尿及血管扩张药物、对症治疗,Therapy,Surgical treatment：严重AI，需要换瓣治疗Indications for surgery：有症状的左室功能不全者虽无症状，但已有左室功能不全，左室收缩末期容积增加，EF下降Surgical contrai
28、ndiction：LVEF0.15-0.20，LVEDD80mm，或 LVEDVI300ml/m,Multivalvular heart disease,多瓣膜病,Etiology,一种疾病同时损害几个瓣膜一瓣损害相继引起近端瓣膜功能受累不同疾病分别导致不同瓣膜损害,Pathophysiology,严重损害掩盖轻的损害近端损害影响较显著，而掩盖轻的损害总的血液动力学明显异常,Examples of common multivalvular heart disease,MS+AI：MS导致心排血量下降，使LV扩大延缓，周围血管缺如MSAS：严重MS常掩盖AS的表现ASMI：为危险的多瓣膜病，相对较少见。AS增加左室后负荷使MI返流加重，前向心搏量减少较二者单独存在时明显,Therapy,主要是择期手术,
