动脉导管未闭

nullnull先天性心脏病 Congenital Heart Disease (CHD)陈小荣包头医学院第二附属医院儿科第十三章null概述 OverviewCHD is defined as an abnormality in circulatory structure or function that is present at birth, even if it is discovered much later CHD is present in 0.687% in China 先天性心脏病是胎儿时期心脏血管发育异常而导致的先天性畸形出生后1年内先天性心血管发育畸形出现率占活产婴儿的6.87‰nullBy the progression of all kinds of inspection techniques and interventional therapy, the prognosis of CHD has taken on a new look. 概述 Overview由于各种心血管检查技术（特别是彩色多普勒超声心动图）的应用，深低温麻醉和体外循环下心内直视手术的发展，先心病介入性治疗进展，临床上对先心病的诊断和治疗发生了很大变化 ，预后大为改观。null目的要求 Objective and Request ●掌握室间隔缺损、房间隔缺损、动脉导管未闭、法洛四联症的血流动力学、临床表现及常见并发症 To master the hemodynamics and clinical situation and the diagnosis of common complications in VSD, ASD, PDA and TOF 熟悉先天性心脏病的病因、分类、预防及治疗原则 Familiar with the etiology and classification of CHD●null病因 Etiology ●遗传因素(内在因素) 与基因突变、染色体畸变有关 Genetic factor (internal factor) CHD result from gene mutation or chromosome aberration●环境因素(外界因素) 与宫内病毒感染有关 Environmental factor (external factor) CHD is mainly correlation to the intrauterine infection null预防 Prevention 加强孕妇保健 The health protection of pregnant woman should be enhanced避免接触药物、辐射等高危因素High risk factors should be avoided 妊娠早期适量补充叶酸 Suit dosage Folic Acid should be filled up in early pregnancy stage null分类Classification ●左向右分流型(潜在青紫型) left-to-right shunts (noncyanosis) ●右向左分流型(青紫型) right-to-left shunts (cyanosis) ●无分流型(无青紫型) non shunts (noncyanosis) PSVSDTOFnull临床常见的先心病 Common CHD in Clinic●室间隔缺损 Ventricular septal defect (VSD) ●房间隔缺损 Atrial septal defect (ASD)●动脉导管未闭 Patent ductus arteriosus (PDA)●法洛四联症 Tetralogy of Fallot (TOF)null室间隔缺损 Ventricular Septal Defect （VSD）null室间隔缺损 Ventricular Septal Defect （VSD）室间隔缺损 Ventricular Septal Defect （VSD） 室间隔缺损 Ventricular Septal Defect （VSD） 由胚胎期室间隔(流入道,小梁部和流出 道)发育不全所致,是最常见的先天性心脏病,约占我国先心病的50%.最多见为膜周部缺损.病理生理病理生理分流量多少取决于缺损面积.心室间压差及肺小动脉,大致可分为三种类型: 小型室缺:缺损直径小于5mm或缺损面积<0.5cm/m体表面积.血流动力学变化不大 中型室缺:缺损直径5—15mm或缺损面积0.5—1.0cm/m体表面积分流量较多,但 因肺血管床有很丰富的后备容受量,肺动脉收缩压和肺血管阻力可在较长时期不增高.:缺损病理生理病理生理大型室间隔缺损:缺损直径大于15mm或缺损面积>1.0cm/m体表面积.随着肺血管病变进行系性发展则渐变为不可逆的阻力性肺动脉高压. 当右室收缩压超过左室收缩压,左向右分流逆转为双向分流或右向左分流,出现紫绀,即艾森曼格(Eiseenmenger)综合症.null室间隔缺损 血流动力学示意图 Hemodynamics Figure of VSDPVSVCRAPAIVCRVPVLAAOLV肺静脉上腔静脉右心房肺动脉下腔静脉右心室肺静脉左心房主动脉左心室VSD murmurnull室间隔缺损血流动力学变化 Hemodynamics of VSD小室缺 可无血流动力学变化Small VSD no hemodynamics changes 大室缺 大量左向右分流 Large VSD Large left-to-right shunts动力型肺动脉高压 Dynamic PH梗阻型肺动脉高压 Obstructed PH艾森门格综合征 Eisenmenger Syndrome肺动脉高压Pulmonary Hypertension (PH)nullVSD临床表现Clinical findings of VSD●症状 Symptoms 反复呼吸道感染，生长发育落后、乏力、气短、声嘶 frequent respiratory infections, grow slowly, very poor weight, dyspnea, exercise intolerance, fatigue, congestive heart failure ●体征 Signs LSB3-4可闻3-4/6级粗糙全收缩期吹风样杂音，向周围广泛传导，伴震颤 A grade Ⅲ-Ⅳ/Ⅵ, medium-to-high pitched , harsh pansystolic murmur at the left sternal border (LSB) in the 3rd and 4th intercostal spaces 肺动脉瓣第二音亢进 The pulmonary component of S2 is accentuation in intensity 合并主动脉瓣关闭不全时可闻舒张期杂音 A diastolic murmur indicate VSD combine with aortic regurgitation null室间隔缺损并发症Complication of VSD●支气管肺炎 Bronchopneumonia ●充血性心力衰竭 Congestive heart failure ●肺水肿 Pulmonary edema ●感染性心内膜炎 Infective endocarditisnull房间隔缺损 Atrial Septal Defect （ASD）房间隔缺损房间隔缺损房间隔缺损(atrial septal defect)是小儿时期常见的先天性心脏病,该病的发病率约为活产婴儿的1/1500,占先天性心脏病发病总数的5%--10%.女性较多见,男女性别比例为1:2.病理解剖病理解剖原发孔型房间隔缺损 部分型心内膜垫缺损 继发孔型房间隔缺损 中央型 静脉窦型房间隔缺损 上腔型和下腔型 冠状静脉窦型 又称冠状静脉窦型缺损 无顶冠状窦nullPVLAPALVPVSVCRAAOIVCRV肺静脉肺静脉上腔静脉右心房主动脉下腔静脉右心室左心房肺动脉左心室房间隔缺损 血流动力学示意图 Hemodynamics Figure of ASDASD murmurnull上、下腔静脉血 肺静脉 右心房（扩大） 左心房 右心室 （增大） 左心室（血量 减少） 肺血流量明显增加（肺充血） 肺小动脉痉挛、增厚 体循环供血不足 右向左分流 （消瘦、乏力、心悸、气短等） 艾森门格综合征 (少数病人晚期）ASD房间隔缺损 血流动力学变化 Hemodynamics of ASDnull●症状 Symptoms 同室缺相似 Symptoms of ASD are similar with that of VSD ●体征 Signs LSB2-3可闻1-3/6级柔和收缩期喷射音，无震颤 A grade Ⅰ-Ⅲ/Ⅵ ejection SM is heard best at the LSB in the 2nd intercostal space，no thrillP2固定分裂 S2 at the pulmonary area is widely split and often fixed 肺动脉瓣第二音亢进 The pulmonary component of S2 is accentuation in intensity 三尖瓣区可闻舒张期杂音 A mid-diastolic murmur can often be heard in tricuspid areaASD临床表现Clinical findings of ASDnull房间隔缺损并发症Complication of ASD●支气管肺炎 Bronchopneumonia ●充血性心力衰竭 Congestive heart failure ●感染性心内膜炎 Infective endocarditis辅助检查辅助检查X线表现 透视下可见肺动脉总干及分支随心脏搏动而一明一暗的”肺门舞蹈”征,心影略呈梨形.原发孔型房缺伴二尖瓣裂缺者,左心房及左心室增大.治疗治疗小于3mm的房间隔缺损多在3个月内自然闭合,大于8mm的房缺一般不会自然闭合. 反复呼吸道感染,发生心力衰竭或合并肺动脉高压者应尽早手术.房间隔缺损也可通过介入性 心导管术,应用双面蘑菇伞关闭缺损.null动脉导管未闭 Patent ductus arteriosus (PDA）动脉导管未闭动脉导管未闭为小儿先天性心脏病常见类型之一,占先天性心脏病发病总数的15%.胎儿期动脉导管被动开放是血液循环的重要通道,出生后,大约15小时既发生功能性关闭,80%在生后3个月解剖性关闭.到一年,在解剖学上应完全关闭.若持续开放,并产生病理,生理改变,即称动脉导管未闭.病理分型病理分型管型:导管长度多在1cm左右,直径粗细不等 漏斗型:长度与管型相似,但其近主动脉端粗大, 向肺动脉端逐渐变窄; 窗型:肺动脉与主动脉紧贴,两者之间为一孔道, 直径往往较大.nullPVSVCRAPAIVCRVPDAPVLAAOLV肺静脉肺静脉上腔静脉下腔静脉右心房主动脉肺动脉右心室左心房左心室动脉导管未闭 血流动力学示意图 Hemodynamics Figure of PDA动脉导管PDA murmurnull右心室血流 体循环 舒张压 肺动脉 主动脉 供血减少 脉压增宽 肺血流量 肺动脉高压 艾森门格综合征 左房、左室扩大 （差异性紫绀） （右心室肥大） PDA动脉导管未闭 血流动力学变化 Hemodynamics of PDAnull●症状 Symptoms与VSD及ASD相同 Symptoms of PDA are similar with that of VSD and ASD ●体征 SignsLSB2闻及粗糙、响亮的连续性机器样杂音，占据整个收缩期及舒张期 A characteristic thrill and continuous and rough “machinery” murmur with a late systolic accentuation 心尖区可闻及舒张中期隆隆样杂音 A diastolic flow murmur is often heard at the apex 周围血管征 Peripheral vascular sigh 下半身青紫(差异性紫绀)和杵状趾 Differential cyanosis and clubbing PDA临床表现Clinical findings of PDA差异性紫绀差异性紫绀当肺动脉压力超过主动脉压时,左向右分流明显减少或停止,产生肺动脉血流逆向分流入主动脉,患儿呈现差异性紫绀(differential cyanosis),下半身青紫,左上肢有轻度青紫,右上肢正常.nullPDA堵闭前心音图S1S2nullPDA堵闭后心音图S1S2null动脉导管未闭并发症Complication of PDA●支气管肺炎 Bronchopneumonia ●感染性心内膜炎 Infective endocarditis ●充血性心力衰竭 Congestive heart failure 感染性动脉炎 少见的并发症有肺动脉和动脉导管瘤样扩张,动脉导管钙化及血栓形成.治疗治疗早产儿动脉导管未闭的处理视分流大小,呼吸窘迫综合症情况而定.生后一周内使用消炎痛治疗,仍有10%的病人需手术治疗.采用介入疗法选择弹簧圈,蘑菇伞等关闭动脉导管. 但在有些病例中,如完全性大血管转位,肺动脉闭锁,三尖瓣闭锁,,严重的肺动脉狭窄中,动脉导管为依赖性者,对维持患婴生命致关重要,此时应该应用前列腺素E以维持动脉导管开放.null左向右分流先心病共同临床特点Common features of left-to-right shunting CHD●一般情况下无青紫 In general there are noncyanosis ●心前区有粗糙的收缩期杂音 A rough systolic murmur is heard in precordium ●肺循环血量多，易患肺炎 Pulmonary flow increase and easy to suffer from bronchopneumonia ●体循环血量少，影响生长发育 Systemic flow decrease and the patient grow slowlynull法洛四联症 Tetralogy of Fallot （TOF）法洛四联症法洛四联症法洛四联症(tetralogy of Fallot, TOF)是婴儿期后最常见的青紫型先天性心脏病,约占所有先天性心脏病的10%.1888年法国医生Etienne Fallot详细描述了该病的病理改变及临床表现,故而得名. 法洛四联症四种解剖畸形 The four malformations of TOF 法洛四联症四种解剖畸形 The four malformations of TOF ● 右室流出道梗阻 ●室间隔缺损 Ventricular septal defect ●主动脉骑跨 Overriding of the aorta ●右心室肥厚 Right ventricular hypertrophy 以上四种畸形中仅室间隔缺损 及右心室流出道狭窄是必须存在,室间隔缺损必须足够大使左右心室的压力相等;右心室流出道狭窄是决定患儿的病理生理,病情严重程度及预后的主要原因.而且,狭窄可随时间推移逐渐加重.nullPVSVCRAPAIVCRVPVLAAOLV肺静脉肺静脉上腔静脉右心房肺动脉下腔静脉右心室左心房主动脉左心室法洛四联症 血流动力学示意图 Hemodynamics Figure of TOFTOF murmurnull法洛四联症四种解剖畸形 The four malformations of TOF●肺动脉狭窄Obstruction to right ventricular outflow ●室间隔缺损 Ventricular septal defect ●主动脉骑跨 Overriding of the aorta ●右心室肥厚 Right ventricular hypertrophy null右 心室（肥厚） 左心室 肺动脉狭窄 主动脉 （血流量 、扩张） 肺血流量减少（肺野清晰） 混合血进入循环 （青紫、发育落后、乏力 血氧合不足 （杵状指趾等） 蹲踞、阵发性昏厥）分流分流法洛四联症 血流动力学变化 Hemodynamics of TOFnull ●青紫：本病最突出的症状 Cyanosis is the main symptoms ●气促和缺氧发作 Dyspnea and hypoxemic spells ●蹲踞症状 Squatting posture ●心脏体征 LSB2-4闻及 2-4/6级收缩期喷射性杂音 There is a grade Ⅱ-Ⅳ/Ⅵ, rough, ejection-type SM that is maximal at the LSB in the 2nd to 3rd intercostal space and that radiates well to the back●杵状指趾 Clubbing of the terminal digits TOF临床表现Clinical findings of TOFnull杵状指 Clubbing of fingers null杵状指趾 Clubbing of fingers and toesnull●脑血栓 Cerebral embolism ●脑脓肿Cerebral abscess ●感染性心内膜炎Infective endocarditis法洛四联症并发症Complication of TOFnullX Ray of TOFX线检查 右室大、心尖上翘呈靴形，肺动脉段凹陷，肺野清晰治疗治疗一般护理 平时应经常饮水,预防感染,及时补液,防治脱水和并发症. 缺氧发作的治疗 发作轻者使其取胸膝位即可缓解,重者应立即吸氧,给予新福林 每次0.05mg/kg静注,或心得安每次0.1mg/kg.必要时也可皮下注射吗啡每次0.1-------0.2mg/kg,纠正酸中毒,给予5%碳酸氢钠1.5—5.0ml/kg静注,经常有缺氧发作者,可口服心得安1-3mg/(kg.d). 外科治疗 null先天性心脏病的诊断Diagnosis of CHD●病史History ●体查Physical examination ●心电图Electrocardiogram ●心脏X线检查Chest X-ray ●超声心动图Echocardiogram ●心导管检查和心血管造影Cardiac catheterization and angiocardiographynull临床上有诊断意义的心脏杂音Clinical significance heart murmur●全收缩期杂音 Pansystolic murmur ●收缩晚期杂音 Late systolic murmur ●舒张期杂音 Diastolic murmur ●连续性杂音 Continuous murmur ●强度≥3/6级的杂音Grade ≥ 3/6 murmurnull先天性心脏病的治疗Treatment of CHD ●内科治疗 Medical management 治疗心衰，良好护理 Anticongestive heart failure measures, good nursing ●外科治疗 Operation treatment开胸心脏修补手术 Repair the defect through transthoracic cardiac surgery operation ●介入治疗Interventional treatment 利用堵闭器材导管关闭继发孔ASD和PDA Catheterization closure with an interventional device is generally recommended for ostium secundum ASD and PDA nullASD介入治疗Interventional treatment of ASDASD闭合器nullPDA介入治疗Interventional treatment of PDAPDA闭合器null