湘雅儿科精品

先天性心脏病 Congenital Heart Disease (CHD),Dept. Pediatric of 2nd Xiangya Hospital of CSU,Wang Cheng,中南大学儿科学教研室,第十三章,概述 Overview,CHD 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,By 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。,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,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,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,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),PS,VSD,TOF,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),室间隔缺损 Ventricular Septal Defect （VSD）,室间隔缺损 血流动力学示意图 Hemodynamics Figure of VSD,PV,SVC,RA,PA,IVC,RV,PV,LA,AO,LV,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,VSDmurmur,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),VSD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 -/, 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,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 endocarditis,房间隔缺损 Atrial Septal Defect （ASD）,PV,LA,PA,LV,PV,SVC,RA,AO,IVC,RV,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 ASD,ASDmurmur,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 ASD,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 -/ ejection SM is heard best at the LSB in the 2nd intercostal space，no thrill,P2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 area,ASD临床表现Clinical findings of ASD,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,动脉导管未闭 Patent ductus arteriosus(PDA）,PV,SVC,RA,PA,IVC,RV,PDA,PV,LA,AO,LV,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,PDAmurmur,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 PDA,症状 Symptoms,与VSD及ASD相同 Symptoms of PDA are similar with that of VSD and ASD,体征 Signs,LSB2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,PDA堵闭前心音图,S1,S2,PDA堵闭后心音图,S1,S2,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,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 slowly,法洛四联症Tetralogy of Fallot （TOF）,PV,SVC,RA,PA,IVC,RV,PV,LA,AO,LV,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 TOF,TOFmurmur,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,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 TOF,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 -/, 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 TOF,杵状指 Clubbing of fingers,杵状指趾 Clubbing of fingers and toes,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 TOF,X Ray of TOF,X线检查右室大、心尖上翘呈靴形，肺动脉段凹陷，肺野清晰,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 angiocardiography,