儿科先天性心脏病课件
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病因和预防 Etiology and Prevention
虽然引起先心病的原因尚未完全明确，但加强孕妇 的保健，特别是在妊娠早期适量补充叶酸，积极预 防风疹、流感等病毒性疾病以及避免与发病有关的 因素接触，对预防先心病具有积极意义
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
房间隔缺损
Atrial Septal Defect，ASD
主要内容
发病率及自然闭合率 病理解剖 病理生理及血液动力学改变 临床表现 特殊检查
心电图 X光胸片 超声心动图 心导管及造影
并发症 治疗
百度文库
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
病理生理及血液动力学改变
Pathophysiology
分流量大小与下列因素有关：
缺损口的大小 左右心室的顺应性 肺循环的阻力
右心房 ASD分流 左心房
血流量增加
血容量减少
右心室 扩大、肥大
左心室 血容量减少
肺动脉 扩张
主动脉 血容量减少
肺动脉循环 充血
体循环 供血不足
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
病因和预防 Etiology and Prevention
在胎儿心脏发育阶段，若有任何因素影响了心脏胚 胎发育，使心脏某一部分发育停顿或异常，即可造 成先天性畸形 这类有关因素很多，大致可分为内在和外来的两类， 以后者为多见
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
发病率及自然闭合率
Incidence and the rate of nature closure
房间隔缺损（ASD)是常见的先心病之一，其发生率 约占先心病的20-30％，其中80％左右为继发孔ASD 继发孔ASD自然闭合率多在1岁之内，闭合率为39%， 1岁以后可能性很小
胚胎第 5 周开始
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
法洛四联症
Tetralogy of Fallot,TOF
房间隔缺损
Atrial Septal Defect
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
概述
先心病是胎儿时期心脏血管发育异常而致的畸形， 是小儿最常见的心脏病 心脏胚胎发育的关键时期是在胚胎的第2～8周， 先天性心脏畸形的形成主要就在这一时期 发病率为7～8/1000 近半个世纪来，由于医学的发展，许多常见的先心 病，甚至复杂的先心病得到准确诊断，大多数可以 得到彻底根治
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
先心病分类 按血液动力学改变分为
无分流型(无青紫型) 即心脏左、右两侧或动、静 脉之间无异常通路或分流，如肺动脉狭窄和主动脉 缩窄等
今天三节课的主要内容
房间隔缺损
Atrial Septal Defect，ASD
室间隔缺损
Ventricular Septal Defect，VSD
动脉导管未闭
Patent Ductus Arteriosus，PDA
病因和预防 Etiology and Prevention
内在因素主要与遗传有关，特别是染色体畸变。 近年来的研究已经证明
房室间隔缺损和动脉干畸形等与第21号染色体 长臂某些区带的过度复制或缺失有关 另外，第7、12、15和22号染色体上也有与形成 心血管畸形有关的基因
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
先心病分类 按血液动力学改变分为
左向右分流型(潜在青紫型) 正常情况下由于体循 环压力高于肺循环，故平时血液从左向右分流而不 出现青紫。当哭闹、屏气或剧烈活动等情况致使肺 动脉或右心压力增高并超过左心压力时，可使血液 自右向左分流而出现暂时性青紫，如室间隔缺损、 动脉导管未闭和房间隔缺损等
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
病理解剖
Pathology
ASD根据缺损部位分为四型：
①中央型（卵圆窝型），约占76％ ②上腔型（静脉窦型），上腔静脉入口下方，约占 3.5% ③下腔型，下腔静脉入口处，约占12% ④混合型，约占8.5%
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
临床表现
Clinical Manifestations
体格检查
多数生长发育正常，无发绀。分流量大者，可见 身体较瘦长 心脏听诊 胸骨左缘第2～3肋间听到2～3／6级 收缩期喷射性杂音，此杂音系由于通过肺动脉瓣 的血流量增加，致使肺动脉瓣相对性狭窄所致， 故杂音较柔和，且较局限，传导不广泛。另一个 重要特点是P2增强，而且伴有第2心音固定分裂。 重者可出现右心衰竭的表现。
先天性心脏病
congenital heart disease
重庆医科大学儿童医院心内科 何健峰 讲师
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
心电图
Electrocardiography
主要特征
心电轴右偏 右心房大 Vl导联常呈rsR波形，系由右心室容量负荷增加， 使右心室扩张及室上嵴肥厚传导延迟所致
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
临床表现
Clinical Manifestations
临床表现主要取决于缺损及其分流量的大小 症状
缺损小，分流量少者可无任何症状 缺损大，分流量多时，病人可有活动后心悸、 气短，且易发生呼吸道感染 婴幼儿时期一般很少有症状，多数是在体查时 被发现
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
病因和预防 Etiology and Prevention
外来因素
较重要的为宫内感染，如风疹、流行性感冒、流 行性腮腺炎和柯萨奇病毒感染等 其他如孕母缺乏叶酸、与大剂量放射线接触、受 药物影响(抗癌药、甲糖宁等)、患有代谢性疾病 (糖尿病、高钙血症等)或能造成宫内缺氧的慢性 病等 总之，先天性心血管畸形可能是胎儿周围环境因 素与遗传因素相互作用所致
先心病分类 按血液动力学改变分为
右向左分流型(青紫型) 某些原因致使右心压力增 高并超过左心，使血流持续从右向左分流时，或因 大动脉起源异常，使大量静脉血流入体循环，均可 出现持续性青紫。此型常见有法洛四联症和大动脉 转位等
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
心电图
Electrocardiography
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
虽然引起先心病的原因尚未完全明确，但加强孕妇 的保健，特别是在妊娠早期适量补充叶酸，积极预 防风疹、流感等病毒性疾病以及避免与发病有关的 因素接触，对预防先心病具有积极意义
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
房间隔缺损
Atrial Septal Defect，ASD
主要内容
发病率及自然闭合率 病理解剖 病理生理及血液动力学改变 临床表现 特殊检查
心电图 X光胸片 超声心动图 心导管及造影
并发症 治疗
百度文库
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
病理生理及血液动力学改变
Pathophysiology
分流量大小与下列因素有关：
缺损口的大小 左右心室的顺应性 肺循环的阻力
右心房 ASD分流 左心房
血流量增加
血容量减少
右心室 扩大、肥大
左心室 血容量减少
肺动脉 扩张
主动脉 血容量减少
肺动脉循环 充血
体循环 供血不足
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
病因和预防 Etiology and Prevention
在胎儿心脏发育阶段，若有任何因素影响了心脏胚 胎发育，使心脏某一部分发育停顿或异常，即可造 成先天性畸形 这类有关因素很多，大致可分为内在和外来的两类， 以后者为多见
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
发病率及自然闭合率
Incidence and the rate of nature closure
房间隔缺损（ASD)是常见的先心病之一，其发生率 约占先心病的20-30％，其中80％左右为继发孔ASD 继发孔ASD自然闭合率多在1岁之内，闭合率为39%， 1岁以后可能性很小
胚胎第 5 周开始
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
法洛四联症
Tetralogy of Fallot,TOF
房间隔缺损
Atrial Septal Defect
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
概述
先心病是胎儿时期心脏血管发育异常而致的畸形， 是小儿最常见的心脏病 心脏胚胎发育的关键时期是在胚胎的第2～8周， 先天性心脏畸形的形成主要就在这一时期 发病率为7～8/1000 近半个世纪来，由于医学的发展，许多常见的先心 病，甚至复杂的先心病得到准确诊断，大多数可以 得到彻底根治
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
先心病分类 按血液动力学改变分为
无分流型(无青紫型) 即心脏左、右两侧或动、静 脉之间无异常通路或分流，如肺动脉狭窄和主动脉 缩窄等
今天三节课的主要内容
房间隔缺损
Atrial Septal Defect，ASD
室间隔缺损
Ventricular Septal Defect，VSD
动脉导管未闭
Patent Ductus Arteriosus，PDA
病因和预防 Etiology and Prevention
内在因素主要与遗传有关，特别是染色体畸变。 近年来的研究已经证明
房室间隔缺损和动脉干畸形等与第21号染色体 长臂某些区带的过度复制或缺失有关 另外，第7、12、15和22号染色体上也有与形成 心血管畸形有关的基因
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
先心病分类 按血液动力学改变分为
左向右分流型(潜在青紫型) 正常情况下由于体循 环压力高于肺循环，故平时血液从左向右分流而不 出现青紫。当哭闹、屏气或剧烈活动等情况致使肺 动脉或右心压力增高并超过左心压力时，可使血液 自右向左分流而出现暂时性青紫，如室间隔缺损、 动脉导管未闭和房间隔缺损等
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
病理解剖
Pathology
ASD根据缺损部位分为四型：
①中央型（卵圆窝型），约占76％ ②上腔型（静脉窦型），上腔静脉入口下方，约占 3.5% ③下腔型，下腔静脉入口处，约占12% ④混合型，约占8.5%
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
临床表现
Clinical Manifestations
体格检查
多数生长发育正常，无发绀。分流量大者，可见 身体较瘦长 心脏听诊 胸骨左缘第2～3肋间听到2～3／6级 收缩期喷射性杂音，此杂音系由于通过肺动脉瓣 的血流量增加，致使肺动脉瓣相对性狭窄所致， 故杂音较柔和，且较局限，传导不广泛。另一个 重要特点是P2增强，而且伴有第2心音固定分裂。 重者可出现右心衰竭的表现。
先天性心脏病
congenital heart disease
重庆医科大学儿童医院心内科 何健峰 讲师
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
心电图
Electrocardiography
主要特征
心电轴右偏 右心房大 Vl导联常呈rsR波形，系由右心室容量负荷增加， 使右心室扩张及室上嵴肥厚传导延迟所致
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
临床表现
Clinical Manifestations
临床表现主要取决于缺损及其分流量的大小 症状
缺损小，分流量少者可无任何症状 缺损大，分流量多时，病人可有活动后心悸、 气短，且易发生呼吸道感染 婴幼儿时期一般很少有症状，多数是在体查时 被发现
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
病因和预防 Etiology and Prevention
外来因素
较重要的为宫内感染，如风疹、流行性感冒、流 行性腮腺炎和柯萨奇病毒感染等 其他如孕母缺乏叶酸、与大剂量放射线接触、受 药物影响(抗癌药、甲糖宁等)、患有代谢性疾病 (糖尿病、高钙血症等)或能造成宫内缺氧的慢性 病等 总之，先天性心血管畸形可能是胎儿周围环境因 素与遗传因素相互作用所致
先心病分类 按血液动力学改变分为
右向左分流型(青紫型) 某些原因致使右心压力增 高并超过左心，使血流持续从右向左分流时，或因 大动脉起源异常，使大量静脉血流入体循环，均可 出现持续性青紫。此型常见有法洛四联症和大动脉 转位等
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society
心电图
Electrocardiography
With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society