1、Congenital Heart Disease(CHD)Shengjing Hospital Pediatrics Yu Xuexin Pediatrics,Shengjing Hospital IntroductionIntroduction CHD is defined as an abnormality in circulatory structure or function that is present at birth,even if it is discovered much later.Incidence:6.9 in alive neonatal.150,000 neona
2、tal suffer from CHD in China per year.New treatments：catheterization、development of operation,etc.Pediatrics,Shengjing Hospital Object and RequestObject and Request Familiar with the etiology and classification of CHD.Master the hemodynamics、clinical menifestation and diagnosis of common complicatio
3、ns in VSD,ASD,PDA and TOF.Pediatrics,Shengjing Hospital EtiologyEtiology Internal factors：gene mutation or chromosome aberration.External factors:intraureteral infection、ray、drug、metabolic diseases、intraureteral hypoxia.Pediatrics,Shengjing Hospital ClassificationClassification left-to-right shunts
4、Cyanosis maybe VSD、PDA、ASD right-to-left shunts non-shunts Cyanosis TOF、dislocation of main artery Pulmonary artery stenosis、aortic stenosis Based on shunt between right and left heart Pediatrics,Shengjing Hospital Patent ductus arteriosus PDA Common CHD in ClinicCommon CHD in Clinic Atrial septal d
5、efect ASD Ventricular septal defect VSD Tetralogy of Fallot TOF 1 2 3 4 Pediatrics,Shengjing Hospital Ventricular Septal DefectVentricular Septal Defect（VSDVSD）1、membrane defect 85%2、muscular defect 3、funnel defect 10%20-50 VSD can close up without treatment.1、minor defect 2、media defect 3、major def
6、ect Anatomy Most common，30%in CHD。Pediatrics,Shengjing Hospital PathobiologyPathobiology RV blood,pulmonary hypertension,persistent cyanosis(Eisenmenger syndrome)LV blood,body circulation Pediatrics,Shengjing Hospital HemodynamicsHemodynamics Before pulmonary hypertensionBefore pulmonary hypertensio
7、n RA RV（blood）Pulmonary artery(dilation)Pulmonary circlation (congestion)RV(dilation)LA(hypertrophy)LV(hypertrophy)（射血量减少）（射血量减少）body circulation Blood volume shunt Pediatrics,Shengjing Hospital HemodynamicsHemodynamics Body circulation（mixed blood）RA LA Pulmonary Artery dilation RV（Dilation)After p
8、ulmonary hypertensionAfter pulmonary hypertension LV Dynamic Pulmonary hypertesion Obstructive pulmonary hypertesion shunt Pediatrics,Shengjing Hospital Clinical ManifestationClinical Manifestation 症状：分流量大时：生长迟缓、体重不增、消瘦、喂症状：分流量大时：生长迟缓、体重不增、消瘦、喂养困难、活动后乏力、气短、多汗、反复呼吸道感染、养困难、活动后乏力、气短、多汗、反复呼吸道感染、心衰。声音嘶哑（
9、肺动脉压迫喉返神经）。心衰。声音嘶哑（肺动脉压迫喉返神经）。体征：胸骨左缘体征：胸骨左缘3 3、4 4肋间肋间-粗糙的全收缩期杂音，粗糙的全收缩期杂音，向四周传导，伴有震颤。向四周传导，伴有震颤。肺动脉第二音亢进。肺动脉第二音亢进。二尖瓣相对狭窄的较柔和舒张中期杂音二尖瓣相对狭窄的较柔和舒张中期杂音 Pediatrics,Shengjing Hospital ExaminationExamination X X线：左、右心室增大，以左室增大为主，主动脉线：左、右心室增大，以左室增大为主，主动脉弓影较小，肺动脉段突出，肺野充血。艾森曼格弓影较小，肺动脉段突出，肺野充血。艾森曼格综合征：肺动脉主支
10、增粗，肺外周血管影很少，综合征：肺动脉主支增粗，肺外周血管影很少，宛如枯萎的枯枝。宛如枯萎的枯枝。心电图心电图 USUS Pediatrics,Shengjing Hospital Complications and treatmentComplications and treatment 合并症合并症 支气管肺炎、心衰、肺水肿、亚急性细菌性心内膜炎支气管肺炎、心衰、肺水肿、亚急性细菌性心内膜炎 治疗治疗 小型缺损：不一定手术治疗。小型缺损：不一定手术治疗。中型缺损：中型缺损：5 5-6 6岁做手术。岁做手术。大型缺损并反复心衰者：可在大型缺损并反复心衰者：可在6 6月月-2 2岁内做手术。岁
11、内做手术。介入治疗介入治疗 Pediatrics,Shengjing Hospital Atrial Septal Defect ASDASD 5 5-10%10%病理解剖：病理解剖：1.1.原发孔型：约占原发孔型：约占15%15%，缺损位于心内膜垫与房间隔交界处。，缺损位于心内膜垫与房间隔交界处。2.2.继发孔型：中央型，最常见，约占继发孔型：中央型，最常见，约占75%75%，缺损位于房间隔，缺损位于房间隔中心卵圆窝。中心卵圆窝。3.3.静脉窦型：约占静脉窦型：约占5%5%，分为上腔型和下腔型。，分为上腔型和下腔型。4.4.冠状静脉窦型：约占冠状静脉窦型：约占2%2%，缺损位于冠状静脉窦上端
12、与左，缺损位于冠状静脉窦上端与左心房间。心房间。Pediatrics,Shengjing Hospital Atrial Septal Defect ASDASD Hemodynamics Pediatrics,Shengjing Hospital Hemodynamic ChangeHemodynamic Change 上、下腔静脉血上、下腔静脉血 肺静脉肺静脉 右心房（扩大）右心房（扩大）左心房左心房 右心室右心室 （增大）（增大）左心室（血量左心室（血量 减少）减少）肺血流量明显增加（肺充血）肺血流量明显增加（肺充血）肺小动脉痉挛、增厚肺小动脉痉挛、增厚 体循环供血不足体循环供血不足 右
13、向左分流右向左分流 （消瘦、乏力、心悸、气短等）（消瘦、乏力、心悸、气短等）艾森曼格综合征艾森曼格综合征 (少数病人晚期少数病人晚期）Pediatrics,Shengjing Hospital ASD EtibiologyASD Etibiology Pulmonary circulation blood Pulmonary circulation blood volume increase volume increase Body circulation blood Body circulation blood volume decreasevolume decrease Pediatric
14、s,Shengjing Hospital Clinical MenifestationClinical Menifestation SymptomsSymptoms：分流量大：肺充血、体循环血量不足。体型瘦：分流量大：肺充血、体循环血量不足。体型瘦长、面色苍白、乏力、多汗、生长发育迟缓。反复呼吸道长、面色苍白、乏力、多汗、生长发育迟缓。反复呼吸道感染、心衰。感染、心衰。听诊听诊：第一心音亢进，肺动脉第二心音增强：第一心音亢进，肺动脉第二心音增强 第二心音固定分裂第二心音固定分裂 胸骨左缘第二肋间胸骨左缘第二肋间2 2-3 3级喷射性收缩期杂音级喷射性收缩期杂音 三尖瓣舒张期杂音三尖瓣舒张期杂音
15、 Pediatrics,Shengjing Hospital 房缺辅助检查房缺辅助检查 X X线：分流大。右心房及右心线：分流大。右心房及右心室增大为主，心胸比大于室增大为主，心胸比大于0.50.5。“肺门舞蹈”。梨形心。“肺门舞蹈”。梨形心。ECGECG：电轴右偏，：电轴右偏，P P-R R间期延间期延长，长，V1V1及及V3V3导联成导联成rSrrSr或或rsRrsR等不完全性右束支传导阻滞。等不完全性右束支传导阻滞。B B-USUS：右心房、右心室增大及：右心房、右心室增大及室间隔的矛盾运动。室间隔的矛盾运动。Pediatrics,Shengjing Hospital ASDASD并发症
16、和治疗并发症和治疗 并发症并发症 支气管肺炎、心衰等支气管肺炎、心衰等 治疗治疗 学龄前手术学龄前手术 介入性心导管术介入性心导管术 应用双面蘑菇伞关闭缺损，适用继应用双面蘑菇伞关闭缺损，适用继发孔型房缺发孔型房缺 Pediatrics,Shengjing Hospital 占先心病总数15%。动脉导管未闭（动脉导管未闭（PDAPDA）病理解剖及分型病理解剖及分型 Pediatrics,Shengjing Hospital 动脉导管未闭病理生理动脉导管未闭病理生理 1 1、肺循环充血肺循环充血 2 2、体循环供血不足体循环供血不足 3 3、肺动脉高压时，产生右向左分流，出现下、肺动脉高压时，产生右向左分流，出现下半身青紫半身青紫差异性青紫差异性青紫 Pediatrics,Shengjing Hospital 动脉导管未闭动脉导管未闭 PDAPDA 血流动力学示意图血流动力学示意图 Pediatrics,Shengjing Hospital 动脉导管未闭临床表现动脉导管未闭临床表现 症状：与症状：与VSDVSD、ASDASD相同相同 体征体征：胸骨左缘上方连续性：胸骨左缘上方连续性“机械
