【临床医学】先天性心脏病概论黄国英.ppt

Cardiovascular Diseases in Children（Conspectus）,The Aim of This Class,To understand the classification of CHDTo know well the embryological development of heart,causes of CHD and its pathogenesis To know well the approaches of evaluation of CHDTo know the characteristics of fetal circulation&changes after birthTo have a general idea about cardiovascular diseases in children,and the principle of treatment of CHD,Childrens Hospital,Founded in 1952,Professor CHEN Cuizhen,Cardiovascular Diseases in Children,Congenital heart diseaseViral myocarditisRheumatic heart diseaseCardiomyopathyKawasaki DiseaseArrhythmiaCongestive heart failurePericarditisInfective endocarditisEtc.,Embyological Development of Heart,Formation of primitive heart tubeLooping of primitive heart tubeFormation of endocardial cushionFormation of interatrial septumFormation of interventricular septumSeptation of aortic and pulmonary arteries,Formation and Looping of Primitive Heart Tube,Formation of Endocardial Cushion,AV canal,posterior,right AV orifice,anterior,left AV orifice,Formation of Endocardial Cushion,Septation of Atria and Ventricles,ostium primum,1LA,胚胎第3周,胚胎第4周,胚胎第5-6周,胚胎第7-8周,2LV,3RA,4RV,5septum primum,6septum secundum,7 endocardial cushion,ostium secundum,foramen ovale,8,9ventricular septa,Interventricular foramen,Septation of Atria,Septation of Ventricles,IVS is composed of three parts：muscular septumcushion componentA-P septum component,Septation of Great Arteries,AO,PA,PA,AO,PA,PA,AO,AO,PA,AO,Malposition of Great Arteries,TOF,TGA,DORV,Taussig-Bing,Evolvement of Arterial Arches,obsolete soon after birth,carotid arteriessubclavian A.brachiocephalic A.,ductus arteriosus,branches ofpulmonary artery,1st pair,2nd pair,3rd pair,4th pair,5th pair,6th pair,Fetal Circulation&Changes after Birth,afterbirth,beforebirth,Congenital Heart Diseases,Definition：Cardiovascular malformations present during embryonic stages（胚胎期发生的心血管畸形）Prevalence：78/1000 newborns（reported data）6.87/1000 newborns in Shanghai（epidemiologic survey during 19891991）,Etiologic Considerations,Hereditary factorsTrisomies of chromosome 21,18,13 CATCH22 syndrome（deletion of 22 q11)（22号染色体长臂q11区域微小缺失）Gene defects：Mutations of Fibrillin in Williams syndromeDefects of Elastin in Marfans syndromeMutations of Cx43 in Hypoplastic left heart,Etiologic Considerations,Environmental factorsExposed to the following factors during the first trimester of pregnancyViral infections:rubella,influenza,enterovirus,paratitisPhysical and chemical factors:drugs,radiation,alcohol,tobaccoDiseases:diabetes,connective tissue problems,congenital defects,hypertension syndrome,Pathogenesis,Hypostasis（本质）：Heart development is a very complex process involving many coordinated stepsAbnormal embryological development of the heart leads to CHDMechanism（机制）：Genetic basis interacted with the environmental factors play role possibly in most cases,教学要求1.掌握先天性心脏病的分类2.熟悉心血管胚胎发育、先心病病因及病理机制 3.熟悉小儿循环系统常用检查方法 4.了解胎儿循环特点及出生后变化 5.了解小儿循环系统疾病概况及先心病治疗方法 复习与思考题1.先心病的分类及其代表性疾病2.先心病的临床表现及常用的检查方法 3.心脏胚胎发育与先心病的关系,Classification of CHD Based on Hemodynamics,Left-to-right shunt lesions：VSD,ASD,PDARight-to-left shunt lesions：TOF,D-TGA,TANon-shunt lesions：PS,AS,CoA,Left-to-right Lesions,Patent Ductus Arteriosus,Atrial Septal Defect,Ventricular Septal Defect,Right-to-left shunt lesions,Tetralogy of FallotTransposition of the Great ArteriesTricuspid Atresia,Non shunt lesions,Pulmonary StenosisAortic StenosisCoarctation of the aorta,Diagnostic Evaluation of CHD,Approaches of evaluation：History takingPhysical examinationElectrocardiogram（心电图）Chest Xray（胸片）Echocardiogram（超声心动图）Magnetic resonance imaging（磁共振成像）Cardiac catheterization（心导管检查）Angiocardiography（心血管造影）,History Taking,From mother and family:History of mother during pregnancyAbnormal history of previous pregnancy Family history of CHD,chromosome diseasesFrom the child Recurrent pneumoniaCyanosis Heart murmur found beforeCardiac disfunction：feeding difficulty,tachypnea,sweating,edemaOthers：hoarseness when crying,skinny,Cardiac Examination,Inspection：Prominence of precordium（心前区隆起）Increased cardiac activity（心脏搏动弥散）Jugular venous distension（颈静脉怒张）Palpation：Apex impulse(心尖搏动）Heave of impulse（抬举样搏动）Precordial thrill（震颤）Percussion：Estimation of size and location of the heart,Cardiac Examination,Auscultation：Rate and rhythm of heart beatsNormal heart sounds:S1,S2(A2&P2),S3,S4 Abnormal heart sounds:splitting,intensity,ejection clicks,S3,S4 Murmurs:systolic,diastolic,continuousPericardial friction rub,Cardiac Examination,Heart Rate and Rhythm Neonate 120140 bpmInfancy 110130 bpmToddlers age100120 bpmPreschool age 80100 bpmSchool age 70 90 bpm,Cardiac Examination,Differential diagnosis of heart murmurCHDInnocentlocation24LSB24LSB,apexphaseSM,DM,CMSM,shortqualityharsh softintensitygradeIIgradeIIIradiationextensivelocalizedvariationlessmore,Noncardiac Examination,Arterial pulse:rate,rhythm,quality,amplitude,uniformity at limbsArterial blood pressure:Extremities：clubbing of fingers&toes,edemaAbdomen:hepatomegaly,spleenomegaly,Arterial Blood Pressure,Upper limbs：Systolic BP(mmHg)=age+80Diastolic BP(mmHg)=2/3 BPsLower limbs：BP is 20mmHg higherAbnormal BP：20mmHg higher or lower,Electrocardiography,EKG is essential in assessing hypertrophy of atria or ventricles,arrhythmias and disorders of conductive system,Enlargement of Atria,Enlargement of left atrium：P-wave 0.09 sec in durationDouble peaks of P-wave with interval of 0.04 sec or moreEnlargement of right atrium：P II 0.25 mv in amplitudePV1 0.20 mv in amplitude,Enlargement of Left Ventricle,Rv5 3.0 mv in amplitudeSv1 1.5 mv in amplitudeRv5+Sv1 4.5mv in amplitudeSv1 1.5 mv（shown as QS）,Enlargement of Right Ventricle,The following signs shown in lead V1:qR complex R 0.7 mvrsR complex with R 1.5 mvRs complex with R/s ratio as follows：51-3yr2.53-5yr25-12yr1.5 12y1,Roentgenography,Chest X-ray is essential in the evaluation of heart size and pulmonary vascularity,Echocardiography,The major noninvasive method for diagnosis of CHDTo define anatomy,function,chamber&vessel size,and valve abnormalities,Techniques of Echocardiography,M-mode EchocardiographyTwo-dimensional EchocardiographyDoppler EchochardiographyThree-dimensional EchocardiographyTransesophageal EchocardiographyFetal EchocardiographyOthers,M-mode Echocardiography,Two-dimensional Echocardiography,Doppler Echochardiography,Christian Doppler:Doppler shift（频移）Flow direction：to detect shunting and regurgitationDuration：systole or diastoleFlow character：laminar or turbulent,Pulsed Doppler EchochardiographyContinuous-wave Doppler EchochardiographyColor Doppler Echochardiography,Doppler Shift&Flow Character,Pulsed Doppler Echochardiography,Continuous-wave Doppler Echochardiography,PG4 x Vmax2,Color Doppler Echochardiography,Color Doppler Echochardiography,Dynamic Three-dimensional Echocardiography,3D view of ASD,Real-time Three-dimensional Echocardiography,Magnetic Resonance Imaging,Valuable tool in the evaluation of CHDParticularly in the imaging of vascular structures of the thorax,Cardiac Catheterization&Angiocardiography,Performed for the need of further anatomic or physiologic informationPerformed for the purpose of treatment in the catheterization laboratory,Cardioangiography,Principle of Management of CHD,Physical activities properly Prevention and cure of infection timelyProtection of heart function Follow-up regularlyTherapeutic methods：Interventional catheterizationSurgical repair,Interventional Catheterization,Bolloon Atrioseptostomy(Rashkind,1966)Balloon Angioplasty Amplatzer,1998),Surgical Repair of CHD,