小儿心脏疾病课件

1、Cardiovascular Diseases in Children（General Introduction ）（黄国英） HUANG Guo-ying, M.D.Professor, Department of PediatricsFudan University Shanghai Medical C教学要求1.熟悉心血管胚胎发育、先心病病因及病理机制 2.了解胎儿循环特点及出生后变化 3. 熟悉小儿循环系统常用检查方法 4.掌握先天性心脏病的分类5.了解小儿先心病治疗方法 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

2、Cardiovascular Diseases in ChildrenCongenital heart diseasesViral myocarditisRheumatic heart diseaseCardiomyopathyKawasaki DiseaseArrhythmiaCongestive heart failurePericarditisInfective endocarditisEtc.Fetal Heart DevelopmentFormation of primitive heart tubeLooping of primitive heart tubeFormation o

3、f endocardial cushionFormation of interatrial septumFormation of interventricular septumSeptation of aortic and pulmonary arteriesFormation and Looping of Primitive Heart Tube胚胎第3周胚胎第4周胚胎第5-6周胚胎第7-8周Formation of Endocardial CushionAV canalposteriorright AV orificeanteriorleft AV orifice胚胎第3周胚胎第4周胚胎第

4、5-6周胚胎第7-8周Formation of Endocardial CushionSeptation of Atria and Ventriclesostium primum1LA胚胎第3周胚胎第4周胚胎第5-6周胚胎第7-8周2LV3RA4RV5septum primum6septum secundum7 endocardial cushionostium secundumforamen ovale8,9ventricular septaInterventricular foramenSeptation of VentriclesIVS is composed of three part

5、s：muscular septumcushion componentA-P septum componentSeptation of Great ArteriesAOPAPAAOPAPAAOAOPAPAPAPAMalposition of Great ArteriesTOFTGADORVTaussig-BingEvolvement of Pharyngeal Archesobsolete soon after birthcarotid arteriessubclavian A.brachiocephalic A.ductus arteriosusbranches ofpulmonary art

6、ery1st pair2nd pair3rd pair4th pair5th pair6th pairFetal Circulation & Changes after BirthafterbirthbeforebirthCongenital Heart DiseasesDefinition：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 （

7、Epidemiologic survey during 19891991）Etiologic ConsiderationsHereditary factors (Associated with 15% CHD)Trisomies of chromosome 21, 18, 15, 13 Deletion of chromosome 22q11 （22号染色体长臂q11区域微小缺失）Gene defects：Defects of Elastin in Williams syndromeMutations of Fibrillin in Marfans syndromeMutations of T

8、bx5 in Holt-Oram syndromeMutations of Cx43 in hypoplastic left heartEtiologic ConsiderationsEnvironmental factorsExposed to the following factors during the first trimester of pregnancyViral infections: rubella, influenza, enterovirus , parotitisPhysical and chemical factors: drugs, radiation, alcoh

9、ol, tobaccoMaternity diseases: diabetes, connective tissue problems, hypertension syndromePathogenesisHypostasis（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

10、 environmental factors play role possibly in most casesClassification of CHD Based on HemodynamicsLeft-to-right shunt lesions：VSD, ASD, PDARight-to-left shunt lesions：TOF, D-TGA, TANon-shunt lesions：PS, AS, CoALeft-to-right LesionsPatent Ductus ArteriosusAtrial Septal DefectVentricular Septal Defect

11、Right-to-left shunt lesionsTetralogy of FallotTransposition of the Great ArteriesTricuspid AtresiaNon-shunt lesionsPulmonary StenosisAortic StenosisCoarctation of the aortaRequired from This LessonTo understand the embryological development of heart, causes of CHD and the pathogenesis To know the ch

12、aracteristics of fetal circulation & changes after birth To understand the diagnostic methods for CHDTo understand fully the classification of CHDTo know the principle of treatment of CHDDiagnostic Tools for CHDHistory takingPhysical examinationElectrocardiography（心电图检查）Chest Roentgenography（X线胸片）Ec

13、hocardiography（超声心动图）Magnetic resonance imaging（磁共振成像）Computed Tomography (计算机断层摄影术)Cardiac catheterization（心导管检查） Angiocardiography（心血管造影）History TakingFrom mother and family:History of mother during pregnancyAbnormal history of previous pregnancy Family history of CHD, chromosome diseasesFrom the

14、child Recurrent pneumoniaCyanosis Heart murmur found beforeCardiac dysfunction：feeding difficulty, tachypnea, sweating, edemaOthers： skinny, hoarseness when cryingCardiac ExaminationInspection：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：

15、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 heartCardiac ExaminationAuscultation：Rate and rhythm of heart beatsNormal heart sounds: S1, S2 (A2 & P2), S3 , S4 Abnormal heart sounds: splitting, intensity, ejection clicks, S3 , S4 Mur

16、murs: systolic, diastolic, continuousPericardial friction rubCardiac ExaminationHeart Rate and Rhythm Neonate120140 bpmInfancy110130 bpmToddlers age100120 bpmPreschool age 80100 bpmSchool age 70 90 bpmCardiac ExaminationDifferential diagnosis of heart murmurCHDInnocentlocationprecordium24LSB, apexph

17、aseSM, DM, CMSM, shortqualityharsh softintensitygradeII 0.7 mvrsR complex with R 1.5 mvRs complex with R/s ratio as follows：51-3yr2.53-5yr25-12yr1.5 12y1RoentgenogramChest X-ray is essential in the evaluation of heart size and pulmonary vascularityEchocardiographyThe major noninvasive diagnostic met

18、hod for CHDTo define anatomy, function, chamber & vessel size, and valve abnormalitiesModelities of Echocardiography M-mode EchocardiographyTwo-dimensional EchocardiographyDoppler EchochardiographyThree-dimensional EchocardiographyTransesophageal EchocardiographyFetal EchocardiographyM-mode Echocard

19、iogramTwo-dimensional EchocardiogramDoppler EchochardiogramPulsed Doppler EchochardiographyContinuous-wave Doppler EchochardiographyColor Doppler EchochardiographyPulsed Doppler EchochardiogramContinuous-wave Doppler EchochardiogramPG4 x Vmax2 Color Doppler EchochardiogramReal-time Three-dimensional Echocardiogram Magnetic Resonance ImagingValuable tool in the evaluation of CHDParticularly in the imaging of vascular structures of the thoraxSpiral Computed TomographyAnother valuable tool in the evaluation of vascular structures of the thoraxCardiac CatheterizationPerformed for the need of