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1、儿童先天性心脏病儿童先天性心脏病CardiovascularDiseasesiCardiovascularDiseasesinChildrennChildren英文课件英文课件Cardiovascular Diseases in ChildrenCongenital heart diseasesCongenital heart diseasesViral myocarditisRheumatic heart diseaseCardiomyopathyKawasaki DiseaseArrhythmiaCongestive heart failurePericarditisInfective e
2、ndocarditisEtc.Etiologic ConsiderationsHereditary factors Hereditary factors(Associated with 15%CHD)(Associated with 15%CHD)TrisomiesTrisomies of chromosome 21,18,15,13 of chromosome 21,18,15,13 Deletion of chromosome 22q11 Deletion of chromosome 22q11 Gene defectsGene defects:Defects of Elastin in
3、Williams syndrome Defects of Elastin in Williams syndrome Mutations of Mutations of FibrillinFibrillin in in MarfansMarfans syndrome syndrome Mutations of Tbx5 in Holt-Mutations of Tbx5 in Holt-OramOram syndrome syndrome Mutations of Cx43 in Mutations of Cx43 in hypoplastichypoplastic left heart lef
4、t heartEtiologic ConsiderationsEnvironmental factors Exposed to the following factors during the first trimester of pregnancy Viral infections:rubella,influenza,enterovirus,parotitis Physical and chemical factors:drugs,radiation,alcohol,tobacco Maternity diseases:diabetes,connective tissue problems,
5、hypertension syndromePathogenesisHypostasis:Heart development is a very complex process involving many coordinated steps Abnormal embryological development of the heart leads to CHD Mechanism:Genetic basis interacted with the environmental factors play role possibly in most casesFetal Heart Developm
6、entFormation of primitive heart tube Looping of primitive heart tube Formation of endocardial cushion Formation of interatrial septum Formation of interventricular septum Septation of aortic and pulmonary arteries Formation and Looping of Primitive Heart Tube3 weeks of intrauterine life4 weeks of in
7、trauterine life5-6 weeks of intrauterine life7-8 weeks of intrauterine life11 Fetal Circulation&Changes after Birthafter birthbefore before birthbirthDifferences between the fetal and postnatal circulationPresence of placental circulation,which provides gas exchange for the fetus.Absence of gas flow
8、 of blood to the lungs and thus little pulmonary venous return to left atriumPresence of ductus venosus,joining the portal vein with the inferior vena cava,providing a low resistance bypass for umbilical venous blood to reach the inferior vena cavaWidely open foramen ovale to enable oxygenated blood
9、(through umbilical veins)to reach the left atrium and ventricle for distribution to the coronaries and the brainWide open ductus arteriosus to allow right ventricular blood to reach the descending aorta,since lungs are non-functioning.Circulatory adjustments at birth-transitional circulationLoss of
10、placental circulation and clamping of the umbilical cord,after birth,results in a sudden increase in systemic vascular resistance with the exclusion of the low resistance placental circulation.Classification of CHD Based on HemodynamicsLeft-to-right shunt lesions:VSD,ASD,PDA Right-to-left shunt lesi
11、ons:TOF,D-TGA,TA Non-shunt lesions:PS,AS,CoALeft-to-right LesionsPatent Ductus ArteriosusPatent Ductus ArteriosusAtrial Septal DefectVentricular Septal DefectVentricular Septal DefectRight-to-left shunt lesionsTetralogy of Tetralogy of FallotFallot Transposition of the Transposition of the Great Art
12、eries Great Arteries Tricuspid AtresiaTricuspid AtresiaNon-shunt lesionsPulmonary Stenosis Aortic Stenosis Coarctation of the aortaClinic featuresCyanosisDifficult feeding and poor growthDifficult breathingFrequent respiratory infectionsSpecific syndromesClinic features-CyanosisSevere caseCry or exe
13、rtsfeedingClinic features-Difficult feeding and poor growthParents complain that the child has difficulty with feeds.Slow feeding,small volumes consumed Growth rate is not appropriate for age.(growth retardation affects weight more than height.)Clinic features-Difficult breathingTachypneaRespiratory
14、 rates 60/min 50/min 2mon 40/min 12monClinic features-Frequent respiratory infectionsFrequentSevereLong durationDifficult to treatClinic features-Specific syndromesTrisomy 21 Table 15-7 P401Diagnostic Tools for CHDHistory taking History taking Physical examination Physical examination Electrocardiog
15、raphyElectrocardiography(心电图检查)(心电图检查)(心电图检查)(心电图检查)Chest RoentgenographyChest Roentgenography(X X线胸片)线胸片)线胸片)线胸片)EchocardiographyEchocardiography(超声心动图)(超声心动图)(超声心动图)(超声心动图)Magnetic resonance imagingMagnetic resonance imaging(磁共振成像)(磁共振成像)(磁共振成像)(磁共振成像)Computed Tomography(Computed Tomography(计算机断层摄
16、影术计算机断层摄影术计算机断层摄影术计算机断层摄影术)Cardiac catheterizationCardiac catheterization(心导管检查)(心导管检查)(心导管检查)(心导管检查)AngiocardiographyAngiocardiography(心血管造影)(心血管造影)(心血管造影)(心血管造影)History Taking From mother and family:From mother and family:History of mother during pregnancy History of mother during pregnancy Abnorm
17、al history of previous pregnancy Abnormal history of previous pregnancy Family history of CHD,chromosome diseases Family history of CHD,chromosome diseases From the child From the child Recurrent pneumonia Recurrent pneumonia Cyanosis Cyanosis Heart murmur found before Heart murmur found before Card
18、iac dysfunctionCardiac dysfunction:feeding difficulty,tachypnea,feeding difficulty,tachypnea,sweating,edema sweating,edema OthersOthers:skinny,hoarseness when cryingskinny,hoarseness when cryingCardiac ExaminationInspectionInspection:Prominence of precordiumProminence of precordium(心前区隆起)(心前区隆起)(心前区
19、隆起)(心前区隆起)Increased cardiac activityIncreased cardiac activity(心脏搏动弥散)(心脏搏动弥散)(心脏搏动弥散)(心脏搏动弥散)Jugular venous distensionJugular venous distension(颈静脉怒张)(颈静脉怒张)(颈静脉怒张)(颈静脉怒张)PalpationPalpation:Apex impulse(Apex impulse(心尖搏动)心尖搏动)心尖搏动)心尖搏动)Heave of impulseHeave of impulse(抬举样搏动)(抬举样搏动)(抬举样搏动)(抬举样搏动)Pre
20、cordial thrillPrecordial thrill(震颤)(震颤)(震颤)(震颤)PercussionPercussion:Estimation of size and location of the heartEstimation of size and location of the heartCardiac Examination AuscultationAuscultation:Rate and rhythm of heart beats Rate and rhythm of heart beats Normal heart sounds:Normal heart soun
21、ds:S S1 1,S,S2 2(A(A2 2&P&P2 2),S),S3 3,S,S4 4 Abnormal heart sounds:Abnormal heart sounds:splitting,intensity,ejection clicks,Ssplitting,intensity,ejection clicks,S3 3,S,S4 4 Murmurs:Murmurs:systolic,diastolic,continuous systolic,diastolic,continuous Pericardial friction rubPericardial friction rub
22、Cardiac ExaminationHeart Rate and Rhythm Neonate120140 bpm Infancy110130 bpm Toddlers age 100120 bpm Preschool age 80100 bpm School age 70 90 bpmCardiac ExaminationDifferential diagnosis of heart murmur CHDInnocent locationprecordium24LSB,apex phaseSM,DM,CMSM,short qualityharsh soft intensitygradeII
23、 0.7 mv R 0.7 mv rsRrsR complex with R 1.5 mv complex with R 1.5 mv RsRs complex with R/s ratio as follows complex with R/s ratio as follows:1yr5 5 1-3yr1-3yr2.5 2.5 3-5yr3-5yr2 2 5-12yr5-12yr1.5 1.5 12y 12y11RoentgenogramChest X-ray is essential in the evaluation of heart size and pulmonary vascula
24、rityEchocardiographyThe major noninvasive The major noninvasive diagnostic method for diagnostic method for CHD CHD To define anatomy,To define anatomy,function,chamber&function,chamber&vessel size,and valve vessel size,and valve abnormalitiesabnormalitiesModelities of Echocardiography M-mode Echoca
25、rdiography Two-dimensional Echocardiography Doppler Echochardiography Three-dimensional Echocardiography Transesophageal Echocardiography Fetal EchocardiographyM-mode EchocardiogramTwo-dimensional EchocardiogramDoppler EchochardiogramPulsed Doppler Pulsed Doppler EchochardiographyEchochardiography C
26、ontinuous-wave Doppler Continuous-wave Doppler EchochardiographyEchochardiography Color Doppler Color Doppler EchochardiographyEchochardiography Pulsed Doppler Echochardiogram Color Doppler EchochardiogramReal-time Three-dimensional Echocardiogram Magnetic Resonance ImagingValuable tool in the evalu
27、ation of CHD Particularly in the imaging of vascular structures of the thoraxSpiral Computed TomographyAnother valuable Another valuable tool in the tool in the evaluation of evaluation of vascular structures vascular structures of the thoraxof the thoraxCardiac Catheterization Performed for the nee
28、d of Performed for the need of further anatomic or further anatomic or physiologic information physiologic information Performed for the Performed for the purpose of treatment in purpose of treatment in the catheterization the catheterization laboratorylaboratoryCardioangiography Nadas Criteria One
29、major or two minor One major or two minor creteriacreteriaMajor creteriaSystolic murmur grate III or more in intensityDiastolic murmurCentral cyanosisCongestive cardiac failureMinor creteriaSystolic murmur less than grade IIIAbnormal second soundAbnormal electrocardiogramAbnormal X-rayAbnormal blood
30、 pressurePansystolic murmurA pansystolic murmur is always abnormal no matter what is its intensity.There are only three lesions that produce a pansystolic murmur VSD mitral reguragitation tricuspid regurgitationEjection systolic murmurMay be due to an organic cause or it may be functional.Grade III
31、ejection systolic murmur of a functional type may be heard in anemia or high fever.Principle of Management of CHDPhysical activities properly Prevention and cure of infection timely Protection of heart function Follow-up regularly Therapeutic methods:Interventional catheterization Surgical repairInterventional CatheterizationBolloon Atrioseptostomy (Rashkind,1966)Balloon Angioplasty&Valvuloplasty (Kan,1982)Device Occlusion of Defects(Sideris,1990;Amplatzer,1998)Surgical Repair of CHD 结束!结束!