儿童先天性心脏病各论课件

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单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,2018/7/24,#,Ventricular Septal Defect,(,VSD,),1,Ventricular Septal Defect(VSD,Ventricular Septal Defect,The disorder of embryological development of interventricular septum,Most common form of CHD in children,Accounting for 25,2,Ventricular Septal Defect The,Position of VSD,:,Membranous(60-70%): the commonest locationt,Subpulmonic(3-6%): risk of aortic valve prolapse,Muscular(20-30%) : occur anywhere in the muscular part of septum,Anatomic Types,Subpulmonic,Membranous,Muscular,3,Position of VSD: Anatomic Type,Size of VSD,Small: 10,mm,4,Size of VSD4,Before Pulmonary,hypertension,Right atrium,Right,ventricle (Flow,),Pulmonary artery,(expansion),Pulmonary blood flow,Right ventricle (Hypertrophy,),Left,atrium,(,Hypertrophy),Left ventricle (,Hypertrophy,),Ejection,volume,Systemic blood flow,Shunt,Systemic blood,(,Mixed,),Right atrium,Left,atrium,After Pulmonary hypertension,Left ventricle,Pulmonary hypertension,reversible (dynamic),Irreversible,(pulmonary vascular disease),Eisenmeingers syndrome,Shunt,Pulmonary artery,(expansion),Right ventricle (Hypertrophy,),Hemodynamic Characteristics,5,Before Pulmonary hypertensionR,Small VSD,asymptomatic,Pan-systolic murmur of grade,heard at left sternal border in the 3rd4th intercostal spaces, radiating over precordium,( 34 LSB SM,o,),Clinical Manifestations,6,Small VSD Clinical Manifestati,Medium,Large,VSD,(symptoms),Pulmonary plethora-Recurrent chest infection,Systemic blood flow,-Failure,to thrive (slow weight gain,),Poor cardiac function:,Cyanosis when right-to-left shunt occurs, mostly due to severe pulmonary hypertension,Clinical Manifestations,7,MediumLarge VSD Clinical Mani,Medium,Large,VSD,Poor cardiac function:,at infancy,:,difficulty with feeding, sweating, tachypnea, and hepatomegaly;,in older children,:,dyspnea on excursion, easy fatigability, palpitation, exercise intolerance,Clinical Manifestations,8,MediumLarge VSD Clinical Mani,Medium,Large,VSD,(signs),2,4 LSB SM,o,DM at apex due to large blood flow across normal mitral valve,(relative mitral stenosis),P,2,increased with split,Cyanosis with clubbing in late stage,Clinical Manefestation,9,MediumLarge VSD Clinical Mane,Electrocardiogram,Small VSD: ECG usually normal,Mediumlarge,LV hypertrophy when pulmonary vascular resistance is normal,Both LV & RV hypertrophy when pulmonary hypertension occurs due to increased vascular resistance & increased flow,RV hypertrophy in Eisenmengers syndrome,10,ElectrocardiogramSmall VSD: EC,Chest X-ray,Small VSD:,may be normal,Mediumlarge VSD:,Increased vascular markings in lungs,Heart/chest ratio: 0.55,Enlargement of LV and/or RV,Dilated main pulmonary artery segment,Smaller aorta in size,11,Chest X-raySmall VSD: may be n,Echocardiogram,Display position and size of the defect,Display shunting,Measure pressure gradient,Display size of chambers and vessels:,Enlarged LA, LV and/or RV,12,EchocardiogramDisplay position,Echocardiogram,2DE & CDE displays VSD,13,Echocardiogram2DE & CDE displa,Prognosis & Complications,Asymptomatic,3050,close spontaneously by 2 years of age,Congestive heart failure,Pulmonary hypertension,Infundibulum stenosis (,漏斗部狭窄,),Prolapse of aortic valve (,主动脉瓣脱垂,),Infective endocarditis (,感染性心内膜炎,),14,Prognosis & ComplicationsAsymp,Medical Management,Physical activities properly,Prevention and cure of infection timely,Follow-up regularly,Anti congestive measures:,digitalis(,洋地黄,),diuretics(,利尿剂,),vasodilators,(,扩管药物,),Transcatheter closure (,经导管封堵术),15,Medical ManagementPhysical act,Indications for Surgical Repair,Congestive heart failure with failure to thrive or recurrent pneumonia,Progressive pulmonary hypertension,Evidence of infundibulum stenosis,Evidence of prolase of aortic valve,Supracristal VSD,History of infective endocarditis,16,Indications for Surgical Repai,Atrial Septal Defect,(,ASD,),17,Atrial Septal Defect(ASD)17,Atrial Septal Defect,The disorder of embryological development of interatrial septum,Accounting for 10,of CHD,18,Atrial Septal DefectThe disord,上腔静脉,下腔静脉,静脉窦型缺损,继发孔型缺损,原发孔型缺损,主动脉,冠状静脉窦型,fossa,ovalis ASD,(,75%,),Sinus venosus ASDs,(,5%,),Ostium,primum,ASD(15%),Anatomic Types,Coronary sinus ASD,(,2,%,),19,上腔静脉下腔静脉静脉窦型缺损继发孔型缺损原发孔型缺损主动脉冠,20,20,Hemodynamic Characteristics,Superior and Inferior vena cava,RA(Flow,),RV (Hypertrophy,),ASD,Shunt,Pulmonary vein,LA flow,Aorta,Ejection volume,Pulmonary artery,(expansion),Pulmonary blood flow,LV flow,Systemic blood flow,21,Hemodynamic CharacteristicsSup,Symptoms are similar to VSDs,such as poor growth and development, recurrent pneumonia, poor cardiac function,but occur less frequently in infants,Some patients even remain asymptomatic through life,Clinical Manifestations,22,Symptoms are similar to VSDs,Signs,:,2,3LSB SM,o,The murmur is caused by increased flow across pulmonic valves,(i.e. relative pulmonary stenosis),4LSB DM can often be heard,(relative tricuspid stenosis),P,2,increased with fixed split,(,固定分裂),Clinical Manifestations,23,Signs: Clinical Manifestations,Electrocardiogram,Axis right deviation, V1, V3R have incomplete right bundle branch block diagram,导联以,S,为主,,导联以,R,为主,电轴右偏。,R,aVR,0.5mv, R,S,1, V,1,呈,RSr, QRS,0.08,,示不完全右束支转导阻滞。,RV1,SV5,2.5mv,,提示右室大。,24,Electrocardiogram Axis right d,Chest X-ray,Increased vascular markings in lungs,Heart/chest ratio: 0.55,Enlargement of RA, RV,Dilated main pulmonary artery segment,Smaller aorta in size,25,Chest X-ray Increased vascular,Echocardiogram,Display position and size,Display shunting,Display paradoxic motion (,矛盾运动,) of ventricular septum,Display size of chambers and vessels:,Enlarged RA and RV,Dilated MPA,Smaller AO,26,EchocardiogramDisplay position,Echocardiogram,ostium primum ASD,ostium secundum ASD,27,Echocardiogram ostium primum A,Prognosis & Complications,Asymptomatic (often in childhood),Heart failure (occur in middle adulthood),Atrial tachyarrhythmias (adulthood),Pulmonary hypertension (uncommon),Infective endocarditis (rarely occur,),Spontaneous closure,most frequently if ASD 4mm,frequently if ASD 8 mm,mostly closed before age 2 years,28,Prognosis & ComplicationsAsymp,Medical Management,No need of physical restriction for most patients,Prevention and cure of infection timely,Follow-up regularly,Anti congestive measures:,digitalis,diuretics,vasodilators,Transcatheter closure (,经导管封堵术),29,Medical ManagementNo need of p,Patent Ductus Arteriosus,(,PDA,),30,Patent Ductus Arteriosus(PDA),Accounting for 15,of CHD,Incidence may be as high as 2060% in preterm infants weighing 0.55,Enlargement of LA, LV,Dilated main pulmonary artery segment,prominent aorta in size,39,Chest X-raySmall shunt: normal,Echocardiogram,ductal shunting,40,Echocardiogramductal shunting4,Prognosis & Complications,Asymptomatic,Congestive heart failure,Pulmonary hypertension,Infective endocarditis,spontaneous closure of ductal shunt,90% close functionally by 4 days after birth,80% close anatomically in 3 month, and 95% in 1 year of age,41,Prognosis & ComplicationsAsymp,Medical Management,Physical activities properly,Prevention and cure of infection timely,Follow-up regularly,Anti congestive measures:,digitalis,diuretics,vasodilators,Transcatheter closure (,经导管封堵术),42,Medical ManagementPhysical act,Tetralogy of Fallot,(,TOF,),43,Tetralogy of Fallot(TOF)43,Tetralogy of Fallot,Accounting for 10,of CHD,44,Tetralogy of Fallot44,Obstruction to RV outflow,:,infundibular and/or valvular level with hypoplasia of PA,Large VSD,Aorta that overrides,the VSD,Hypertrophy of RV,Anatomic features,45,Obstruction to RV outflow:infu,RA,RV,LA,(,flow,),LV,(,flow,),AO,(,flow,),Mixed blood enter,Systemic circulation,(Expand,),(Hypertrophy),Obstruction to,RV outflow,Pulmonary blood,flow,Oxygen,exchange,is insufficient,VSD Shunt,Overriding aorta,Shunt,(,Right-to-left,shunting,),Hypoxia,Hemodynamic Characteristics,46,RARV LA LV AOMix,Symptoms,:,Owing to anoxia,Cyanosis (mostly seen from 4 months of age and progressive),Retarded growth and development, easy fatigability and dyspnea on excursion,Squatting when walking,Hypoxemic spell,(缺氧发作),: sudden onset of dyspnea; deepening of cyanosis; irritability or syncope; convulsion,;,absence of cardiac murmur,(,a hallmark of severe situation,),Clinical Manifestations,47,Symptoms:Owing to anoxia Clini,48,48,49,49,Signs,:,Cyanosis,Clubbing of fingers and toes,3LSB SM,0, radiating widely,P,2,decreased,S,2,usually predominantly aortic and single,Clinical Manifestation,50,Signs: Clinical Manifestation5,Clubbing,杵,状指,51,Clubbing51,Laboratory Findings,Reponses to anoxia:,Hemoglobin increased,Hematocrit elevated,RBC count increased,blood mucosity increased,52,Laboratory FindingsReponses to,Electrocardiogram,Right axis deviation &,RV h,ypertrophy,in almost all cases,RA expand in some cases,12-year-old boy with TOF,53,Electrocardiogram Right axis d,Chest X-ray,Decreased vascular markings in lungs,“Boot-shaped” heart usually in normal size,Concave PA segment,Enlarged aorta with 25% right aortic arch,54,Chest X-rayDecreased vascular,Echocardiogram,Enlarged overriding aorta with large subaortic VSD,Narrowing of RV outflow tract (IS and/or PS) with small MPA,Thickening of RV wall,55,EchocardiogramEnlarged overrid,Prognosis & Complications,All patients requir heart surgery,Complications:,Embolism,Abscess in the brain,Infective endocarditis,Arrhythmias,56,Prognosis & ComplicationsAll p,Medical Management,Prevention from embolization,Cyanotic spell: propranolol , morphine, NaHCO,3,Maintain patency of ductus: prostaglandin E,Increase pulmonary flow:,balloon dilation procedure,57,Medical Management Prevention,Surgical Repair,Total correction: performed as early as possible in almost all cases,58,Surgical RepairTotal correctio,
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