医学类教学课件：儿科 CHD

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1、Congenital Heart DiseaseCardiovascular Development Key periods:the 2nd 8th weeksFetal CirculationPhysiological Changes in the Circulation After BirthvChanges in the pulmonary circulation vClosure of the ductus arteriosus vClosure of foramen ovale vArrest of circulation through the placenta Epidemiol

2、ogy of Congenital Heart DiseasevCongenital heart disease occurs in approximately 8 of 1000 live birthsvAbout 2-3 out of 1000 total newborn infants will be symptomatic with heart disease in the 1st yr of life Etiology The etiology of most specific congenital heart defects is still unknown Genetic Fac

3、tors:(1)single gene mutation or multifactorial inheritance pattern (2)certain types of VSD(supracristal)are more common in children of Asian background (3)the recurrence risk of congenital heart disease increases from 0.8%to about 2-6%if a 1st degree relative is affected.(4)approximately 3%of patien

4、ts with congenital heart disease have an identifiable single gene defect,such as Marfan or Noonan syndrome (5)5-8%of patients with congenital heart disease have an associated chromosomal abnormality:90%of trisomy 18,50%of trisomy 21,40%of Turner syndromeInfective Factors:congenital rubella syndromeE

5、nvironmental Factors:physical and chemical factorsDrug FactorsMaternal Diseases:Diabetes mellitus,SLEClassification of Congenital Heart DiseaseLeft to right Shunt Lesions:potential cyanotic CHD(ASD,VSD,PDA)Right to Left Shunt Lesions:cyanotic CHD(TOF,TGA)No Shunt Lesions:acyanotic CHD(PS,AS,COA,MS,M

6、VP)The Common Features of Left to Right Shunt CHDvPotential cyanosisvSystolic heart murmur in left sternal border(LSB)vPulmonary blood flow increasevSystemic blood flow decreaseDiagnostic MethodsvHistory and Physical ExaminationvECGvChest X-rayvEchocardiography M-mode,two dimension-morphology Pulsed

7、,continuous wave and colour doppler Transesophageal Echocardiography(TEE)Fetal EchocardiographyvCatheterization pressure,resistance,oxygen saturation,cardiac output,pathway,angiocardiography,myocardial biopsy,electrophysiologic study,interventional treatmentvMRI,CT,ECTTreatment in Patients with CHDv

8、Non-operation Drug:PGE1,Indomethacin Interventional treatmentvOperationAtrial Septal Defect(ASD)Development of Atrial Septum Classification of ASDvOstium Primum Defect (AV Canal or Endocardial Cushion or Atrioventricular septal defect)In the lower portion of the atrial septum and overlies the mitral

9、 and tricuspid valves,common with a cleft in the anterior leaflet of mitral valve and mitral regurgitationvOstium Secundum DefectvSinus Venosus Defect Patent foramen ovale Isolated patent foramen ovale is usually of no hemodynamic significanceType of Secundum ASDPathophysiology of ASDPathophysiology

10、 of ASD SVC IVC LV LA RA RV AO PV PA Hyperkinetic pulmonary hypertension extremely large pulmonary blood flow Obstructive pulmonary hypertension pulmonary arteriolar medial thickness pulmonary vascular obstructive disease RVP RAP RAPLAP RA to LA Shunt cyanosis-Eisenmenger syndromeASDcomparative pulm

11、onary valve stenosisPathophysiology of ASDv Left to right shunt can occur both in systole and diastole because the LA pressure is always little higher than the RA pressure both in systole and diastolev The degree of Left-to-Right shunting is dependent on:1.size of defect 2.relative compliances of th

12、e RV and LV 3.relative vascular resistances in the pulmonary and systemic circulationsPathophysiology of ASDvThe large blood flow through the right side of the heart results in:l.enlargement of RA and RV and dilatation of PA 2.the pulmonary artery pressure(PAP)remains normal because of the absence o

13、f a high pressure communication between the pulmonary and systemic circulations 3.pulmonary vascular resistance remains low throughout childhood,but it may begin to increase in adulthood 4.the LV is normal in sizeClinical Manifestationsv Asymptomaticv A soft systolic murmur at the 2nd intercostal sp

14、ace of LSB(increased RV blood flow across the pulmonary valve-comparative pulmonary valve stenosis)v Typical wide and fixed splitting of P2(The pulmonic 2nd heart sound)throughout the respiratory cycle(increased RV blood flow producing a prolonged pulmonary valve close constantly throughout the resp

15、iratory cycle)Clinical Manifestationsv An early-to-mid diastolic murmur at the lower LSB(increased blood flow across the tricuspid valve-comparative tricuspid valve stenosis)v P2 louder and pulmonic ejection click when pulmonary hypertensionChest X-rayv Enlargement of the RA and RVv Pulmonary artery

16、 is largev Pulmonary vascularity is increasedv LV is normal v Enlargement of the LV in Ostium Primum DefectECGv Right axis deviationv Enlargement of the RA and RVv Minor right ventricular conduction delay(ICRBBB:an rsR pattern in the right precordial leads)v Enlargement of the LV in Ostium Primum De

17、fectEchocardiographyv Enlargement of the RA and RVv Ventricular septum and posterior wall of LV move in same directionv Position and size of ASDv Direction and degree of shunt in ASDCatheterization Oxygen saturation in RA is higher than in SVC and IVC Right cardiac catheter is easy to enter LA throu

18、gh the ASD Prognosis and Complicationsvsymptoms usually do not appear until the 3rd decade or latervpulmonary hypertension and heart failure are late manifestationsvinfective endocarditis is extremely rareTreatmentvSurgery is advised for all symptomatic patients and also for asymptomatic patients wi

19、th a shunt ratio of at least 2:lvThe timing for elective closure is usually at some time prior to entry schoolvOcclusion devices implanted by interventional cardiac catheterizationASD封堵器封堵器Interventional TreatmentVentricular Septal Defect（VSD）The most common cardiac malformation accounting for 25-50

20、%of CHDClassification of VSDv Membranous VSD most common defectv Outlet(supracristal,infundibular,subpulmonary)VSD situated just beneath the pulmonary valvev Inlet VSDv Muscular VSDAO LV RVH PA LA PV Hyperkinetic pulmonary hypertension extremely large pulmonary blood flow Obstructive pulmonary hyper

21、tension pulmonary arteriolar medial thickness pulmonary vascular obstructive disease RVP RVPLVP RV to LV Shunt cyanosis-Eisenmenger syndromeVSDPathophysiology of VSDPathophysiology of VSDv The degree of Left-to-Right shunting is dependent on:1.size of defect 2.pulmonary and systemic vascular resista

22、ncesv Left to right shunt is only in systole because the LV pressure is higher than the RV pressure in systole,and the both pressure is equal in diastolePathophysiology of VSDv The large blood flow through the VSD results in:l.Enlargement of LV and LA(volume overload),dilatation of PA,enlargement of

23、 RV when pulmonary hypertension 2.Pulmonary hypertension:early:extremely large pulmonary blood flow Hyperkinetic pulmonary hypertension late:pulmonary arteriolar medial thickness and pulmonary vascular obstructive disease Obstructive pulmonary hypertension When the ratio of pulmonary to systemic res

24、istance approaches 1:1,the shunt become bidirectional,signs of heart failure abate,and the patient becomes cyanosis-Eisenmenger syndromeClinical Manifestations according to the size of defect and the pulmonary blood and pressureSmall VSDv Asymptomaticv A loud,harsh,or blowing holosystolic murmur at

25、the 3rd-4th intercostal space of LSB with thrillv Chest X-ray:normal or minimal cardiomegaly and a borderline increase in pulmonary vasculaturev ECG:normal or left ventricular hypertrophyLarge VSDv Dyspnea,feeding difficulties,poor growth,profuse perspiration,recurrent pulmonary infections and cardi

26、ac failure in early infancyv Prominence of the left precordium and sternum,parasternal lift and systolic thrillv A loud,harsh,or blowing holosystolic murmur at the 3rd-4th intercostal space of LSBv P2 louder when pulmonary hypertensionv Mid-diastolic,low-pitched rumble at the apex:increased blood fl

27、ow across the mitral valveLarge VSDChest X-ray:v Gross cardiomegaly with prominence of LV,RV,LA and PAv Pulmonary vascular markings increase and frank pulmonary edema may be presentv Aortic knob is smallECG:Biventricular hypertrophy,P waves may be notched or peakedEchocardiographyv Enlargement of th

28、e LA and LV,and RV and PA in patients with pulmonary hypertensionv Position and size of VSDv Direction and degree of shunt in VSD Catheterization Oxygen saturation in RV is higher than in RARV and PA pressure are normal or increaseRight cardiac catheter can enter LV and Aorta through defect in some

29、very large VSD patients Prognosis vThe natural course of VSD depends to a large degree on the size of the defect 30-50%of small perimembranous and muscular VSD will close spontaneously,most frequently during the 1st yr of life.The vast majority of defects that close will do so before age 45 yr.It is

30、 less common for moderate or large defects to close spontaneously.Outlet VSD is no possible to close spontaneouslyComplicationsv The large VSD:repeated episodes of respiratory infection,congestive heart failure(CHF)and failure to thrive,and pulmonary hypertensionv Infective endocarditisTreatmentv Sm

31、all VSD does not need restrictions of physical activity and surgery repair.These patients can be followed by a combination of clinical examinations and noninvasive laboratory tests until the defects has closed spontaneouslyv Large and outlet VSD needs medical management to control CHF and to prevent

32、 the development of pulmonary vascular disease,and surgery repair early in patients with pulmonary hypertension肌部肌部VSD 封堵器封堵器Interventional TreatmentInterventional Treatment Patent Ductus Arteriosus (PDA)v During fetal life,most of the pulmonary arterial blood is shunted through the ductus arteriosu

33、s into the aorta.Functional closure of the ductus normally occurs soon after birthv The aortic end of the ductus is just distal to the origin of the left subclavian artery,and the ductus enters the pulmonary artery at its bifurcationFigure of PDA AO PA PV LV LA Hyperkinetic pulmonary hypertension ex

34、tremely large pulmonary blood flow Obstructive pulmonary hypertension pulmonary arteriolar medial thickness pulmonary vascular obstructive disease PAPAOP RVP RVH PA to AO Shunt Low extremity cyanosis(differential cyanosis)-Eisenmenger syndromePDAPathophysiology of PDAPathophysiology of PDADifferenti

35、al CyanosisPathophysiology of PDAv The degree of Left-to-Right shunting is dependent on:1.the size of the ductus 2.ratio of pulmonary and systemic vascular resistancesv Left to right shunt can occur both in systole and diastole because the aortic pressure is always higher than the pulmonary arterial

36、 pressure both in systole and diastolePathophysiology of PDAv The large blood flow through the PDA results in:1.enlargement of LV and LA(volume overload),dilatation of PA,enlargement of RV when pulmonary hypertension 2.pulmonary hypertension:early:extremely large pulmonary blood flow-Hyperkinetic pu

37、lmonary hypertension late:pulmonary arteriolar medial thickness and pulmonary vascular obstructive disease-Obstructive pulmonary hypertension 3.when the ratio of pulmonary to systemic resistance approaches 1:1,the shunt becomes bidirectional,signs of heart failure abate,and the patient becomes cyano

38、sis(differential cyanosis)-Eisenmenger syndrome Pathophysiology of PDAv Small PDA:PAP,RVP and RAP are normalv Large PDA:PAP may be elevated to systemic levels during both systole and diastoleClinical ManifestationsAccording to the size of the ductus:v Small PDA:Asymptomaticv Large PDA:poor feeding a

39、nd retardation of physical growth,sometimes with hoarseness Clinical ManifestationsvProminence of the left precordium and apical impulse in large PDAvWide pulse pressure(40 mmHg)and bounding arterial pulse:peripheral blood vessel sign due to runoff of blood into pulmonary artery during diastolevThri

40、ll,maximal in the 2nd left intercostal space,may radiate toward the left clavicle,down LSB or toward apex,usually in systole,but also may be throughout the cardiac cycleClinical ManifestationsvClassic murmur:1.Continuous,machinery and rolling thunder murmur 2.Begining soon after onset of the 1st sou

41、nd,reaches maximal intensity at the end of systole,and wanes in late diastole 3.Located at the 2nd left intercostal space and radiate down the LSB or to left clavicle 4.When pulmonary vascular resistance increases or heart failure,the diastolic component of the murmur may be less prominent or absent

42、 Chest X-rayv Prominent pulmonary artery with increased intrapulmonary vascular markingsv Normal or minimal cardiomegaly in small PDA and marked cardiomegaly in large PDA.The chambers involved are LA and LV,and RV when pulmonary hypertensionv Aortic knob is prominentECGv normal in small PDAv left ve

43、ntricular or biventricular hypertrophy in large PDAEchocardiographyv enlargement of the LA and LV,and RV and PA in patients with pulmonary hypertensionv position,shape and size of PDAv direction and degree of shunt in PDA Catheterization 1.Oxygen saturation in PA is higher than in RV2.RV and PA pres

44、sure are normal or increase3.Right cardiac catheter can enter descending aorta through PDA in most of patients4.Angiocardiography is needed in some patients with very tiny PDAPrognosis and Complicationsv The patients with a small PDA may live a normal span with few or no cardiac symptomsv Spontaneou

45、s closure of the ductus after infancy is extremely rarev The repeated episodes of respiratory infection,congestive heart failure,failure to thrive and pulmonary hypertension are common in large PDAv Infective endocarditis may be seen at any age,pulmonary and systemic emboli may occurv Aneurysmal dil

46、atation of the pulmonary artery or the ductusTreatmentv PDA in premature infant:indomethacinv Irrespective of age,patients with PDA require treatment(including surgical and interventional closure)in order to prevent infective endocarditis,congestive heart failure and the development of pulmonary vas

47、cular diseaseInterventional Treatment Those patients with left to right shunt(VSD,PDA,ASD,et)whose shunts have become partially or totally right to left as a result of the development of pulmonary vascular disease 所有左向右分流的先天性心脏病（所有左向右分流的先天性心脏病（ASD、VSD、PDA 等）由等）由于器质性肺血管病变导致梗阻性肺动脉高压，右心系统压力于器质性肺血管病变导致梗

48、阻性肺动脉高压，右心系统压力等于或超过左心系统，出现双向或右向左分流等于或超过左心系统，出现双向或右向左分流Eisenmenger syndrome 艾森曼格综合征艾森曼格综合征Tetralogy of Fallot（TOF）Pathophysiologyv Obstruction to right ventricular outflow tract(infundibular stenosis)-the most important malformation(sometimes with pulmonary valve and artery stenosis)v Ventricular sep

49、tal defect large and nonrestrictive VSD just below the aortic valvev O v e r r i d e o f t h e a o r t a(dextroposition of the aorta in 25%patients)v Right ventricular hypertrophyPathophysiologyv The degree of right ventricular outflow obstruction determines the timing of onset of symptoms,the sever

50、ity of cyanosis,and the degree of right ventricular hypertrophyv The degree of right ventricular outflow obstruction and override of the aorta can become more and more severe with growth Obstruction to right ventricular outflow tract Spasm of Stenotic Infundibulum Hypoxic Spells RVP RVPLVP Pulmonary

51、 Blood Flow Collateral Artery From DAO RVH Override of the aorta RV to LV Shunt Gas Exchange in Lung AO Blood from LV and RV Artery Oxygen Saturation Cyanosis Polycythemia Clubbing Squatting Cerebral thromboses Brain abscess VSDPathophysiology of TOF Clinical ManifestationsvCyanosis Often is not pre

52、sent at birth,but with increasing hypertrophy of the RV infundibulum and growth,cyanosis occurs later in the 1st yr of life It is most prominent in the mucous membranes of the lips and mouth,and in the fingernails and toenails In infants with severe RV outflow obstruction,cyanosis is noted immediate

53、ly in the neonatal period vParoxysmal hypercyanotic attacks (hypoxic spells)1.Spasm of stenotic infundibulum pulmonary blood flow most of all RV blood into aorta severe systemic hypoxia and metabolic acidosis 2.A particular problem during the first 2 yr of life 3.Hyperpneic,restless,cyanosis increas

54、es,gasping respirations ensue and syncopeClinical ManifestationsClinical Manifestations4.Frequently in the morning upon first awakening or following episodes of vigorous crying5.Temporary disappearance or decrease in intensity of the systolic murmur due to the decrease of flow across the RV outflow

55、tract6.Last a few minutes or hours,rarely fatal.Followed by generalized weakness and sleep.Sevre spells may progress to anconsciousness and mnvulsions or hemiparesiClinical ManifestationsvClubbing of fingers and toesvDyspnea and Squatting l.Squatting systemic artery pressure and resistance increase

56、LVP increase 2.Squatting venous return decrease RVP decrease l,2 Right to left shunt decrease through VSD and hypoxia can be improvedvGrowth and development may be delayed in patients with severe untreated tetralogy of Fallotv Left anterior hemithorax may bulge anteriorly due to RV hypertrophyv Subs

57、ternal right ventricular impulsev Systolic thrill in the 3rd and 4th parasternal spaces along LSB in 50%casesClinical ManifestationsClinical Manifestationsv Systolic Murmur 1.ejection or holosystolic,loud and harsh 2.most intense in the 2nd-4th intercostal space of LSB 3.caused by turbulence through

58、 the RV outflow tract due to the infundibular stenosisv The 2nd heart sound is single or the pulmonic component is softChest X-rayv Narrow base,concavity of pulmonary artery and normal heart sizev Rounded apical shadow above the diaphragm than normalv Pulmonary vascular marking decreasev Large aorta

59、 and 25%cases with dextroposition of aortic archv Cardiac silhouette-boot or wooden shoeECGRight axis deviation and RVHEchocardiographyv The aorta widen and override on the large ventricular septal defect v Obstruction to right ventricular outflow tract(infundibular stenosis)v Right ventricular hype

60、rtrophyv Left ventricular is smallCardiac Catheterization and Angiocardiographyv RVP increases and equals to the LVPv Oxygen saturation in Ao and LV decreasesv Angiocardiography in RV and LV can show the position and size of VSD,size of LV，width and overriding degree of aorta，and degree of obstructi

61、on of RVOT,et.Prognosis and Complicationsv Cerebral thromboses 1.usually in cerebral veins,occasionally in cerebral arteries mon in the presence of extreme polycythemia and dehydration under the age of 2 yrv Brain abscess usually over the age of 2 yrv Bacterial endocarditisv Congestive heart failare

62、 unusually in TOFTreatment of hypoxic spells Depending on the frequency and severity of hypercyanotic attacks,one or more of the following procedures should be instituted in sequence:1.Knee-chest position 2.Administration of oxygen 3.injection of morphine subcutaneously in a dose not in excess of 0.

63、2 mg/kg 4.Correction of metabolic acidosis with intravenous administration of sodium bicarbonate if the spell is unusually severe and there is lack of response to the foregoing therapy 5.-Adrenergic blockade by intravenous administration of propranolol(0.1 to a maximum of 0.2 mg/kg)6.Drugs that increase systemic vascular resistance,such as intravenous methoxamine or phenylephrine,decrease the right-to-left shuntTreatment of TOFDepend on the severity of the RVOT obstructionvpalliative surgeryvtotal correction谢谢谢谢Thank You