1、Pediatric and Congenital Heart Disease Common ProblemsCommon ProblemsASD,VSD,PDA and TOFXinhua Childrens Hospital,School of Medicine,Shanghai Jiaotong UniversityKun Sun M.D.，Ph.D.Heart StructurelHeart is an complex spatial structurelDifferences in the pathophysiology of cardiac diseasePediatrics:con
2、genital heart diseaseAdults:atherosclerotic heart disease lClinical presentation-raising the suspicionlImage is EVERYTHINGlMainstay:echocardiographyecho anatomy and hemodynamicslCardiac catheterizationimaginghemodynamicsinterventionStages of Heart Formationl1.Early blood vessel formationl 1)Intraemb
3、ryonic blood vessel at 13 daysl 2)Extraembryonic blood vessels at 17 daysl2.Development of heartl 1)Position and cardiac tube at 22 days l Heart beating at 26 days.l 2)Formation of heart loop at 22-24daysl 3)Formation of ventriclel 4)Development of sinus venosusl3.Formation of cardiac septa,valves,l
4、 arterial system,systemic veinsl 2 weeks formation start l 4 weeks circulation startl 8 weeks four chamber heart Incidence of Congenital Heart Diseasesl1.Lt to Rt Shunt (53%)l PDA 17%l ASD 16.5%l VSD 13%l AVSD 3.5%l Abn.PV return 3%l2.Rt to Lt Shunt (11%)l TOF 4.5%l TA 3%l PA+VSD 2.5%l PA+IVS 0.5%l3
5、.Admixture Lesion(15%)l TGA 5%l Univ.Ht.5%l Atrial isomerism 2%l DORV 2%l Truncus 0.8%l Corrected TGA 0.5%l4.Obstructive Lesion (15%)l Coarctation 9.5%l PS 2%l MS etc.1.5%l LVOTO 1.3%l HLHS 0.9%l IAA 0.6%l5.Valvular Lesionl Ebstein 1%l AR 0.5%l MR 0.5%l SV aneurysm 0.5%l6.Miscellaneousl Arrhythmia 5
6、%l Vascular ring 0.5%Fetal CirculationParallel circulation(combined output)Ductus Venosus shunts blood from the UV to the IVC bypassing the liver Foramen Ovale shunts blood from the RA to the LADuctus Arteriosus shunts blood from the PA to the descending aorta bypassing the lungsBlood from the LV pe
7、rfuses the heart&brain with well oxygenated blood50%2/3Transitional Circulation Dramatic changes in circulation at the moment of birth and onwards:Air breadth-lung expansion-Rp Qp -LA pressure -PFO P O2 -ductus arteriosus and venosus Obliteration of placental circulation -Rs IVC pressure -PFO Forame
8、n OvalelClosure occurs in two stagesFunctional closure occurs when LA pressure higher than RA pressurelThis is reversible in the presence of hypoxemia or hypovolemiaPermanent closure occurs in 5-7 monthslProbe PatencyIs present in 50%of children 5 years old&in more than 25%of adultsIs a potential av
9、enue for air emboli to enter the systemic circulationA patent FO may be beneficial in certain heart malformationsPatients who rely on the patency of the foramen require a balloon atrial septoplastyDuctus ArteriosuslClosure occurs in two stages80%Functional closure occurs 24 hours after birthlThis is
10、 reversible in the presence of hypoxemia or hypovolemia80%Permanent closure occurs in 3 months95%Permanent closure occurs in 6 monthslFibrous connective tissue forms&permanently seals the lumenThis becomes the ligamentum arteriosum The PDA in the preterm infant is due to a weak vasoconstrictor respo
11、nse to O2 and should be considered a normal not pathologic responselThis PDA may still need surgical correctionlA left to right shunt through the ductus can flood the lungs of the premature infant prolonging mechanical ventilation,eventually leading to pulmonary edema&right sided heart failurelA PDA
12、 may also be beneficialIn cyanotic congenital heart malformations with right to left&decreased pulmonary blood flowlThe PDA may be the major route by which the blood reaches the pulmonary arteries to receive O2lIn this case closure of the DA causes severe cyanosis,tissue hypoxia&acidemialTo keep the
13、 ductus open prior to palliative or corrective surgery of the heart malformation,PGE 1(0.05-0.1mcg/kg/min)can be administered IVlTo help close the ductus prior to surgical intervention to ligate the PDA,Indomethacin(0.1-0.2mg/kg)can be administeredThis is an inhibitor of PGE synthesisTransitional&Ne
14、onatal CirculationlThere are gross structural differences&changes in the heart during infancyAt birth the right&left ventricles are essentially the same in size&wall thicknessDuring the 1st month volume load&afterload of the LV increases whereas there is minimal increase in volume load&decrease in a
15、fterload on the RVlBy four weeks the LV weighs more than the RVlThis continues through infancy&early childhood until the LV is twice as heavy as the RV as it is in the adultlParameters are much different for the infant than for the adultHeart rate:higherlDecreasing to adult levels at 5 years oldCard
16、iac output:higherlEspecially when calculated according to body weight&it parallels O2 consumptionCardiac index:constantlBecause of the infants high ratio of surface area to body weightO2 consumption:depends heavily on temperaturelThere is a 10-13%increase in O2 consumption for each degree rise in co
17、re temperature Circulation Variables in InfantsNormal range of systolic pressure=(age*2）+80*0.133kPa 2.67kPa Hypertension Hypotension 16 weeksPurposes of CatheterizationlAnatomic diagnosislHemodynamic assessmentlInterventional procedureEquipmentlBiplane monitor/Cine with digital subtraction lPatient
18、 monitoring:EKG,BP,pulse oximeterlPhysiologic signal amplifier and recording devicelBlood gas,O2 consumption,Dye/ThermodilutionlEmergency treatment tools:lRoom for Others:anesthesia,echo,exerciseCatheterization RoomFluoroscopic Monitor Physiologic Signal-Display&RecordingElectrophysiologic StudyOthe
19、r Imaging ToolslMagnetic Resonance Imaging(MRI)lCT/Electron-beam CT(EBCT)lRadionuclide/SPECTlPositron Emission TomographyMagnetic Resonance Imaging Spin echo Gradient echo Velocity encoded Magnetic Resonance ImaginglSectional still image/cine image/3-DlFlow information/volume flow lLess window depen
20、dant/post-op study,older age/functional evaluationComputerized TomographyRadionuclide StudyRadionuclide StudylIn aggregate,congenital heart disease(CHD)comprises a relatively large percentage of all malformations.lThe effect on the patient depends on the severity and type of the malformation,but can
21、 range from lethal to clinically insignificant.l Prevalence:All births:1.56 to 7.7/1000Stillbirths:1 to 34.5/1000Live births:2.0 to 10.2/1000Affected by mortality,diagnostic criteria,length of follow-up,etc.Etiologic Basis of Congenital Heart Diseasesl1.Primary genetic factors (10%)l 1)Chromosomal 5
22、-10%l 2)Single mutant gene 3%l Recessivel Dominantl2.Genetic-environmental interaction (90%)l 1)Multifactorial inheritance,majorityl 2)Risks to offspring of an affected parentl 3)Environmental contributionl Drugsl Infectionsl Maternal conditionsl1.Drugsl Alcoholl Amphetaminesl Anticonvulsantsl Chemo
23、therapyl Sex hormonel Thalidomidel l2.Infectionsl Rubella l Coxsakie virusl3.Maternal conditionsl Old agel Diabetesl Lupusl Phenylketonurial4.OtherslLeft to Right Shunt Type（potential cyanotic type）ASD VSD PDAlRight to Left Shunt Type（cyanotic type）TOF TGA DORVlNonshunt Type（Acyanotic type）PS COA AS
24、Atrial Septal DefectlLeornardo da Vinci described the patent foramen ovale in the early 1500s:“I have found a perforating channel from left auricle to right auricle”lIn 1875,Karl von Rokitansky provided a superb account of pathological anatomy of the atrial septal defect together with its embryologi
25、cal basislHe even distinguished between primum and secundum defectsAtrial Septum lPrimitive atrium partitions by growth of septum primumlAtrial communication maintained via ostium primum(OP)lBefore closure of OP fenestrations develop in septum primumlSeptum secundum develops just to the right of sep
26、tum primumlOpening through septum secundum on the RA side with a flap valve on the LA side is the foramen ovale30 days33 days37 daysNew bornFrom Moss,1989Atrial Septal DefectlAccounts for 20%of CHD lTwo to three times more common in women lMay go unrecognized for decades lUnoperated survival beyond
27、age 40-50 is no more than 50%with a subsequent increase in mortality at 6%per year after age 50Atrial Septal DefectlOstium secundum defectMost common(60-70%)Mid portion of IAS(FO site)Isolated defect associated with MVPlOstium Primum defect15-20%of ASDsInferolateral portion of IASFrequently associat
28、ed with cleft in anterior MV leaflet and MRAtrial Septal DefectlSinus Venosus defect5-10%of ASDsSuperior and posterior in relation to fossa ovalisAlmost always associated with PAPVD into RA or IVC/SVClCoronary sinus defect50 mmHg),and CV symptomsl15%of CHDlrisks and history that of the shunt magnitu
29、delsmall,restrictive-asymtpomatic,risk of endocarditislmoderate to large-heart failure,arterial“steal”Patent ductus arteriosusPatent Ductus ArteriosisAnatomical:tube type,funnel type,window type Diameter：small 0.3cm;medium 0.5cm;largr 1.0cmlR-to-L shunts:CYANOSIS in 1year after birth(or diagnosis if
30、 tetralogy of Fallot),prevalence 10%of CHDlBasic abnormality is malalignment of the outflow(infundibular)portion of the ventricular septumlTetrad:malalignment of outlet septum-VSDaortic over ridepulmonary outflow tract stenosisright ventricular hypertrophy Embryology of Tetralogy of FallotPathology
31、of Tetralogy of Fallot Infundibular deviated to anterior,superior and leftPathology of Tetralogy of FallotlRVOTO1.50 RVOTO2.20%50 PS3.Superior pulmonary valve stenosis or peripheral pulmonary stenosis or absent left pulmonary artery4.Asssociated heart malformation 40inner heart malformation 2030out
32、heart malformation 20%right sided aortic arch Tetralogy of FallotTetralogy of FallotlDegree of the right to left shunt is related to the degree of right ventricular outflow obstruction.Outflow tract stenosis becomes worse with growth.lPulmonary hypertension is not a problem,but infective endocarditi
33、s and“paradoxical emboli”(including brain abscesses)are potential complications.lpresentation typically in infancyMurmur-present from initial assessmentCyanosis if outflow obstruction is severeRange of presentation a reflection of the variability of outflow obstructionMost critical obstructive lesio
34、n-pulmonary atresia may be associated with hypoplasia of the pulmonary arterieslOccur at 2m9mA paroxysm of hyperpnea(rapid and deep respiration)increasing cyanosisdecreased intensity of the heart murmurMechanism of tet spellcyanosis cyanosis RVOT contract RVOT contract R RLshuntLshunthyperpneaSat%Sa
35、t%Venous returnVenous returnR RLshuntLshunthyperpneaDiscussion for ASD,VSD,PDA,TOFlClinical FeatureslPhysical ExaminationlEKGlChest X-raylTreatmentCase study30Atrial Septal Defect-Clinical FeatureslPresent late Left right shunt,amount based on defect size and compliance of pulmonary and systemic cir
36、culationAt birth right ventrical is thick,thins out and pulmonary resistance decreases,increase in shuntlRight ventricular volume overload Subtle failure to thrive,fatiguelProminent pulmonary flow Recurrent pulmonary infectionslInsufficient systemic flow poor development,thin,sweating and failure to
37、 thrivelPotential cyanotic crying,pneumonia,CHFlOn examination may detect RV impulselMay also detect a palpable P2(dilated pulmonary trunk)lS1 is normal but there is a fixed split S2lFlow across the ASD does not produce a murmurlA soft SEM is often audible in 2nd LICS secondary to rapid ejection of
38、a large RV stroke volume into a dilated PAlA murmur grade 3/6 often means that a large shunt is present or there is an associated PS presentlThere may be a mid-diastolic rumble from increased flow through TVlWhen RV failure ensues PSM of TR is heard1)Right axis deviation RVH2)IRBBB rsR RVH3)Prolong
39、P-R period crest hypertrophy、RV dilation4)High P wave RA enlargement 5)Left axis deviation Ostium Primum defectASD-CXRCardiomegalyPulmonary plethoraRV enlargementProminent PAEchocardiography1)Dilated RA，RV，RVOT Right heart volume overload 2)Flat ventricular septum3)ASD signEchocardiographylDilated R
40、A，RV，RVOT l Right heart volume overload lFlat ventricular septuml ASD signCatheterization1)When？a)Clinical outcome not match the diagnosis b)Suspicions of pulmonary hypertension 2)To measure:a)Oxygen Saturation RAIVC,SVC b)amount of Shunt c)Pulmonary pressure and resistanceClinical course and treatm
41、entlWell tolerated in childhood,symptoms may appear after 2nd decade,eventual pulmonary hypertension,atrial dysrythmia,and tricuspid insufficiency.Heart failurelTreatment:Surgical correction at 2-6 years for all symptomatic patients and asymptomatic patients with pulmonary blood flow 2X systemic eve
42、ntual pulmonary hypertension,atrial dysrythmia,and tricuspid insufficiency.Operate earlier if with prominent symptom or with CHFlIndication1)Defect size 8kglDevice1)Sideris2)Cardio Seal3)Amplazer4)Cribriform Cribriform applies to multifenestrated ASDv self-expandable double equal disk structurev 3 m
43、m length connection sectionv polyester fiber packing insideFully Biodegradable Occluderlwovened by PDO sutureRA sideLA sideFully Biodegradable OccluderPre-interventionDuring interventionDuring interventionDuring interventionDuring interventionASD-DevicelArterial pulse is often normallThere may be a
44、systolic thrill on palpation of the precordium(maximal in 3rd or 4th ICS)lPansystolic,high frequency murmur(grade 4-6/6)with small VSD and normal PAPlOnce PAP increases above the systemic pressures the holosystolic murmur disappearslIncrease flow across pulmonary valve causes a SEM lA loud P2 compon
45、ent is heard in this settinglClinical severity grading:Small:Causes negligible Hemodynamic changes.LV size normal w/o PHTNModerate:Causes LV and LA enlargement,and usually some PHTN(reversible)Large:Results in pulmonary vascular obstructive disease and Eisenmenger physiology unless there is coexiste
46、nt RVOTOlPathologic and surgical classification:Perimembranous:bordered by fibrous continuity of an AV valve and an arterial valve,usually with inlet or outlet extensionMuscular:bordered by muscular rim,usually trabecularDoubly committed:bordered by fibrous continuity of both the aortic and pulmonar
47、y valves lMay be normal but often shows LVH and LAElPresence of RAD represents elevated RVP and PAPlPostoperative RBBB is commonCXR in VSDlCardiomegaly with LAE and LVE will be seen with large L to R shuntslA large defect associated with a small heart and oligemic lung fields should raise the suspic
48、ion of pulmonary vascular diseaselWhen repair is performed in the first two years of life,asymptomatic adult survival with normal growth and development can be anticipatedlWhen surgery is undertaken in older children,a late postopeartive increase in LV chamber size,together with decreased systolic f
49、unction is seenlDevelopment of late postoperative PHTN is largely determined by the age at surgery and preoperative PVRlRisk of SBE persists and requires prophylaxislif symptomatic,or signs of significant shuntlmedical therapy with digoxin and diureticslsurgical closure indicationspatch closure,usin
50、g cardio-pulmonary bypasspersistent large shunt,CHF and growth failurepersistent cardiomegaly by echo or catheterizationloutcome following surgery excellentloften undertaken in infancy if patients with pulmonary blood flow 2X systemic eventual pulmonary hypertensionlOperationlInterventional therapy
