主動(dòng)脈弓變異課件

1、Aortic Arch Anomalies1Development of Aortic Arch and great vessels2345781011121415171819202122Anatomical CategoriesAbnormalities of branchingNormal L Aortic Arch & VariantsAbnormal L Aortic ArchAbnormalities of arch position 3. R Aortic Arch4. Cervical Aortic ArchSuperpneumarary arches 5. Double Aor

2、tic Arch6. Persistent Fifth AA7. Interrupted Aortic Arch 8. Anomalous origin of PA branches and other AA anomalies24Clinical ClassificationVascular ringsNon ring vascular compression of trachea, bronchi, oesophagusNon compressive arch malformationDuct dependent arch anomalies25Sidedness of Aortic ar

3、chL & R aortic arch definitionsRefers to which bronchus is crossed by the archNormal Cross the L main bronchus at T5Branching. general rule 1st arch vessel contain a carotid a. contralateral to Ao AImportance of sidedness of Ao archBT shunt on side of In ARepair of oesophageal atresia side opp arch2

4、7Anatomical CategoriesAbnormalities of branchingNormal L Aortic Arch & VariantsAbnormal L Aortic ArchAbnormalities of arch position 3. R Aortic Arch4. Cervical Aortic ArchSuperpneumarary arches 5. Double Aortic Arch6. Persistent Fifth AA7. Interrupted Aortic Arch 8. Anomalous origin of PA branches a

5、nd other AA anomalies281. Normal L Aortic Arch & VariantsVariants1. Common brachiocephalic trunk Present in 10% of L archesNo consequences291. Normal L Aortic Arch & VariantsVariants2. Separate origin of L vertebral a. from aortic arch (normal from L subclavian)Size 12, 3 that of TOF8% of DTGA, 16%

6、of TGA+VSD+PS have RAA433.1 RAA with Mirror Image BranchingAlmost always ass. with congenital intracardiac diseaseConotruncal anomalies TOF, TA, TGA, DORV, LTGA, PA with RV aortaOther lesions VSD, PA with IVSDuctus is commonly L sided - attached to L innom. A. no vascular ring443.1 RAA with Mirror I

7、mage BranchingDiagnosisUsually no retro-oesophageal compression/ vascular ringEcho/AngioDistinctive branching patternCxR/ Ba oesophagography R indentation of trachea/oesophagusTreatment RAA only - No Rx needed45Variant L ductus to RE diverticulum from R Desc AoVascular ringNo arch vv from diverticul

8、um(Rarely true mirror image of normal L ductus disappear and R 6th arch continue as ductus)3.1 RAA with Mirror Image Branching46 vascular ring+Many asymptomatic, in most no other heart defect3.2 RAA with Retro-oesophageal diverticulum (Of Kommerell)47DiagnosisPresentation vascular ring+CxR R AA ? RE

9、 Div of ComBa OesophagogramEchoAngio charact branching pattern, abrupt change in caliber from diverticulum to SCAMRI 3.2 RAA with Retro-oesophageal diverticulum (Of Kommerell)483.2 RAA with Retro-oesophageal diverticulum (Of Kommerell)RxSymptomatic Sx division of ligamentum (L thoracotomy/ Median st

10、ernotomy)If resp symps/ dysphagia resection of entire diverticulum (R thoracotomy)49Loss of L 6th ductal arch and persistence of R 6th No vascular ringSmaller posterior indentation of Oesophagus Rx not needed (no ring) except for ass anomalies3.3 R AA with Retro-oesophageal L SCA50DiagnosisCxR, Ba S

11、tudyEcho branching pattern + L desc AoAngio difficult to DD from Normal L AA go by branching patternMRIRx when symptomatic need division3.4 R AA with L Desc Ao & L ductus51Vascular ring+ Very rareSite of arch dissolution L branch of aortic sac(Exception to the general rule 1st arch vessel contain a

12、carotid a. contralateral to Ao A.)3.5 R AA with Retro-oesophageal Innom A.52DiagnosisSingle carotid A. arising from prox. AortaDD interrupted AA, isolated L carotid/Innominate A.Differentiating feature normal size AARxDivision of the ring if symptomatic if still symptomatic detachment of Inn a and r

13、eimplantation in to AA3.5 R AA with Retro-oesophageal Innom A.533.6 RAA with isolation of contralateral arch vesselsUncommon Vessel arises exclusively from PA via ductus arteriosus without connection to aorta 3 different forms CHD + in 50% of cases 2/3 have TOF Most common isolation isolated SCA54 I

14、solation of L SCADissolution L 4th arch & L distal dorsal Ao3.6 RAA with isolation of contralateral arch vessels552. Isolation of L CCADissolution L 4th arch & L horn of aortic sac with 6th arch connecting to 3rd arch3.6 RAA with isolation of contralateral arch vessels563. Isolation of L Innom. A Di

15、ssolution L horn of aortic sac and distal L dorsalaorta3.6 RAA with isolation of contralateral arch vessels57Clinical F.Low pulse volume/ BP in affected arteryWhen subclavian and vertebral A are involved subclavian steal syndromeCerebral insufficiency, L arm ischaemia If ductus remain patent PA stea

16、l (flow down vertebral a. in to low res. PA)Suspect RAA+ low pulse in L UL3.6 RAA with isolation of contralateral arch vessels58DiagnosisAngio delayed filling of SCABA oesophagography not helpfulDoppler echo reversal of flow in vertebral arteryRxRepair of CHD + ligation of ductus if patent to preven

17、t stealCNS syms/ claudication of arm surgical reimplantation of SCA to aorta3.6 RAA with isolation of contralateral arch vessels59Anatomical CategoriesAbnormalities of branchingNormal L Aortic Arch & VariantsAbnormal L Aortic ArchAbnormalities of arch position 3. R Aortic Arch4. Cervical Aortic Arch

18、Superpneumarary arches 5. Double Aortic Arch6. Persistent Fifth AA7. Interrupted Aortic Arch 8. Anomalous origin of PA branches and other AA anomalies604. Cervical Aortic ArchRare anomalyAA above the level of clavicleTwo main subcategories614. Cervical Aortic ArchEmbryological explanationPersistence

19、 of ductus caroticus + involution of 4th arch 3rd arch becomes AA (int & ext carotid arising separately)Failure of the normal descent of AA At 3/52 of POA cephalic location at 7/52 POA intrathoracic location 624. Cervical Aortic ArchContralateral descending Ao. and Anomalous SCA Usually RAADescend t

20、o T4 level cross behind Oeso. to L gives off L SCA & Ductus vascular ring Ipsilateral descending aorta and normal branch patternTypically LAAnon ringAA obstruction due to long, tortuous, hypoplastic, retroesophageal segment634. Cervical Aortic ArchPresentations:Pulsatile masses in supraclavicualar f

21、ossa in neckDD aneurysm of carotid/ SCADifferentiation compression of pulsatile mass loss of femoral pulseVascular ringSubclavian steal syndrome CxRWide upper mediastinum + absent aortic knobAnterior deviation of trachea644. Cervical Aortic ArchRx necessaryIf hypoplasia of cervical arch+Symptomatic

22、vascular ringAneurysm of cervical arch itself65Anatomical CategoriesAbnormalities of branchingNormal L Aortic Arch & VariantsAbnormal L Aortic ArchAbnormalities of arch position3. R Aortic Arch4. Cervical Aortic ArchSuperpneumarary arches5. Double Aortic Arch6. Persistent Fifth AA7. Interrupted Aort

23、ic Arch 8. Anomalous origin of PA branches and other AA anomalies665. Double Aortic ArchBoth R & L arches persistVascular ring +Variations +Hypoplasia of one arch (usually L)Atresia of one arch (usually L)Both arches widely patentR arch is more superiorly located675. Double Aortic ArchDouble AA with

24、 both arches patentSymmetrical origin of 4 brachiocephalic Aa685. Double Aortic Arch2. Double AA with atretic L arch distal to the origin of L SCASimilar to mirror image RAA (but with L Desc Ao)Indistinguishable (except at Sx) from RAA with L DA 695. Double Aortic Arch3. Double AA with atretic segme

25、nt between L CCA and L SCASimilar to RAA with diverticulum of Kommerell705. Double Aortic ArchAtretic R archRareCan simulate L atresia patterns715. Double Aortic ArchDescending aorta could be L or RRarely ass. with CHD -TOF is most commonTGAEmbryological explanationBoth 4th arches and dorsal aortae

26、persistBut usually only one 6th arch (ductus)725. Double Aortic ArchClinical featuresvascular ring syms depend on tightness of ringWhen both arches widely patent tight ring stridor in 1st wkAtretic L arch loose ring present at 3-6/12 or laterRarely double AA present in adulthood with swallowing/resp

27、. symsDiagnosisCxR RAA indent trachea superiorly and LAA inferiorlyBa oeso, Echo, Angio, MRI confirm diagnosis 735. Double Aortic ArchMxIf symps + due to vascular ring Sx divisionIf undergoing Sx for other CHD divisionRing should be divided in the smaller limbLigamentum also should be divided74Anato

28、mical CategoriesAbnormalities of branchingNormal L Aortic Arch & VariantsAbnormal L Aortic ArchAbnormalities of arch position 3. R Aortic Arch4. Cervical Aortic ArchSuperpneumarary arches 5. Double Aortic Arch6. Persistent Fifth AA7. Interrupted Aortic Arch 8. Anomalous origin of PA branches and oth

29、er AA anomalies756. Persistent Fifth AA RareBoth arches appear on the same side of tracheaCan be ass with COA 3 Subtypes Except for COA 1st & 2nd subtypes no physiological significance 766. Persistent Fifth AA Double lumen AA with both lumina patentFrequently ass with major cardiac anomaly776. Persi

30、stent Fifth AA Atresia/interruption of the superior arch (4th) with patent inferior (5th) arch Common origin of all brachiocephalic vessels from the ascending aortaCan be ass with COA786. Persistent Fifth AA Systemic to pulmonary artery connection arising proximal to 1st brachiocephalic VvOnly in pu

31、lmonary atresia5th arch remnant arises as the 1st branch of the Asc Ao connects to the junction of MPA and one branch PAIpsilateral/contralateral to definitive AA (4th)796. Persistent Fifth AA Diagnosis“Subway” vessel beneath the normal archIn atresia of superior arch common brachiocephalic trunk wi

32、th all 4 vv arising from single v Branching pattern persistent 5th archAtretic segment not visualized in IxsAt Sx fibrous band + between L SCA and Desc Ao80Anatomical CategoriesAbnormalities of branchingNormal L Aortic Arch & VariantsAbnormal L Aortic ArchAbnormalities of arch position 3. R Aortic A

33、rch4. Cervical Aortic ArchSuperpneumarary arches 5. Double Aortic Arch6. Persistent Fifth AA7. Interrupted Aortic Arch 8. Anomalous origin of PA branches and other AA anomalies817. Interrupted Aortic ArchComplete separation of ascending and descending aortaDetermination of sidedness of AABranching p

34、attern- 1st Br. Prox to Int. contains a Carotid a. opposite the side of the AARetroesophageal/ isolated subclavian a is always opposite the side of the archImportance of sidednessInterrupted R AA only seen in ass with Digeorge syndrome827. Interrupted Aortic Arch3 main categories 9 sub categories Ma

35、in categoriesInterruption distal to SCA that is ipsilateral to 2nd Carotid AInterruption between 2nd carotid and ipsilateral SCAInterruption between carotid arteriesSubcategoriesWithout retro-esophageal or isolated SCAWith retro-esophageal SCAWith isolated SCA837. Interrupted Aortic ArchInterruption

36、 distal to SCA that is ipsilateral to 2nd Carotid Aassociations Aortico-pulmonary septal defects + Intact IVSTGA + Interrupted AA847. Interrupted Aortic ArchInterruption between 2nd carotid and ipsilateral SCAWithout retro-esophageal or isolated SCAMore common than type A 857. Interrupted Aortic Arc

37、hInterruption between 2nd carotid and ipsilateral SCAWith retro-esophageal SCADigeorge syndrome + interruption have type B867. Interrupted Aortic ArchInterruption between carotid arteriesRare877. Interrupted Aortic ArchAssociationsDigeorge syndrome Vs IAA / Truncus 43% of Digeorges had type B interr

38、uption68% of IAA had Digeorge34% of Digeorges had TA33% of TA had Digeorge887. Interrupted Aortic ArchPresentationDuct dependant L heart obstructive lesionsAcute cardiovascular collapse / heat failure after spont closure of PDA after 1st few days of lifeInitial MxFluid resuscitationInduction and mai

39、ntenance of ductal patency with PGE1Inotropic support SOSClinical featurespulse discrepancy depends on branching patternAbsence of all limb pulses type B interruption with anomalous SCA DD - critical AS (carotid pulse is also week)897. Interrupted Aortic ArchDifferential cyanosispink upper body + bl

40、ue lower bodyUncommonly seen bse pulm blood is also highly saturated due to large LR shunt through VSD907. Interrupted Aortic ArchDiagnosisEchocardiogram Most important tool for diagnosis of IAASuspect whenMarked discrepancy between Asc Ao and MPA + malalignment VSD + posterior deviation of infundib

41、ular septum (PS LAX)AngiographyDifficult bse high flow through VSD poor image quality of Asc AoCan diagnose when both carotids prox and both SCA distal to interruptionWide separation of carotids from Desc Ao IAA917. Interrupted Aortic ArchManagementSx approach depend on degree of subaortic obstructi

42、onSubaortic diameter 5-6 mm 1ry repair(patch closure of VSD + Ao Arch reconstruction)Subaortic diameter 3 mm inadequate to support normal COP927. Interrupted Aortic ArchPA banding is not a satisfactory palliation for VSD with interrupted Ao AWill lead to BVH with progressive subaortic stenosis compl

43、icate definitive repairRepair of Ao Archdirect anastomosis + homograft augmentation In infancy avoid artificial tube grafts Rapidly overgrownFibrous encasement complicate later repair93Anatomical CategoriesAbnormalities of branchingNormal L Aortic Arch & VariantsAbnormal L Aortic ArchAbnormalities o

44、f arch position 3. R Aortic Arch4. Cervical Aortic ArchSuperpneumarary arches 5. Double Aortic Arch6. Persistent Fifth AA7. Interrupted Aortic Arch 8. Anomalous origin of PA branches and other AA anomalies948. Other Anomalies of the Aortic Arch SystemAnomalous origin of the pulmonary artery from the

45、 ascending aortaAnomalous origin of the LPA from the RPAInnominate artery compression of the trachea958.1 Anomalous origin of the pulmonary artery from the ascending aorta One branch PA arising from Asc Ao + MPA arising separately from the heartRPA more commonly arise from Ao (82%) 968.1 Anomalous origin of the pulmonary artery from the ascending aorta InvestigationsCxR differential PBF (esp in TOF with oligemia)Echo diagnosticCarefully search for origins of both PAs in TOFCar