主动脉弓变异

1、1Aortic Arch Anomalies2Development of Aortic Arch and great vessels34567891011121314151617181920212223 24Anatomical CategoriesAbnormalities of branchingNormal L Aortic Arch & VariantsAbnormal L Aortic ArchAbnormalities of arch position 3. R Aortic Arch4. Cervical Aortic ArchSuperpneumarary arches 5.

2、 Double Aortic Arch6. Persistent Fifth AA7. Interrupted Aortic Arch 8. Anomalous origin of PA branches and other AA anomalies25Clinical Classification Vascular rings Non ring vascular compression of trachea, bronchi, oesophagus Non compressive arch malformation Duct dependent arch anomalies26Clinica

3、l features of vascular rings Stridor increase with RTI Recurrent pneumonia/ bronchitis Hyperextension of neck (esp. in infants) Reflex apnoea associated with eating Swallowing difficulty Chocking of food27Sidedness of Aortic arch L & R aortic arch definitions Refers to which bronchus is crossed by t

4、he arch Normal Cross the L main bronchus at T5 Branching. general rule 1st arch vessel contain a carotid a. contralateral to Ao A Importance of sidedness of Ao arch BT shunt on side of In A Repair of oesophageal atresia side opp arch28Anatomical CategoriesAbnormalities of branching Normal L Aortic A

5、rch & 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 other AA anomalies291. Normal L Aortic Arch & VariantsVariants

6、1. Common brachiocephalic trunk Present in 10% of L archesNo consequences301. 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% of TGA+VSD+PS have RAA443.1 RAA with Mirror Image Branching Almos

7、t always ass. with congenital intracardiac disease Conotruncal anomalies TOF, TA, TGA, DORV, LTGA, PA with RV aorta Other lesions VSD, PA with IVS Ductus is commonly L sided - attached to L innom. A. no vascular ring453.1 RAA with Mirror Image Branching Diagnosis Usually no retro-oesophageal compres

8、sion/ vascular ring Echo/Angio Distinctive branching pattern CxR/ Ba oesophagography R indentation of trachea/oesophagus Treatment RAA only - No Rx needed46 Variant L ductus to RE diverticulum from R Desc AoVascular ringNo arch vv from diverticulum(Rarely true mirror image of normal L ductus disappe

9、ar and R 6th arch continue as ductus)3.1 RAA with Mirror Image Branching47 vascular ring+ Many asymptomatic, in most no other heart defect3.2 RAA with Retro-oesophageal diverticulum (Of Kommerell)48 Diagnosis Presentation vascular ring +CxR R AA ? RE Div of Com Ba Oesophagogram Echo Angio charact br

10、anching pattern, abrupt change in caliber from diverticulum to SCA MRI 3.2 RAA with Retro-oesophageal diverticulum (Of Kommerell)493.2 RAA with Retro-oesophageal diverticulum (Of Kommerell) RxSymptomatic Sx division of ligamentum (L thoracotomy/ Median sternotomy)If resp symps/ dysphagia resection o

11、f entire diverticulum (R thoracotomy)50 Loss of L 6th ductal arch and persistence of R 6th No vascular ring Smaller posterior indentation of Oesophagus Rx not needed (no ring) except for ass anomalies3.3 R AA with Retro-oesophageal L SCA51 Diagnosis CxR, Ba Study Echo branching pattern + L desc Ao A

12、ngio difficult to DD from Normal L AA go by branching pattern MRI Rx when symptomatic need division3.4 R AA with L Desc Ao & L ductus52 Vascular ring+ Very rare Site of arch dissolution L branch of aortic sac(Exception to the general rule 1st arch vessel contain a carotid a. contralateral to Ao A.)3

13、.5 R AA with Retro-oesophageal Innom A.53 Diagnosis Single carotid A. arising from prox. Aorta DD interrupted AA, isolated L carotid/Innominate A. Differentiating feature normal size AA Rx Division of the ring if symptomatic if still symptomatic detachment of Inn a and reimplantation in to AA3.5 R A

14、A with Retro-oesophageal Innom A.543.6 RAA with isolation of contralateral arch vessels Uncommon 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 SCA55 Isolation of L SCA Dissolutio

15、n L 4th arch & L distal dorsal Ao3.6 RAA with isolation of contralateral arch vessels562. 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 vessels573. Isolation of L Innom. A Dissolution L horn of aortic

16、sac and distal L dorsalaorta3.6 RAA with isolation of contralateral arch vessels58 Clinical F. Low pulse volume/ BP in affected artery When subclavian and vertebral A are involved subclavian steal syndrome Cerebral insufficiency, L arm ischaemia If ductus remain patent PA steal (flow down vertebral

17、a. in to low res. PA) Suspect RAA+ low pulse in L UL3.6 RAA with isolation of contralateral arch vessels59 Diagnosis Angio delayed filling of SCA BA oesophagography not helpful Doppler echo reversal of flow in vertebral artery Rx Repair of CHD + ligation of ductus if patent to prevent steal CNS syms

18、/ claudication of arm surgical reimplantation of SCA to aorta3.6 RAA with isolation of contralateral arch vessels60Anatomical CategoriesAbnormalities of branchingNormal L Aortic Arch & VariantsAbnormal L Aortic ArchAbnormalities of arch position 3. R Aortic Arch4. Cervical Aortic ArchSuperpneumarary

19、 arches 5. Double Aortic Arch6. Persistent Fifth AA7. Interrupted Aortic Arch 8. Anomalous origin of PA branches and other AA anomalies614. Cervical Aortic ArchRare anomalyAA above the level of clavicleTwo main subcategories624. Cervical Aortic ArchEmbryological explanationPersistence of ductus caro

20、ticus + 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 634. Cervical Aortic ArchContralateral descending Ao. and Anomalous SCA Usually RAADescend to T4 level cros

21、s behind Oeso. to L gives off L SCA & Ductus vascular ring Ipsilateral descending aorta and normal branch pattern Typically LAA non ring AA obstruction due to long, tortuous, hypoplastic, retroesophageal segment644. Cervical Aortic ArchPresentations:Pulsatile masses in supraclavicualar fossa in neck

22、DD aneurysm of carotid/ SCADifferentiation compression of pulsatile mass loss of femoral pulseVascular ringSubclavian steal syndrome CxRWide upper mediastinum + absent aortic knobAnterior deviation of trachea654. Cervical Aortic ArchRx necessaryIf hypoplasia of cervical arch+Symptomatic vascular rin

23、gAneurysm of cervical arch itself66Anatomical 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 Aortic Arch 8. A

24、nomalous origin of PA branches and other AA anomalies675. Double Aortic Arch Both R & L arches persist Vascular ring + Variations + Hypoplasia of one arch (usually L) Atresia of one arch (usually L) Both arches widely patent R arch is more superiorly located685. Double Aortic ArchDouble AA with both

25、 arches patentSymmetrical origin of 4 brachiocephalic Aa695. 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 705. Double Aortic Arch3. Double AA with atretic segment be

26、tween L CCA and L SCASimilar to RAA with diverticulum of Kommerell715. Double Aortic ArchAtretic R archRareCan simulate L atresia patterns725. Double Aortic ArchDescending aorta could be L or RRarely ass. with CHD -TOF is most commonTGAEmbryological explanationBoth 4th arches and dorsal aortae persi

27、stBut usually only one 6th arch (ductus)735. 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. sym

28、sDiagnosisCxR RAA indent trachea superiorly and LAA inferiorlyBa oeso, Echo, Angio, MRI confirm diagnosis 745. 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 divided75Anatomical

29、 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 other AA

30、 anomalies766. Persistent Fifth AA Rare Both arches appear on the same side of trachea Can be ass with COA 3 Subtypes Except for COA 1st & 2nd subtypes no physiological significance 776. Persistent Fifth AA Double lumen AA with both lumina patentFrequently ass with major cardiac anomaly786. Persiste

31、nt 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 COA796. Persistent Fifth AA Systemic to pulmonary artery connection arising proximal to 1st brachiocephalic VvOnly in pulmo

32、nary 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)806. Persistent Fifth AA Diagnosis“Subway” vessel beneath the normal archIn atresia of superior arch common brachiocephalic trunk with

33、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 Ao81Anatomical CategoriesAbnormalities of branchingNormal L Aortic Arch & VariantsAbnormal L Aortic ArchAbnormalities of arch position 3. R Aortic Arch

34、4. Cervical Aortic ArchSuperpneumarary arches 5. Double Aortic Arch6. Persistent Fifth AA7. Interrupted Aortic Arch 8. Anomalous origin of PA branches and other AA anomalies827. Interrupted Aortic ArchComplete separation of ascending and descending aortaDetermination of sidedness of AABranching patt

35、ern- 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 syndrome837. Interrupted Aortic Arch3 main categories 9 sub categories Main

36、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 SCAA.With isolated SCA847. Interrupted Aortic ArchInterruption

37、distal to SCA that is ipsilateral to 2nd Carotid Aassociations Aortico-pulmonary septal defects + Intact IVSTGA + Interrupted AA857. Interrupted Aortic ArchInterruption between 2nd carotid and ipsilateral SCAWithout retro-esophageal or isolated SCAMore common than type A 867. Interrupted Aortic Arch

38、Interruption between 2nd carotid and ipsilateral SCAWith retro-esophageal SCADigeorge syndrome + interruption have type B877. Interrupted Aortic ArchInterruption between carotid arteriesRare887. Interrupted Aortic ArchAssociationsDigeorge syndrome Vs IAA / Truncus 43% of Digeorges had type B interru

39、ption68% of IAA had Digeorge34% of Digeorges had TA33% of TA had Digeorge897. 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 main

40、tenance 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)907. Interrupted Aortic ArchDifferential cyanosispink upper body + blu

41、e lower bodyUncommonly seen bse pulm blood is also highly saturated due to large LR shunt through VSD917. Interrupted Aortic ArchDiagnosisEchocardiogram Most important tool for diagnosis of IAASuspect whenMarked discrepancy between Asc Ao and MPA + malalignment VSD + posterior deviation of infundibu

42、lar 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 IAA927. Interrupted Aortic ArchManagementSx approach depend on degree of subaortic obstructio

43、nSubaortic diameter 5-6 mm 1ry repair(patch closure of VSD + Ao Arch reconstruction)Subaortic diameter 3 mm inadequate to support normal COP937. Interrupted Aortic ArchPA banding is not a satisfactory palliation for VSD with interrupted Ao AWill lead to BVH with progressive subaortic stenosis compli

44、cate definitive repairRepair of Ao Archdirect anastomosis + homograft augmentation In infancy avoid artificial tube grafts Rapidly overgrownFibrous encasement complicate later repair94Anatomical CategoriesAbnormalities of branchingNormal L Aortic Arch & VariantsAbnormal L Aortic ArchAbnormalities of

45、 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 anomalies958. Other Anomalies of the Aortic Arch SystemAnomalous origin of the pulmonary artery from the

46、ascending aortaAnomalous origin of the LPA from the RPAInnominate artery compression of the trachea968.1 Anomalous origin of the pulmonary artery from the ascending aorta One branch PA arising from Asc Ao + MPA arising separately from the heart RPA more commonly arise from Ao (82%) 978.1 Anomalous origin of the pulmonary artery from the ascending aorta Investigations CxR differential PBF (esp in TOF with oligemia) Echo diagnostic Carefully search for origins of b