主动脉弓变异

1、主动脉弓变异第一页，共102页。Development of Aortic Arch and great vessels第二页，共102页。第三页，共102页。第四页，共102页。第五页，共102页。第六页，共102页。第七页，共102页。第八页，共102页。第九页，共102页。第十页，共102页。第十一页，共102页。第十二页，共102页。第十三页，共102页。第十四页，共102页。第十五页，共102页。第十六页，共102页。第十七页，共102页。第十八页，共102页。第十九页，共102页。第二十页，共102页。第二十一页，共102页。第二十二页，共102页。 第二十三页，共102页。Ana

2、tomical 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

3、nd other AA anomalies第二十四页，共102页。Clinical Classification Vascular rings Non ring vascular compression of trachea, bronchi, oesophagus Non compressive arch malformation Duct dependent arch anomalies第二十五页，共102页。Clinical features of vascular rings Stridor increase with RTI Recurrent pneumonia/ bronchit

4、is Hyperextension of neck (esp. in infants) Reflex apnoea associated with eating Swallowing difficulty Chocking of food第二十六页，共102页。Sidedness of Aortic arch L & R aortic arch definitions Refers to which bronchus is crossed by the arch Normal Cross the L main bronchus at T5 Branching. general rule

5、 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 arch第二十七页，共102页。Anatomical CategoriesAbnormalities of branching Normal L Aortic Arch & VariantsAbnormal L Aortic ArchAbnormalities of arc

6、h 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 anomalies第二十八页，共102页。1. Normal L Aortic Arch & VariantsVariants1. Common brachiocephalic trunk Present in

7、 10% of L archesNo consequences第二十九页，共102页。1. 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 RAA第四十三页，共102页。3.1 RAA with Mirror Image Branching Almost always ass. with

8、 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 ring第四十四页，共102页。3.1 RAA with Mirror Image Branching Diagnosis Usually no retro-oesophageal compression/ va

9、scular ring Echo/Angio Distinctive branching pattern CxR/ Ba oesophagography R indentation of trachea/oesophagus Treatment RAA only - No Rx needed第四十五页，共102页。 Variant L ductus to RE diverticulum from R Desc AoVascular ringNo arch vv from diverticulum(Rarely true mirror image of normal L ductus disap

10、pear and R 6th arch continue as ductus)3.1 RAA with Mirror Image Branching第四十六页，共102页。 vascular ring+ Many asymptomatic, in most no other heart defect3.2 RAA with Retro-oesophageal diverticulum (Of Kommerell)第四十七页，共102页。 Diagnosis Presentation vascular ring +CxR R AA ? RE Div of Com Ba Oesophagogram

11、 Echo Angio charact branching pattern, abrupt change in caliber from diverticulum to SCA MRI 3.2 RAA with Retro-oesophageal diverticulum (Of Kommerell)第四十八页，共102页。3.2 RAA with Retro-oesophageal diverticulum (Of Kommerell) Rx Symptomatic Sx division of ligamentum (L thoracotomy/ Median sternotomy) If

12、 resp symps/ dysphagia resection of entire diverticulum (R thoracotomy)第四十九页，共102页。Loss 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 SCA第五十页，共102页。Diagnosis

13、CxR, Ba Study Echo branching pattern + L desc Ao Angio difficult to DD from Normal L AA go by branching pattern MRIRx when symptomatic need division3.4 R AA with L Desc Ao & L ductus第五十一页，共102页。 Vascular ring+ Very rare Site of arch dissolution L branch of aortic sac(Exception to the general rul

14、e 1st arch vessel contain a carotid a. contralateral to Ao A.)3.5 R AA with Retro-oesophageal Innom A.第五十二页，共102页。 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

15、 still symptomatic detachment of Inn a and reimplantation in to AA3.5 R AA with Retro-oesophageal Innom A.第五十三页，共102页。3.6 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 ca

16、ses 2/3 have TOF Most common isolation isolated SCA第五十四页，共102页。 Isolation of L SCA Dissolution L 4th arch & L distal dorsal Ao3.6 RAA with isolation of contralateral arch vessels第五十五页，共102页。2. Isolation of L CCADissolution L 4th arch & L horn of aortic sac with 6th arch connecting to 3rd arc

17、h3.6 RAA with isolation of contralateral arch vessels第五十六页，共102页。3. Isolation of L Innom. A Dissolution L horn of aortic sac and distal L dorsalaorta3.6 RAA with isolation of contralateral arch vessels第五十七页，共102页。 Clinical F. Low pulse volume/ BP in affected artery When subclavian and vertebral A ar

18、e involved subclavian steal syndrome Cerebral insufficiency, L arm ischaemia If ductus remain patent PA steal (flow down vertebral a. in to low res. PA) Suspect RAA+ low pulse in L UL3.6 RAA with isolation of contralateral arch vessels第五十八页，共102页。 Diagnosis Angio delayed filling of SCA BA oesophagog

19、raphy not helpful Doppler echo reversal of flow in vertebral artery Rx Repair of CHD + ligation of ductus if patent to prevent steal CNS syms/ claudication of arm surgical reimplantation of SCA to aorta3.6 RAA with isolation of contralateral arch vessels第五十九页，共102页。Anatomical CategoriesAbnormalities

20、 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 anomalies第六十页，共102页

21、。4. Cervical Aortic ArchRare anomalyAA above the level of clavicleTwo main subcategories第六十一页，共102页。4. Cervical Aortic ArchEmbryological explanationPersistence of ductus caroticus + involution of 4th arch 3rd arch becomes AA (int & ext carotid arising separately)Failure of the normal descent of

22、AA At 3/52 of POA cephalic location at 7/52 POA intrathoracic location 第六十二页，共102页。4. Cervical Aortic ArchContralateral descending Ao. and Anomalous SCA Usually RAADescend to T4 level cross behind Oeso. to L gives off L SCA & Ductus vascular ring Ipsilateral descending aorta and normal branch pa

23、ttern Typically LAAnon ringAA obstruction due to long, tortuous, hypoplastic, retroesophageal segment第六十三页，共102页。4. Cervical Aortic ArchPresentations:Pulsatile masses in supraclavicualar fossa in neckDD aneurysm of carotid/ SCADifferentiation compression of pulsatile mass loss of femoral pulseVascul

24、ar ringSubclavian steal syndrome CxRWide upper mediastinum + absent aortic knobAnterior deviation of trachea第六十四页，共102页。4. Cervical Aortic ArchRx necessaryIf hypoplasia of cervical arch+Symptomatic vascular ringAneurysm of cervical arch itself第六十五页，共102页。Anatomical CategoriesAbnormalities of branchi

25、ngNormal 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. Anomalous origin of PA branches and other AA anomalies第六十六页，共102页。5. Double A

26、ortic 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 located第六十七页，共102页。5. Double Aortic ArchDouble AA with both arches patentSymmetrical origin of 4 brachiocephali

27、c Aa第六十八页，共102页。5. 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 第六十九页，共102页。5. Double Aortic Arch3. Double AA with atretic segment between L CCA and L SCASimilar to

28、RAA with diverticulum of Kommerell第七十页，共102页。5. Double Aortic ArchAtretic R archRareCan simulate L atresia patterns第七十一页，共102页。5. Double Aortic ArchDescending aorta could be L or RRarely ass. with CHD -TOF is most commonTGAEmbryological explanationBoth 4th arches and dorsal aortae persistBut usually

29、 only one 6th arch (ductus)第七十二页，共102页。5. 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. symsDi

30、agnosisCxR RAA indent trachea superiorly and LAA inferiorlyBa oeso, Echo, Angio, MRI confirm diagnosis 第七十三页，共102页。5. 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 divided第七十四页

31、，共102页。Anatomical 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

32、branches and other AA anomalies第七十五页，共102页。6. 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 第七十六页，共102页。6. Persistent Fifth AA Double lumen AA with both lumina patentFrequentl

33、y ass with major cardiac anomaly第七十七页，共102页。6. Persistent 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 COA第七十八页，共102页。6. Persistent Fifth AA Systemic to pulmonary artery c

34、onnection arising proximal to 1st brachiocephalic VvOnly in pulmonary 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)第七十九页，共102页。6. Persistent Fifth AA Diagnosis“Subway” vessel beneath th

35、e normal archIn atresia of superior arch common brachiocephalic trunk with 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 Ao第八十页，共102页。Anatomical CategoriesAbnormalities of branchingNormal L Aortic

36、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 anomalies第八十一页，共102页。7. Interrupted Aortic ArchC

37、omplete separation of ascending and descending aortaDetermination of sidedness of AABranching pattern- 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

38、 in ass with Digeorge syndrome第八十二页，共102页。7. Interrupted Aortic Arch3 main categories 9 sub categories Main categoriesInterruption distal to SCA that is ipsilateral to 2nd Carotid AInterruption between 2nd carotid and ipsilateral SCAInterruption between carotid arteriesSubcategoriesWithout retro-eso

39、phageal or isolated SCAWith retro-esophageal SCA1.With isolated SCA第八十三页，共102页。7. Interrupted Aortic ArchInterruption distal to SCA that is ipsilateral to 2nd Carotid Aassociations Aortico-pulmonary septal defects + Intact IVSTGA + Interrupted AA第八十四页，共102页。7. Interrupted Aortic ArchInterruption bet

40、ween 2nd carotid and ipsilateral SCAWithout retro-esophageal or isolated SCAMore common than type A 第八十五页，共102页。7. Interrupted Aortic ArchInterruption between 2nd carotid and ipsilateral SCAWith retro-esophageal SCADigeorge syndrome + interruption have type B第八十六页，共102页。7. Interrupted Aortic ArchInt

41、erruption between carotid arteriesRare第八十七页，共102页。7. Interrupted Aortic ArchAssociationsDigeorge syndrome Vs IAA / Truncus 43% of Digeorges had type B interruption68% of IAA had Digeorge34% of Digeorges had TA33% of TA had Digeorge第八十八页，共102页。7. Interrupted Aortic ArchPresentationDuct dependant L he

42、art obstructive lesionsAcute cardiovascular collapse / heat failure after spont closure of PDA after 1st few days of lifeInitial MxFluid resuscitationInduction and maintenance of ductal patency with PGE1Inotropic support SOSClinical featurespulse discrepancy depends on branching patternAbsence of al

43、l limb pulses type B interruption with anomalous SCA DD - critical AS (carotid pulse is also week)第八十九页，共102页。7. Interrupted Aortic ArchDifferential cyanosispink upper body + blue lower bodyUncommonly seen bse pulm blood is also highly saturated due to large LR shunt through VSD第九十页，共102页。7. Interru

44、pted Aortic ArchDiagnosisEchocardiogram Most important tool for diagnosis of IAASuspect whenMarked discrepancy between Asc Ao and MPA + malalignment VSD + posterior deviation of infundibular septum (PS LAX)AngiographyDifficult bse high flow through VSD poor image quality of Asc AoCan diagnose when b

45、oth carotids prox and both SCA distal to interruptionWide separation of carotids from Desc Ao IAA第九十一页，共102页。7. Interrupted Aortic ArchManagementSx approach depend on degree of subaortic obstructionSubaortic diameter 5-6 mm 1ry repair(patch closure of VSD + Ao Arch reconstruction)Subaortic diameter

46、3 mm inadequate to support normal COP第九十二页，共102页。7. Interrupted Aortic ArchPA banding is not a satisfactory palliation for VSD with interrupted Ao AWill lead to BVH with progressive subaortic stenosis complicate definitive repairRepair of Ao Archdirect anastomosis + homograft augmentation In infancy

47、 avoid artificial tube grafts Rapidly overgrownFibrous encasement complicate later repair第九十三页，共102页。Anatomical CategoriesAbnormalities of branchingNormal L Aortic Arch & VariantsAbnormal L Aortic ArchAbnormalities of arch position 3. R Aortic Arch4. Cervical Aortic ArchSuperpneumarary arches 5.

48、 Double Aortic Arch6. Persistent Fifth AA7. Interrupted Aortic Arch 8. Anomalous origin of PA branches and other AA anomalies第九十四页，共102页。8. Other Anomalies of the Aortic Arch SystemAnomalous origin of the pulmonary artery from the ascending aortaAnomalous origin of the LPA from the RPAInnominate art

49、ery compression of the trachea第九十五页，共102页。8.1 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%) 第九十六页，共102页。8.1 Anomalous origin of the pulmonary artery from the ascending aorta Investigations CxR differential PBF (esp in TOF with oligemia) Echo di