主動脈弓變異學(xué)習(xí)課件

1、1Aortic Arch Anomalies1Aortic Arch Anomalies2Development of Aortic Arch and great vessels2Development of Aortic Arch an33445566778899101011111212131314141515161617171818191920202121222223 23 24Anatomical CategoriesAbnormalities of branchingNormal L Aortic Arch & VariantsAbnormal L Aortic ArchAbnorma

2、lities 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 anomalies24Anatomical CategoriesAbnorma25Clinical ClassificationVascular ringsNon ring vascular

3、compression of trachea, bronchi, oesophagusNon compressive arch malformationDuct dependent arch anomalies25Clinical ClassificationVascu26Clinical features of vascular ringsStridor increase with RTIRecurrent pneumonia/ bronchitisHyperextension of neck (esp. in infants)Reflex apnoea associated with ea

4、tingSwallowing difficultyChocking of food26Clinical features of vascula27Sidedness of Aortic archL & 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 AImporta

5、nce of sidedness of Ao archBT shunt on side of In ARepair of oesophageal atresia side opp arch27Sidedness of Aortic archL & 28Anatomical CategoriesAbnormalities of branchingNormal L Aortic Arch & VariantsAbnormal L Aortic ArchAbnormalities of arch position 3. R Aortic Arch4. Cervical Aortic ArchSupe

6、rpneumarary arches 5. Double Aortic Arch6. Persistent Fifth AA7. Interrupted Aortic Arch 8. Anomalous origin of PA branches and other AA anomalies28Anatomical CategoriesAbnorma291. Normal L Aortic Arch & VariantsVariants1. Common brachiocephalic trunk Present in 10% of L archesNo consequences291. No

7、rmal L Aortic Arch & Va301. 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 RAA433. R Aortic Arch13- 34% of T443.1 RAA with Mirror Image BranchingAlmost always ass. with co

8、ngenital 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 Image Br453.1 RAA with Mirror Image BranchingDiagnosisUsually no retro-oesophageal com

9、pression/ vascular ringEcho/AngioDistinctive branching patternCxR/ Ba oesophagography R indentation of trachea/oesophagusTreatment RAA only - No Rx needed453.1 RAA with Mirror Image Br46Variant L ductus to RE diverticulum from R Desc AoVascular ringNo arch vv from diverticulum(Rarely true mirror ima

10、ge of normal L ductus disappear and R 6th arch continue as ductus)3.1 RAA with Mirror Image Branching46Variant 3.1 RAA with Mirro47 vascular ring+Many asymptomatic, in most no other heart defect3.2 RAA with Retro-oesophageal diverticulum (Of Kommerell)47 vascular ring+3.2 RAA with 48DiagnosisPresent

11、ation vascular ring+CxR R AA ? RE Div of ComBa OesophagogramEchoAngio charact branching pattern, abrupt change in caliber from diverticulum to SCAMRI 3.2 RAA with Retro-oesophageal diverticulum (Of Kommerell)48Diagnosis3.2 RAA with Retro-493.2 RAA with Retro-oesophageal diverticulum (Of Kommerell)Rx

12、Symptomatic Sx division of ligamentum (L thoracotomy/ Median sternotomy)If resp symps/ dysphagia resection of entire diverticulum (R thoracotomy)493.2 RAA with Retro-oesophage50Loss of L 6th ductal arch and persistence of R 6th No vascular ringSmaller posterior indentation of Oesophagus Rx not neede

13、d (no ring) except for ass anomalies3.3 R AA with Retro-oesophageal L SCA50Loss of L 6th ductal arch an51DiagnosisCxR, Ba StudyEcho 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 ductus51Diagn

14、osis3.4 R AA with L Des52Vascular ring+ Very rareSite of arch dissolution L branch of aortic sac(Exception to the general rule 1st arch vessel contain a carotid a. contralateral to Ao A.)3.5 R AA with Retro-oesophageal Innom A.52Vascular ring+ 3.5 R AA with53DiagnosisSingle carotid A. arising from p

15、rox. 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 reimplantation in to AA3.5 R AA with Retro-oesophageal Innom A.53Diagnosis3.5 R AA with Retro543.6 RAA with isolation

16、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 SCA543.6 RAA with isolation of co55 Isolation of L SCADissolution L 4th arch & L distal dorsal

17、 Ao3.6 RAA with isolation of contralateral arch vessels55 Isolation of L SCA3.6 RAA w562. 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 vessels562. Isolation of L CCA3.6 RAA573. Isolation of L Innom. A

18、 Dissolution L horn of aortic sac and distal L dorsalaorta3.6 RAA with isolation of contralateral arch vessels573. Isolation of L Innom. A3.58Clinical F.Low pulse volume/ BP in affected arteryWhen subclavian and vertebral A are involved subclavian steal syndromeCerebral insufficiency, L arm ischaemi

19、a 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 vessels58Clinical F.3.6 RAA with isol59DiagnosisAngio delayed filling of SCABA oesophagography not helpfulDoppler echo reversal of flow in vertebral

20、 arteryRxRepair of CHD + ligation of ductus if patent to prevent stealCNS syms/ claudication of arm surgical reimplantation of SCA to aorta3.6 RAA with isolation of contralateral arch vessels59Diagnosis3.6 RAA with isolat60Anatomical CategoriesAbnormalities of branchingNormal L Aortic Arch & Variant

21、sAbnormal 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 anomalies60Anatomical CategoriesAbnorma614. Cervical Aortic ArchRa

22、re anomalyAA above the level of clavicleTwo main subcategories614. Cervical Aortic ArchRare 624. 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 AA At 3/52

23、 of POA cephalic location at 7/52 POA intrathoracic location 624. Cervical Aortic ArchEmbry634. 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 bra

24、nch patternTypically LAAnon ringAA obstruction due to long, tortuous, hypoplastic, retroesophageal segment634. Cervical Aortic ArchContr644. Cervical Aortic ArchPresentations:Pulsatile masses in supraclavicualar fossa in neckDD aneurysm of carotid/ SCADifferentiation compression of pulsatile mass lo

25、ss of femoral pulseVascular ringSubclavian steal syndrome CxRWide upper mediastinum + absent aortic knobAnterior deviation of trachea644. Cervical Aortic ArchPrese654. Cervical Aortic ArchRx necessaryIf hypoplasia of cervical arch+Symptomatic vascular ringAneurysm of cervical arch itself654. Cervica

26、l Aortic ArchRx ne66Anatomical 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. Anomalous origin

27、 of PA branches and other AA anomalies66Anatomical CategoriesAbnorma675. 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 ArchBoth R 685.

28、 Double Aortic ArchDouble AA with both arches patentSymmetrical origin of 4 brachiocephalic Aa685. Double Aortic ArchDouble 695. 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 w

29、ith L DA 695. Double Aortic Arch2. Dou705. Double Aortic Arch3. Double AA with atretic segment between L CCA and L SCASimilar to RAA with diverticulum of Kommerell705. Double Aortic Arch3. Dou715. Double Aortic ArchAtretic R archRareCan simulate L atresia patterns715. Double Aortic ArchAtretic725. D

30、ouble Aortic ArchDescending aorta could be L or RRarely ass. with CHD -TOF is most commonTGAEmbryological explanationBoth 4th arches and dorsal aortae persistBut usually only one 6th arch (ductus)725. Double Aortic ArchDescend735. Double Aortic ArchClinical featuresvascular ring syms depend on tight

31、ness 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. symsDiagnosisCxR RAA indent trachea superiorly and LAA inferiorlyBa oeso, Echo, Angio, MRI confirm diagnosis 735. Dou

32、ble Aortic ArchClinica745. 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 divided745. Double Aortic ArchMx75Anatomical CategoriesAbnormalities of branchingNormal L Aortic Arch &

33、 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 anomalies75Anatomical CategoriesAbnorma766. Persistent Fif

34、th 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 Rare776. Persistent Fifth AA Double lumen AA with both lumina patentFrequently ass with major cardiac anomaly776. Persistent

35、 Fifth AA Doubl786. 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 COA786. Persistent Fifth AA Atres796. Persistent Fifth AA Systemic to pulmonary artery connecti

36、on 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)796. Persistent Fifth AA Syste806. Persistent Fifth AA Diagnosis“Subway” vess

37、el beneath the 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 Ao806. Persistent Fifth AA Diagn81Anatomical CategoriesAbnormal

38、ities 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 anomalies81Anatomi

39、cal CategoriesAbnorma827. Interrupted Aortic ArchComplete 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 arc

40、hImportance of sidednessInterrupted R AA only seen in ass with Digeorge syndrome827. Interrupted Aortic ArchCo837. 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

41、SCAInterruption between carotid arteriesSubcategoriesWithout retro-esophageal or isolated SCAWith retro-esophageal SCAWith isolated SCA837. Interrupted Aortic Arch3 847. Interrupted Aortic ArchInterruption distal to SCA that is ipsilateral to 2nd Carotid Aassociations Aortico-pulmonary septal defect

42、s + Intact IVSTGA + Interrupted AA847. Interrupted Aortic ArchIn857. Interrupted Aortic ArchInterruption between 2nd carotid and ipsilateral SCAWithout retro-esophageal or isolated SCAMore common than type A 857. Interrupted Aortic ArchIn867. Interrupted Aortic ArchInterruption between 2nd carotid a

43、nd ipsilateral SCAWith retro-esophageal SCADigeorge syndrome + interruption have type B867. Interrupted Aortic ArchIn877. Interrupted Aortic ArchInterruption between carotid arteriesRare877. Interrupted Aortic ArchIn887. Interrupted Aortic ArchAssociationsDigeorge syndrome Vs IAA / Truncus 43% of Di

44、georges had type B interruption68% of IAA had Digeorge34% of Digeorges had TA33% of TA had Digeorge887. Interrupted Aortic ArchAs897. Interrupted Aortic ArchPresentationDuct dependant L heart obstructive lesionsAcute cardiovascular collapse / heat failure after spont closure of PDA after 1st few day

45、s of lifeInitial MxFluid resuscitationInduction and maintenance 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. Interrupt

46、ed Aortic ArchPr907. Interrupted Aortic ArchDifferential cyanosispink upper body + blue lower bodyUncommonly seen bse pulm blood is also highly saturated due to large LR shunt through VSD907. Interrupted Aortic ArchDi917. Interrupted Aortic ArchDiagnosisEchocardiogram Most important tool for diagnos

47、is 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 both carotids prox and both SCA distal to interruptionWide separation of

48、 carotids from Desc Ao IAA917. Interrupted Aortic ArchDi927. 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 3 mm inadequate to support normal COP927. Interrupt

49、ed Aortic ArchMa937. 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 avoid artificial tube grafts R

50、apidly overgrownFibrous encasement complicate later repair937. Interrupted Aortic ArchPA94Anatomical CategoriesAbnormalities of branchingNormal L Aortic Arch & VariantsAbnormal L Aortic ArchAbnormalities of arch position 3. R Aortic Arch4. Cervical Aortic ArchSuperpneumarary arches 5. Double Aortic

51、Arch6. Persistent Fifth AA7. Interrupted Aortic Arch 8. Anomalous origin of PA branches and other AA anomalies94Anatomical CategoriesAbnorma958. Other Anomalies of the Aortic Arch SystemAnomalous origin of the pulmonary artery from the ascending aortaAnomalous origin of the LPA from the RPAInnominat

52、e artery compression of the trachea958. Other Anomalies of the Ao968.1 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 978.1 Anomalous origin of the pulmonary artery from the ascending aorta InvestigationsCxR differential PBF (esp in TOF with oligemia)Echo diagnosticCarefully sea