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Aortic arch anomalies Dr.Deepak Raju.

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Presentation on theme: "Aortic arch anomalies Dr.Deepak Raju."— Presentation transcript:

1 Aortic arch anomalies Dr.Deepak Raju

2 Embryology Heart is first seen in the form of two endothelial heart tubes-18th day of foetal life Fusion results in a single tube with a series of dilatations(sinus venosus ,atrium ,ventricle &bulbus cordis) and begins to beat by 22nd day Bulbus cordis represents arterial end of the tube-prox part conus,distal truncus arteriosus First arteries to appear are right and left primitive aorta connected to the endothelial heart tubes Portion lying ventral to foregut(ventral aorta)-connected to first pharyngeal arch-to the portion dorsal to foregut(dorsal aorta)



5 After the fusion of endocardial heart tubes,ventral aorta fuse to form aortic sac
Truncus continues with the aortic sac from which right and left pharyngeal arch arteries arises They arch backward on lateral side of foregut –continues as right and left dorsal aorta-fuse to form descending aorta During 4th and 5th week,successive arterial arches appear in 2nd to 6th pharyngeal arches Each connects ventrally to aortic sac&dorsally to dorsal aorta


7 Greater part of 1st &2nd arch arteries disappear
Greater part of 1st &2nd arch arteries disappear.1st arch remnant-maxillary artery,2nd arch remnant –hyoid and stapedial artery 5th arch artery regress completely 3rd and 4th open to ventral part of aortic sac.6th to dorsal part. Spiral septum formed in truncus in the 5th week extends to aortic sac.blood from pul.artery goes to 6th arch artery,from aorta to 3rd &4th arch arteries. Dorsal aorta gives lateral intersegmental branches to body wall.7th cervical intersegmental supplies upper limb bud.


9 Portion of dorsal aorta b/w 3rd and 4th (ductus caroticus)disappear
Each 6th arch artery connects to the pulmonary vascular tree.portion b/w this connection and dorsal aorta-ductus arteriosus-regresses on right side


11 3rd-common carotid and 4th –
Lt.-aortic arch b/w LCCA and LSCA. Rt-prox RSCA 6TH – prox part –prox pul art distal part-ductus on left and right side involutes Lt dorsal aorta-aortic arch distal to LSCA Rt dorsal aorta- cranial portion-RSCA distal to 4th arch. distal portion-involutes


13 Edward s double aortic arch model
Anomalies of aortic arch to be conceptualised as variations in regression of different segments from the hypothetical double arch


15 Totipotential aortic arch diagram

16 History Anomalous RSCA-Hunauld,1735 Double aortic arch-Hommel 1737 Right aortic arch –Fioratti,Aglieti-1763 Interrupted aortic arch-Steidele-1788 Bayford,1787-dysphagia by vasc ring-coined term dysphagia lusoria Gross,1945-first division of a vasc ring

17 Sidedness of the arch Left and right arch refers to which bronchus is crossed by the arch Echo or angio-branching pattern of brachiocephalic vessels First arch vessel that contains carotid artery opposite side of arch Retroesophageal or isolated vessels-opp to side of arch MRI and CT-conclusive

18 Anatomical classification
Abnormalities of branching Abnormalites of arch position-cervical arch,right arch Supernumary arches-double aortic arch and persistent 5th arch IAA Anomalous origin of pulmonary artery branch

19 Clinical classification
Vascular rings Non-ring vasc.compression Non-compressive arch malformations Ductal dependent arch anomalies

20 vascular ring-aortic arch anomaly in which trachea and esophagus surrounded by vasc. structures Double aortic arch most common(40%),rt.aortic arch with lt.ligamentum(30%),aberrant RSCA(20%),anomalous innominate(10%).

21 Symptoms- Stridor,Pneumonia,bronchitis
Reflex apnoea or choking on eating Hyperextension of neck Increased resp distress a/w intercurrent resp.infections swallowing difficulty

22 3 d΄s opposite to side of arch-diverticulum,dimple,descending aorta
Diverticulum –large vessel from desc.aorta giving rise to a smaller calibre vessel with a sudden taper Dimple –tapered blindly ending outpouching Descending aorta in upper thorax side of arch-connected by ligamentum arteriosum

23 Normal left arch development

24 Variants of left aortic arch
Common brachiocephalic trunk Right innominate and left carotid from single origin 10% of normal Compression of trachea possible Separate origin of left vertebral artery 10% Prox to LSCA 3rd arch vessel smaller than 4th

25 Lt arch with retroesophageal RSCA
0.5% incidence m.c.arch anomaly 38% of down′s Disappearance of Rt 4th arch-distal Rt dorsal aorta becomes prox RSCA Rt 6th arch disappear Usually asymtomatic Barium –smaller filling defect on postr aspect of esophagus slanting upward Angio-earlier filling of Rt carotid on aortic root injection


27 Lt ao.arch and retroesophageal diverticulum of Kommerell
First vasc ring to be diagnosed during life Similar to previous except for persistent 6th arch-ligamentum which completes a vasc . Ring Prox.RSCA dilated to form diverticulum

28 Lt ao.arch,rt.desc aorta,rt.ductus(circumflex aortic arch)
Branching pattern similar to earlier-arch retroesophageal,RSCA the last arch vessel is not retroesophageal Desc.aorta connected to RPA by ligamentum-forms vasc.ring

29 Lt ao arch &isolated RSCA
Right 6th arch persists RSCA from rt ductus RSCA and vertebral fills from PA in foetal life When ductus closes-retrogradely from circle of willis Vertebrobasilar insufficiency Congenital subclavian steal Absent rt arm pulse

30 Lt ao arch with cervical origin of Rt subclavian
Marker of 22q11 deletion Innominate trifurcates in the neck-RSCA travels back to thorax Subclavian origin from 3rd arch

31 Right aortic arch A single aortic arch that crosses rt mainstem bronchus 13-34% in TOF 30-40% in truncus arteriosus 20% in pul.atresia with VSD 7.7% in tricuspid atresia 8-10% in transposition

32 Right aortic arch-mirror image type
Sequence of arch vessels-lt.innominate,rt carotid,RSCA Ligamentum lt sided No vasc ring.can form rarely if Lt. ductus from rt desc aorta CCHD in 98%(48% TOF)


34 Rt ao arch with retroesophageal diverticulum of Kommerell
Sequence –lt carotid,rt carotid ,RSCA,a large retroesophageal vessel( diverticulum) from which LSCA arises Lt ligamentum completes the ring Disappearance of Lt 4th arch and persistence of 6th arch


36 Rt arch with retroesophageal LSCA
Similar to previous one except for the absence of retroesophageal diverticulum Ductus is rt sided No vasc ring Involution of lt 4th and 6th

37 Rt arch with Lt desc aorta and Lt ligamentum
Aortic arch itself crosses midline-connects to lt ductus to form vasc ring

38 Cervical aortic arch Arch found above level of clavicle
Two categories-normal branching pattern or anomalous subclavian artery and vascular ring 2nd group-devided carotid origin(bicarotid trunk or separate origin of ext.&int carotid) Mechanism- Failure of normal descent of aortic arch system Persistence of ductus caroticus&involution of 4th arch-3rd arch becomes definitive aortic arch with separate origin of ext &int carotid from it

39 Double aortic arch Both rt and lt arches present
Persistence of both rt and lt 4th arch which join TA sac to dorsal aorta both of which persist Only one 6th remain Rarely a/w other CHD, when present-TOF most common Both arches can be patent or one hypoplastic or atretic(usu.left) Form complete vasc.rings Symmetric origin of 4 arch vessels from respective arches when both patent


41 Persistent 5th arch First reported by Van praagh in 1969
Double lumen aortic arch in which both arches appear on same side of trachea 2 common sub categories- Subway vessel beneath normal arch(4th arch)that extend from innominate to take off of LSCA Double lumen aortic arch with atresia of superior arch with patent inferior arch-common origin of all brachiocephalic vessels from asc.aorta


43 Interrupted aortic arch
Defined as complete separation of ascending and descending aorta Celoria and Patton classification(1959) Type A-interruption distal to SCA that is ipsilateral to 2nd carotid artery Type B-interruption b/w 2nd carotid and ipsilateral subclavian Type C-interruption b/w carotids

44 Each of the types subcategorised to 3 types
1.without retroesophageal or isolated subclavian artery 2.with retroesophageal subclavian artery 3. with isolated subclavian artery Interrupted rt arch seen only in DiGeorge syn. Type A-aorticopulmonary septal defect,TGA Type B-m.c,a/w conotruncal anomaly,DiGeorge syn. Type C-rare

45 Type A-involution of both dorsal aorta distal to 4th arch,prox to persistent 6th arch
Type b-involution of one 4th arch and one dorsal aorta b/w 4th and 6th Type C-involution of one limb of truncoaortic sac

46 Present with acute cardiovasc collapse after closure of ductus
Absence of all limb pulse with strong carotid pulse suggest type B with anomalous subclavian

47 Anomalous origin of pulmonary artery from ascending aorta
Anomalous pulmonary artery branch arising from ascending aorta in presence of a MPA arising separately Anomalous RPA- More common Embryonic branch pul.artery joins rt side of TA sac,but fails to join MPA before septation High incidence of aorticopulmonary septal defect Anomalous LPA a/w TOF in 74% Embryonic branch pul.artery fails to join TA sac CCF in infancy f/b early devt of pulmonary vascular disease

48 Anomalous origin of LPA from RPA
LPA arises from RPA and passes b/w trachea and esophagus-pulmonary artery sling Tracheal compression-severe resp distress and stridor Isolated anomaly,rarely a/w TOF LPA passes beyond trachea before joining TA sac Anterior indentation on barium swallow

49 Summary Aortic arch anomalies and vascular rings can be interpreted on the basis of embryology With the devt. Of MRI and CT 3-D reconstruction is possible Intervention required only when symptomatic or when a/w other cardiac anomalies

50 Thank you

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