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Aortic arch anomalies Dr.Deepak Raju. Embryology Heart is first seen in the form of two endothelial heart tubes-18 th day of foetal life Fusion results.

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Presentation on theme: "Aortic arch anomalies Dr.Deepak Raju. Embryology Heart is first seen in the form of two endothelial heart tubes-18 th day of foetal life Fusion results."— Presentation transcript:

1 Aortic arch anomalies Dr.Deepak Raju

2 Embryology Heart is first seen in the form of two endothelial heart tubes-18 th 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 22 nd 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)

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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 4 th and 5 th week,successive arterial arches appear in 2 nd to 6 th pharyngeal arches Each connects ventrally to aortic sac&dorsally to dorsal aorta

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

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

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11 3 rd -common carotid and prox.int.carotid 4 th – – Lt.-aortic arch b/w LCCA and LSCA. – Rt-prox RSCA 6 TH – – 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 4 th arch. – distal portion-involutes

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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

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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 5 th 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 opp.to 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 – 3 rd arch vessel smaller than 4 th

25 Lt arch with retroesophageal RSCA 0.5% incidence m.c.arch anomaly 38% of down′s Disappearance of Rt 4 th arch-distal Rt dorsal aorta becomes prox RSCA Rt 6 th 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

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27 Lt ao.arch and retroesophageal diverticulum of Kommerell First vasc ring to be diagnosed during life Similar to previous except for persistent 6 th 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 6 th 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 3 rd 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)

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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 4 th arch and persistence of 6 th arch

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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 4 th and 6 th

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 2 nd group-devided acc.to 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 4 th arch- 3 rd 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 4 th arch which join TA sac to dorsal aorta both of which persist Only one 6 th 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

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41 Persistent 5 th 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(4 th 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

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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 2 nd carotid artery – Type B-interruption b/w 2 nd 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 4 th arch,prox to persistent 6 th arch Type b-involution of one 4 th arch and one dorsal aorta b/w 4 th and 6 th 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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