1 / 50

Aortic arch anomalies

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 in a single tube with a series of dilatations(sinus venosus ,atrium ,ventricle & bulbus cordis ) and begins to beat by 22 nd day

aleda
Download Presentation

Aortic arch anomalies

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript


  1. Aortic arch anomalies Dr.DeepakRaju

  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 &bulbuscordis) and begins to beat by 22nd day • Bulbuscordis represents arterial end of the tube-prox part conus,distaltruncusarteriosus • 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)

  3. 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 5thweek,successive arterial arches appear in 2nd to 6th pharyngeal arches • Each connects ventrally to aortic sac&dorsally to dorsal aorta

  4. 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.

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

  6. 3rd-common carotid and prox.int.carotid • 4th – • Lt.-aortic arch b/w LCCA and LSCA. • Rt-prox RSCA • 6TH – • prox part –proxpul 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

  7. 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

  8. Totipotential aortic arch diagram

  9. 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 dysphagialusoria Gross,1945-first division of a vasc ring

  10. 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

  11. 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

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

  13. 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%).

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

  15. 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 ligamentumarteriosum

  16. Normal left arch development

  17. 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

  18. 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

  19. Lt ao.arch and retroesophagealdiverticulum 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

  20. Lt ao.arch,rt.descaorta,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

  21. Lt ao arch &isolated RSCA • Right 6th arch persists • RSCA from rtductus • 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

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

  23. Right aortic arch • A single aortic arch that crosses rtmainstem bronchus • 13-34% in TOF • 30-40% in truncusarteriosus • 20% in pul.atresia with VSD • 7.7% in tricuspid atresia • 8-10% in transposition

  24. Right aortic arch-mirror image type • Sequence of arch vessels-lt.innominate,rtcarotid,RSCA • Ligamentumlt sided • No vascring.can form rarely if Lt. ductus from rtdesc aorta • CCHD in 98%(48% TOF)

  25. Rtao arch with retroesophagealdiverticulum of Kommerell • Sequence –ltcarotid,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

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

  27. Rt arch with Lt desc aorta and Lt ligamentum • Aortic arch itself crosses midline-connects to ltductus to form vasc ring

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

  29. 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

  30. 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

  31. 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 ipsilateralsubclavian • Type C-interruption b/w carotids

  32. Each of the types subcategorised to 3 types • 1.without retroesophageal or isolated subclavian artery • 2.with retroesophagealsubclavian artery • 3. with isolated subclavian artery • Interrupted rt arch seen only in DiGeorge syn. • Type A-aorticopulmonaryseptaldefect,TGA • Type B-m.c,a/w conotruncalanomaly,DiGeorge syn. • Type C-rare

  33. Type A-involution of both dorsal aorta distal to 4tharch,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

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

  35. 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 aorticopulmonaryseptal 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

  36. 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

  37. 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

  38. Thank you

More Related