Aortic arch anomalies

Dr.Deepak Raju

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)

 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

 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.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
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.

 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 aortaductus arteriosus-regresses on right
side

 3rd-common carotid and prox.int.carotid

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

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

Totipotential aortic arch

diagram

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 ringcoined term dysphagia lusoria
Gross,1945-first division of a vasc ring

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

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

Clinical classification

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

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%).

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

 3 ds opposite to side of archdiverticulum,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

Normal left arch

development

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

Lt arch with retroesophageal

RSCA

0.5% incidence
m.c.arch anomaly
38% of downs
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

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

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

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

Lt ao arch with cervical origin of Rt

subclavian
Marker of 22q11 deletion
Innominate trifurcates in the neckRSCA travels back to thorax
Subclavian origin from 3rd arch

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

Right aortic arch-mirror image type

Sequence of arch vesselslt.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)

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

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

Rt arch with Lt desc aorta and Lt

ligamentum
Aortic arch itself crosses midlineconnects to lt ductus to form vasc
ring

Cervical aortic arch

Arch found above level of clavicle
Two categories-normal branching pattern or
anomalous subclavian artery and vascular ring
2nd 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 arch3rd arch becomes definitive aortic arch with separate
origin of ext &int carotid from it

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

Persistent 5 arch
th

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

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

 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

 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

 Present with acute cardiovasc

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

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

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

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

 Thank you