Aortic Arch Anomalies

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Aortic Arch Anomalies
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Development of Aortic Arch and great vessels
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Anatomical Categories

• Abnormalities of branching
• Normal L Aortic Arch Variants
• Abnormal L Aortic Arch
• Abnormalities of arch position
• 3. R Aortic Arch
• 4. Cervical Aortic Arch
• Superpneumarary arches
• 5. Double Aortic Arch
• 6. Persistent Fifth AA
• 7. Interrupted Aortic Arch
• 8. Anomalous origin of PA branches and other AA
  anomalies

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Clinical Classification

• Vascular rings
• Non ring vascular compression of trachea,
  bronchi, oesophagus
• Non compressive arch malformation
• Duct dependent arch anomalies

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Clinical features of vascular rings

• Stridor increase with RTI
• Recurrent pneumonia/ bronchitis
• Hyperextension of neck (esp. in infants)
• Reflex apnoea associated with eating
• Swallowing difficulty
• Chocking of food

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Sidedness of Aortic arch

• L R aortic arch definitions
• Refers to which bronchus is crossed by the arch
• Normal
• Cross the L main bronchus at T5
• Branching. general rule 1st arch vessel
  contain a carotid a. contralateral to Ao A
• Importance of sidedness of Ao arch
• BT shunt on side of In A
• Repair of oesophageal atresia side opp arch

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Anatomical Categories

• Abnormalities of branching
• Normal L Aortic Arch Variants
• Abnormal L Aortic Arch
• Abnormalities of arch position
• 3. R Aortic Arch
• 4. Cervical Aortic Arch
• Superpneumarary arches
• 5. Double Aortic Arch
• 6. Persistent Fifth AA
• 7. Interrupted Aortic Arch
• 8. Anomalous origin of PA branches and other AA
  anomalies

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1. Normal L Aortic Arch Variants

• Variants
• 1. Common brachiocephalic trunk
• Present in 10 of L arches
• No consequences

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1. Normal L Aortic Arch Variants

• Variants
• 2. Separate origin of L vertebral a. from aortic
  arch (normal from L subclavian)
• Size 1gt2, 3lt4
• DD anomalous R SCA

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Anatomical Categories

• Abnormalities of branching
• Normal L Aortic Arch Variants
• Abnormal L Aortic Arch
• Abnormalities of arch position
• 3. R Aortic Arch
• 4. Cervical Aortic Arch
• Superpneumarary arches
• 5. Double Aortic Arch
• 6. Persistent Fifth AA
• 7. Interrupted Aortic Arch
• 8. Anomalous origin of PA branches and other AA
  anomalies

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2. Abnormal L Aortic Arch

• L AA with retroesophageal R SCA
• L AA with R Desc Ao R ductus

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2.1 L AA with retroesophageal R SCA

• Most common arch anomaly 0.5 of general
  population
• Higher incidence in Downs with CHD 38
• Mostly asymptomatic

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2.1 L AA with retroesophageal R SCA

• Diagnosis
• Echo/angio
• Branch sizes of 12, 34
• 1st no bifurcation, goes to R
• 2nd,3rd to L, non bifurcating
• 4th towards R, disappear behind trachea

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2.1 L AA with retroesophageal R SCA

• Ba oesophagography
• Small filling defect slanting up and R
• MRI

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2.2 L AA with R Desc Ao R ductus

• Branching pattern similar to previous
• Rare
• Arch retro-oesophageal
• Desc Ao connected to PA by R ductus ? ring

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2.2 L AA with R Desc Ao R ductus

• Diagnosis
• Suspect when symps of vascular ring L aortic
  arch present
• CxR
• L Ao A R upper desc. Ao (adults)

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2.2 L AA with R Desc Ao R ductus

• Diagnosis
• Ba oesophagography
• Large indentation directed up and L
• DD R Ao A with retro-oesophageal diverticulum
• Angiography, MRI

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2.2 L AA with R Desc Ao R ductus

• Rx R thoracotomy division of ring

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Anatomical Categories

• Abnormalities of branching
• Normal L Aortic Arch Variants
• Abnormal L Aortic Arch
• Abnormalities of arch position
• 3. R Aortic Arch
• 4. Cervical Aortic Arch
• Superpneumarary arches
• 5. Double Aortic Arch
• 6. Persistent Fifth AA
• 7. Interrupted Aortic Arch
• 8. Anomalous origin of PA branches and other AA
  anomalies

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3. R Aortic Arch

• Definition
• Single aortic arch that crosses over the R main
  bronchus passing to the R of the trachea

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3. R Aortic Arch

• Major types
• R AA with mirror image branching
• R AA with retro-oesophageal L SCA
• R AA with retro-oesophageal diverticulum
• R AA with L descending aorta

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3. R Aortic Arch

• 13- 34 of TOF have RAA
• Incidence in Truncus Arteriosus gt that of TOF
• 8 of DTGA, 16 of TGAVSDPS have RAA

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3.1 RAA with Mirror Image Branching

• Almost always ass. with congenital intracardiac
  disease
• Conotruncal anomalies TOF, TA, TGA, DORV, LTGA,
  PA with RV aorta
• Other lesions VSD, PA with IVS
• Ductus is commonly L sided - attached to L
  innom. A. no vascular ring

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3.1 RAA with Mirror Image Branching

• Diagnosis
• Usually no retro-oesophageal compression/
  vascular ring
• Echo/Angio
• Distinctive branching pattern
• CxR/ Ba oesophagography
• R indentation of trachea/oesophagus
• Treatment
• RAA only - No Rx needed

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3.1 RAA with Mirror Image Branching

• Variant
• L ductus to RE diverticulum from R Desc Ao
• Vascular ring
• No arch vv from diverticulum
• (Rarely true mirror image of normal L ductus
  disappear and R 6th arch continue as ductus)

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3.2 RAA with Retro-oesophageal diverticulum (Of
Kommerell)

• vascular ring
• Many asymptomatic, in most no other heart defect

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3.2 RAA with Retro-oesophageal diverticulum (Of
Kommerell)

• Diagnosis
• Presentation vascular ring
• CxR R AA ? ? RE Div of Com
• Ba Oesophagogram
• Echo
• Angio charact branching pattern, abrupt change
  in caliber from diverticulum to SCA
• MRI

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3.2 RAA with Retro-oesophageal diverticulum (Of
Kommerell)

• Rx
• Symptomatic Sx division of ligamentum (L
  thoracotomy/ Median sternotomy)
• If resp symps/ dysphagia resection of entire
  diverticulum (R thoracotomy)

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3.3 R AA with Retro-oesophageal L SCA

• Loss of L 6th ductal arch and persistence of R
  6th
• No vascular ring
• Smaller posterior indentation of Oesophagus
• Rx not needed (no ring) except for ass anomalies

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3.4 R AA with L Desc Ao L ductus

• Diagnosis
• CxR, Ba Study
• Echo branching pattern L desc Ao
• Angio difficult to DD from Normal L AA go by
  branching pattern
• MRI
• Rx when symptomatic need division

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3.5 R AA with Retro-oesophageal Innom A.

• Vascular ring
• Very rare
• Site of arch dissolution L branch of aortic sac
• (Exception to the general rule 1st arch vessel
  contain a carotid a. contralateral to Ao A.)

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3.5 R AA with Retro-oesophageal Innom A.

• Diagnosis
• Single carotid A. arising from prox. Aorta
• DD interrupted AA, isolated L
  carotid/Innominate A.
• Differentiating feature normal size AA
• Rx
• Division of the ring if symptomatic ? if still
  symptomatic detachment of Inn a and
  reimplantation in to AA

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3.6 RAA with isolation of contralateral arch
vessels

• Uncommon
• Vessel arises exclusively from PA via ductus
  arteriosus without connection to aorta
• 3 different forms
• CHD in gt 50 of cases
• gt 2/3 have TOF
• Most common isolation isolated SCA

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3.6 RAA with isolation of contralateral arch
vessels

• Isolation of L SCA
• Dissolution L 4th arch L distal dorsal Ao

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3.6 RAA with isolation of contralateral arch
vessels

• 2. Isolation of L CCA
• Dissolution L 4th arch L horn of aortic sac
  with
• 6th arch connecting to 3rd arch

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3.6 RAA with isolation of contralateral arch
vessels

• 3. Isolation of L Innom. A
• Dissolution L horn of aortic sac and distal L
  dorsal
• aorta

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3.6 RAA with isolation of contralateral arch
vessels

• Clinical F.
• Low pulse volume/ BP in affected artery
• When subclavian and vertebral A are involved
  subclavian steal syndrome
• Cerebral insufficiency, L arm ischaemia
• If ductus remain patent PA steal (flow down
  vertebral a. in to low res. PA)
• Suspect RAA low pulse in L UL

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3.6 RAA with isolation of contralateral arch
vessels

• Diagnosis
• Angio delayed filling of SCA
• BA oesophagography not helpful
• Doppler echo reversal of flow in vertebral
  artery
• Rx
• Repair of CHD ligation of ductus if patent to
  prevent steal
• CNS syms/ claudication of arm surgical
  reimplantation of SCA to aorta

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Anatomical Categories

• Abnormalities of branching
• Normal L Aortic Arch Variants
• Abnormal L Aortic Arch
• Abnormalities of arch position
• 3. R Aortic Arch
• 4. Cervical Aortic Arch
• Superpneumarary arches
• 5. Double Aortic Arch
• 6. Persistent Fifth AA
• 7. Interrupted Aortic Arch
• 8. Anomalous origin of PA branches and other AA
  anomalies

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4. Cervical Aortic Arch

• Rare anomaly
• AA above the level of clavicle
• Two main subcategories

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4. Cervical Aortic Arch

• Embryological explanation
• Persistence 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 of POA cephalic location ? at 7/52 POA
  intrathoracic location

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4. Cervical Aortic Arch

• Contralateral descending Ao. and Anomalous SCA
• Usually RAA
• Descend to T4 level cross behind Oeso. to L
  gives off L SCA Ductus
• ? vascular ring
• Ipsilateral descending aorta and normal branch
  pattern
• Typically LAA
• non ring
• AA obstruction due to long, tortuous,
  hypoplastic, retroesophageal segment

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4. Cervical Aortic Arch

• Presentations
• Pulsatile masses in supraclavicualar fossa in
  neck
• DD aneurysm of carotid/ SCA
• Differentiation compression of pulsatile mass ?
  loss of femoral pulse
• Vascular ring
• Subclavian steal syndrome
• CxR
• Wide upper mediastinum absent aortic knob
• Anterior deviation of trachea

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4. Cervical Aortic Arch

• Rx necessary
• If hypoplasia of cervical arch
• Symptomatic vascular ring
• Aneurysm of cervical arch itself

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Anatomical Categories

• Abnormalities of branching
• Normal L Aortic Arch Variants
• Abnormal L Aortic Arch
• Abnormalities of arch position
• 3. R Aortic Arch
• 4. Cervical Aortic Arch
• Superpneumarary arches
• 5. Double Aortic Arch
• 6. Persistent Fifth AA
• 7. Interrupted Aortic Arch
• 8. Anomalous origin of PA branches and other AA
  anomalies

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5. Double Aortic Arch

• Both R L arches persist
• Vascular ring
• Variations
• Hypoplasia of one arch (usually L)
• Atresia of one arch (usually L)
• Both arches widely patent
• R arch is more superiorly located

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5. Double Aortic Arch

• Double AA with both arches patent
• Symmetrical origin of 4 brachiocephalic Aa

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5. Double Aortic Arch

• 2. Double AA with atretic L arch distal to the
  origin of L SCA
• Similar to mirror image RAA (but with L Desc Ao)
• Indistinguishable (except at Sx) from RAA with L
  DA

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5. Double Aortic Arch

• 3. Double AA with atretic segment between L CCA
  and L SCA
• Similar to RAA with diverticulum of Kommerell

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5. Double Aortic Arch

• Atretic R arch
• Rare
• Can simulate L atresia patterns

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5. Double Aortic Arch

• Descending aorta could be L or R
• Rarely ass. with CHD -
• TOF is most common
• TGA
• Embryological explanation
• Both 4th arches and dorsal aortae persist
• But usually only one 6th arch (ductus)

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5. Double Aortic Arch

• Clinical features
• vascular ring syms depend on tightness of ring
• When both arches widely patent ? tight ring ?
  stridor in 1st wk
• Atretic L arch ? loose ring ? present at 3-6/12
  or later
• Rarely double AA present in adulthood with
  swallowing/resp. syms
• Diagnosis
• CxR RAA indent trachea superiorly and LAA
  inferiorly
• Ba oeso, Echo, Angio, MRI ? confirm diagnosis

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5. Double Aortic Arch

• Mx
• If symps due to vascular ring ? Sx division
• If undergoing Sx for other CHD ? division
• Ring should be divided in the smaller limb
• Ligamentum also should be divided

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Anatomical Categories

• Abnormalities of branching
• Normal L Aortic Arch Variants
• Abnormal L Aortic Arch
• Abnormalities of arch position
• 3. R Aortic Arch
• 4. Cervical Aortic Arch
• Superpneumarary arches
• 5. Double Aortic Arch
• 6. Persistent Fifth AA
• 7. Interrupted Aortic Arch
• 8. Anomalous origin of PA branches and other AA
  anomalies

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6. Persistent Fifth AA

• Rare
• Both arches appear on the same side of trachea
• Can be ass with COA
• 3 Subtypes
• Except for COA 1st 2nd subtypes no
  physiological significance

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6. Persistent Fifth AA

• Double lumen AA with both lumina patent
• Frequently ass with major cardiac anomaly

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6. Persistent Fifth AA

• Atresia/interruption of the superior arch (4th)
  with patent inferior (5th) arch
• Common origin of all brachiocephalic vessels from
  the ascending aorta
• Can be ass with COA

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6. Persistent Fifth AA

• Systemic to pulmonary artery connection arising
  proximal to 1st brachiocephalic Vv
• Only in pulmonary atresia
• 5th arch remnant arises as the 1st branch of the
  Asc Ao connects to the junction of MPA and one
  branch PA
• Ipsilateral/contralateral to definitive AA (4th)

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6. Persistent Fifth AA

• Diagnosis
• Subway vessel beneath the normal arch
• In atresia of superior arch ? common
  brachiocephalic trunk with all 4 vv arising from
  single v
• Branching pattern ? persistent 5th arch
• Atretic segment not visualized in Ixs
• At Sx fibrous band between L SCA and Desc Ao

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Anatomical Categories

• Abnormalities of branching
• Normal L Aortic Arch Variants
• Abnormal L Aortic Arch
• Abnormalities of arch position
• 3. R Aortic Arch
• 4. Cervical Aortic Arch
• Superpneumarary arches
• 5. Double Aortic Arch
• 6. Persistent Fifth AA
• 7. Interrupted Aortic Arch
• 8. Anomalous origin of PA branches and other AA
  anomalies

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7. Interrupted Aortic Arch

• Complete separation of ascending and descending
  aorta
• Determination of sidedness of AA
• Branching pattern- 1st Br. Prox to Int. contains
  a Carotid a. opposite the side of the AA
• Retroesophageal/ isolated subclavian a is always
  opposite the side of the arch
• Importance of sidedness
• Interrupted R AA only seen in ass with Digeorge
  syndrome

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7. Interrupted Aortic Arch

• 3 main categories ? 9 sub categories
• Main categories
• Interruption distal to SCA that is ipsilateral to
  2nd Carotid A
• Interruption between 2nd carotid and ipsilateral
  SCA
• Interruption between carotid arteries
• Subcategories
• Without retro-esophageal or isolated SCA
• With retro-esophageal SCA
• With isolated SCA

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7. Interrupted Aortic Arch

• Interruption distal to SCA that is ipsilateral to
  2nd Carotid A
• associations
• Aortico-pulmonary septal defects Intact IVS
• TGA Interrupted AA

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7. Interrupted Aortic Arch

• Interruption between 2nd carotid and ipsilateral
  SCA
• Without retro-esophageal or isolated SCA
• More common than type A

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7. Interrupted Aortic Arch

• Interruption between 2nd carotid and ipsilateral
  SCA
• With retro-esophageal SCA
• Digeorge syndrome interruption ?have type B

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7. Interrupted Aortic Arch

• Interruption between carotid arteries
• Rare

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7. Interrupted Aortic Arch

• Associations
• Digeorge syndrome Vs IAA / Truncus
• 43 of Digeorges had type B interruption
• 68 of IAA had Digeorge
• 34 of Digeorges had TA
• 33 of TA had Digeorge

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7. Interrupted Aortic Arch

• Presentation
• Duct dependant L heart obstructive lesions
• Acute cardiovascular collapse / heat failure
  after spont closure of PDA after 1st few days of
  life
• Initial Mx
• Fluid resuscitation
• Induction and maintenance of ductal patency with
  PGE1
• Inotropic support SOS
• Clinical features
• pulse discrepancy depends on branching pattern
• Absence of all limb pulses ? type B interruption
  with anomalous SCA
• DD - critical AS (carotid pulse is also week)

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7. Interrupted Aortic Arch

• Differential cyanosis
• pink upper body blue lower body
• Uncommonly seen bse pulm blood is also highly
  saturated due to large L?R shunt through VSD

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7. Interrupted Aortic Arch

• Diagnosis
• Echocardiogram
• Most important tool for diagnosis of IAA
• Suspect when
• Marked discrepancy between Asc Ao and MPA
  malalignment VSD posterior deviation of
  infundibular septum (PS LAX)
• Angiography
• Difficult bse high flow through VSD ? poor image
  quality of Asc Ao
• Can diagnose when both carotids prox and both SCA
  distal to interruption
• Wide separation of carotids from Desc Ao ? IAA

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7. Interrupted Aortic Arch

• Management
• Sx approach depend on degree of subaortic
  obstruction
• Subaortic diameter gt 5-6 mm ? 1ry repair
• (patch closure of VSD Ao Arch reconstruction)
• Subaortic diameter lt 3 mm inadequate to support
  normal COP

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7. Interrupted Aortic Arch

• PA banding
• is not a satisfactory palliation for VSD with
  interrupted Ao A
• Will lead to BVH with progressive subaortic
  stenosis ? complicate definitive repair
• Repair of Ao Arch
• direct anastomosis homograft augmentation
• In infancy avoid artificial tube grafts
• Rapidly overgrown
• Fibrous encasement complicate later repair

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Anatomical Categories

• Abnormalities of branching
• Normal L Aortic Arch Variants
• Abnormal L Aortic Arch
• Abnormalities of arch position
• 3. R Aortic Arch
• 4. Cervical Aortic Arch
• Superpneumarary arches
• 5. Double Aortic Arch
• 6. Persistent Fifth AA
• 7. Interrupted Aortic Arch
• 8. Anomalous origin of PA branches and other AA
  anomalies

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8. Other Anomalies of the Aortic Arch System

• Anomalous origin of the pulmonary artery from the
  ascending aorta
• Anomalous origin of the LPA from the RPA
• Innominate artery compression of the trachea

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8.1 Anomalous origin of the pulmonary artery from
the ascending aorta

• One branch PA arising from Asc Ao MPA arising
  separately from the heart
• RPA more commonly arise from Ao (82)

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8.1 Anomalous origin of the pulmonary artery from
the ascending aorta

• Investigations
• CxR differential PBF (esp in TOF with oligemia)
• Echo diagnostic
• Carefully search for origins of both PAs in TOF
• Cardiac catheterization
• Only one branch PA can be reached through RV
• MRI - diagnostic

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8.2 Anomalous origin of LPA from RPA

• Pulm artery sling ? partially surround lower
  trachea
• only situation with major vascular structure
  passing between trachea and oesophagus
• ass with complete cartilaginous rings in distal
  trachea ?Tracheal stenosis
• (need direct surgical treatment in addition to
  relief from vascular compression)

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8.2 Anomalous origin of LPA from RPA

• Sx
• division of LPA from RPA and reanastomosis in
  front of the trachea
• Transect the trachea and reanastomose behind the
  PA bifurcation
• If complete cartilaginous tracheal rings ?
  tracheal reconstruction

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8.3 Innominate Artery Compression of Trachea

• Poorly understood condition
• Anterior compression of the trachea at the point
  where it is crossed by the innominate artery
• Suspect
• When signs of severe insp and exp stridor in a
  2-6 mo old anterior indentation of the tracheal
  air column in lateral CxR

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8.3 Innominate Artery Compression of Trachea

• Rx
• Wait for tracheomalacia to resolve typically by
  age 2yrs
• In patients with apnea/ repeated LRTI surgical
  suspension of the innominate artery from the
  sternum

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Summary

• Aortic arch anomalies and ring malformations can
  be worked out from the basic embryologic
  structure
• So try to remember the embryology rather than
  anomalies itself
• Can argue against certain lesions eg RAA with R
  innominate
• Diagnosis has become easier with 3D
  reconstruction
• Treatment needed only when symptomatic or
  associated with other cardiac problems

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Thank You