Aortic arch anomalies

1. AORTIC ARCH ANOMALIES
DR. SUMIT SHANKER
MODERATOR – DR. Y.V. SUBBA REDDY

2. INTRODUCTION
• Account for 15% to 20% of all congenital cardiovascular
diseases.
• Result from errors in the embryologic development of
the branchial arches, including errors of involution or
migration, or abnormal persistence of vascular
structures.
• Strong associations of arch anomalies with chromosomal
and genetic abnormalities are supported by studies
demonstrating a deletion within chromosome

3. • Can be discovered when there are symptoms of airway or
esophageal compression produced by vascular rings, or
anomalies can be found incidentally on imaging studies
obtained for other reasons

4. 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 VASCULAR RING COINED TERM
DYSPHAGIA LUSORIA.
• GROSS,1945-FIRST DIVISION OF A VASCULAR RING.

5. 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
- BEAT BY 22ND DAY.
• BULBUS CORDIS REPRESENTS ARTERIAL END OF THE TUBE- PROX
PART CONUS, DISTAL TRUNCUS ARTERIOSUS.

7. • FIRST ARTERIES TO APPEAR ARE RIGHT AND LEFT
PRIMITIVE AORTA CONNECTED TO THE ENDOTHELIAL
HEART TUBES
 Portion ventral to foregut -
ventral aorta
 Portion dorsal to foregut –
dorsal aorta

8. • AFTER FUSION OF HEART TUBES, VENTRAL AORTA FUSE -
AORTIC SAC
• 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.

11. FATE OF THE AORTIC ARCHES
• FIRST PAIR: A SMALL PART FORMS THE MAXILLARY ARTERY, THE REST
DISAPPEARS.
• SECOND PAIR: A SMALL PART FORMS THE STAPEDIAL & HYOID ARTERIES,
THE REST DISAPPEARS.
• THIRD PAIR: FORMS THE COMMON, INTERNAL & EXTERNAL CAROTID
ARTERIES ON EACH SIDE.
• FOURTH PAIR: FORMS THE AORTIC ARCH (AA) ON THE LEFT SIDE, AND
THE RIGHT SUBCLAVIAN (RSC) ARTERY ON THE RIGHT SIDE.
• FIFTH PAIR: TOTALLY DISAPPEARS.
• SIXTH PAIR: FORMS THE RIGHT & LEFT PULMONARY ARTERIES (PA) AND
THE DUCTUS ARTERIOSUS (DA).

12. • 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

14. • SPIRAL SEPTUM FORMED IN TRUNCUS IN THE 5TH WEEK
EXTENDS TO AORTIC SAC. BLOOD FROM PULMONARY
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

15. BRANCHES OF AORTA
• INNOMINATE ARTERY - RIGHT BRANCH OF TRUNCOAORTIC SAC
• RIGHT COMMON CAROTID ARTERY - RIGHT THIRD ARCH
• RIGHT SUBCLAVIAN - RIGHT FOURTH ARCH AND RIGHT DORSAL
AORTA PROXIMALLY AND RIGHT SEVENTH INTERSEGMENTAL
ARTERY DISTALLY
• LEFT CAROTID - LEFT THIRD AORTIC ARCH.
• LEFT SUBCLAVIAN - LEFT SEVENTH INTERSEGMENTAL ARTERY.

17. AORTIC ARCH ANOMALIES

18. EDWARD’S DOUBLE AORTIC ARCH MODEL
• ANOMALIES OF AORTIC ARCH TO BE CONCEPTUALIZED AS
VARIATIONS IN REGRESSION OF DIFFERENT SEGMENTS FROM A
“HYPOTHETICAL DOUBLE ARCH”.
• THE CONCEPT OF “HYPOTHETICAL DOUBLE AORTIC ARCH”
EMPHASISES THE POTENTIAL CONTRIBUTION OF NEARLY ALL
EMBRYONIC ARCHES TO COMPONENTS OF DEFINITIVE ARCH
SYSTEM.
• DEMONSTRATE POSSIBLE EMBRYOLOGIC EXPLANATIONS FOR EACH
ARCH ANOMALY.

20. TOTIPOTENTIAL AORTIC ARCH
D Ao, descending aorta; Dors Ao, dorsal aorta(e); E, esophagus; LPA, left pulmonary artery; RPA,
right pulmonary artery; T, trachea; TA Sac, truncoaortic sac aortic and pulmonary artery
components; III, IV, VI refer to third, fourth, sixth embryonic arches, respectively, 7 IS, seventh
intersegmental artery.

21. NORMAL LEFT ARCH
• NORMAL LEFT AORTIC ARCH IS DERIVED FROM
• AORTIC PORTION OF THE EMBRYONIC TRUNCUS
ARTERIOSUS
• LEFT BRANCH OF THE TRUNCOAORTIC
SAC
• LEFT FOURTH ARTERIAL ARCH
• LEFT DORSAL AORTA

22. LEFT AND RIGHT ARCH DEFINITION
• LEFT AND RIGHT AORTIC ARCH- REFER TO WHICH BRONCHUS IS
CROSSED BY THE ARCH, NOT TO WHICH SIDE OF THE MIDLINE THE
AORTIC ROOT ASCENDS.
• SIDEDNESS DETERMINED WITH ECHO OR ANGIOGRAPHY BY THE
BRANCHING PATTERN OF THE BRACHIOCEPHALIC VESSELS.
• GENERAL RULE – 1ST ARCH VESSEL CONTAIN A CAROTID ARTERY.
CONTRALATERAL TO AORTIC ARCH

23. AORTIC ARCH ANOMALIES
CLASSIFICATION

24. ANATOMICAL CLASSIFICATION
• 1)ABNORMALITIES OF BRANCHING
• 2)ABNORMALITIES OF ARCH POSITION INCLUDING RIGHT AORTIC
ARCH & CERVICAL AORTIC ARCH
• 3)SUPERNUMERARY ARCHES INCLUDING DOUBLE AORTIC ARCH
AND PERSISTENT FIFTH AORTIC ARCH
• 4)INTERRUPTED AORTIC ARCH
• 5)ANOMALOUS ORIGIN OF A PULMONARY ARTERY BRANCH FROM
THE ASCENDING AORTA OR FROM THE CONTRALATERAL
PULMONARY ARTERY BRANCH.

25. CLINICAL CLASSIFICATION
• VASCULAR RINGS.
• NON-RING VASCULAR COMPRESSION.
• NON-COMPRESSIVE ARCH MALFORMATIONS.
• DUCTAL DEPENDENT ARCH ANOMALIES- INTURRUPTED
AORTIC ARCH , ISOLATED SUBCLAVIAN, CAROTID OR
INNOMINATE ARTERIES.

26. VASCULAR RING
• AORTIC ARCH ANOMALY IN WHICH TRACHEA AND ESOPHAGUS
SURROUNDED BY VASCULAR STRUCTURES.
• THE VASCULAR STRUCTURE NEED NOT TO BE PATENT E.G.
LIGAMENTUM ARTERIOSUM.
• DOUBLE AORTIC ARCH MOST COMMON(40%)
• RT. AORTIC ARCH WITH LEFT LIGAMENTUM(30%)
• ABERRANT RSCA(20%)
• ANOMALOUS INNOMINATE(10%)

27. • SYMPTOMS-
◦ STRIDOR, PNEUMONIA, BRONCHITIS
◦ REFLEX APNOEA OR CHOKING ON EATING.
◦ POSTURE OF HYPEREXTENSION OF NECK IN INFANT.
◦ INCREASED RESPIRATORY DISTRESS A/W INTERCURRENT
RESPIRATORY INFECTIONS.
◦ SWALLOWING DIFFICULTY
◦ OCCASIONALLY, IN PATIENTS WITH ASSOCIATED
INTRACARDIAC ANOMALIES ,THE RESPIRATORY SYMPTOMS MAY
BE MISTAKENLY BE ATTRIBUTED TO CARDIAC DISEASE.

28. • THE VASCULAR RING MAY BE SUSPECTED FROM COMBINATION OF
THE HISTORY AND PLAIN CHEST FILM .
• WHEN ALL ELEMENTS OF THE RING ARE PATENT, VISUALIZATION,
ESPECIALLY BY TOMOGRAPHIC IMAGING, IS STRAIGHTFORWARD.
• IN CASES WHERE THE RING IS COMPLETED BY AN ATRETIC
SEGMENT OF AORTA OR LIGAMENTUM ARTERIOSUM, THOSE
SEGMENTS CANNOT BE VISUALIZED WITH CURRENT IMAGING
TECHNOLOGIES.
• HOWEVER, THESE RINGS ARE RECOGNIZABLE BY THE PRESENCE OF
ONE OF THREE “D” OPPOSITE THE SIDE OF THE AORTIC ARCH.

29. IDENTIFICATION OF VASCULAR RING
• 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

30. VARIANTS OF LEFT AORTIC ARCH
COMMON BRACHIOCEPHALIC TRUNK (BOVINE ARCH)
• 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
• NO FUNCTIONAL SIGNIFICANCE

31. • THE SECOND MOST COMMON PATTERN OF HUMAN AORTIC ARCH BRANCHING
HAS A COMMON ORIGIN FOR THE INNOMINATE AND LEFT COMMON CAROTID
ARTERIES.

32. ABNORMAL LEFT ARCH
• A) LT ARCH WITH RETROESOPHAGEAL RSCA
• B) LT AORTIC ARCH AND RETROESOPHAGEAL DIVERTICULUM OF
KOMMERELL
• C) LT AORTIC ARCH, RT.DESCENDING AORTA,
RT.DUCTUS(CIRCUMFLEX AORTIC ARCH)
• D) LT AORTIC ARCH & ISOLATED RSCA
• E) LT AORTIC ARCH WITH CERVICAL ORIGIN OF RT SUBCLAVIAN.

33. LT ARCH WITH RETROESOPHAGEAL RSCA
• ALSO KNOWN AS ANOMALOUS OR ABERRANT RIGHT SUBCLAVIAN
ARTERY .
• BAYFORD LINKED SUCH CASE WITH HISTORY OF DIFFICULTY IN
SWALLOWING AND COINED TERM “DYSPHAGIA LUSORIA”
• 0.5% INCIDENCE IN GENERAL POPULATION.
• M.C. AORTIC ARCH ANOMALY
• INCIDENCE IN DOWN’S SYNDROME WITH CHD IS VERY HIGH (38%).

34. • DISAPPEARANCE OF RT 4TH ARCH-DISTAL RT DORSAL
AORTA BECOMES PROX RSCA FORMING ITS
RETROESOPHAGEAL PORTION.
• RT 6TH ARCH (DUCTUS) DISAPPEAR.
• USUALLY ASYMPTOMATIC.
• BARIUM –SMALLER FIXED FILLING DEFECT ON POST
ASPECT OF ESOPHAGUS SLANTING UPWARD TO RIGHT
• ANGIO- EARLIER FILLING OF RT CAROTID ON AORTIC
ROOT INJECTION.

37. LT AORTIC ARCH AND RETROESOPHAGEAL
DIVERTICULUM OF KOMMERELL
• FIRST VASCULAR RING TO BE DIAGNOSED DURING LIFE
WITH BARIUM STUDY.
• SIMILAR TO PREVIOUS EXCEPT FOR PERSISTENT RIGHT
6TH ARCH-LIGAMENTUM WHICH COMPLETES A VASC.
RING
• PROX. RSCA DILATED TO FORM DIVERTICULUM.

40. • PLAIN CHEST FILM : LEFT SIDED AORTIC ARCH
WITHVRIGHT SIDED UPPER DESCENDING AORTA.
• BARIUM ESOPHAGOGRAPHY : LARGE
POSTERIORVINDENTATION FROM RETROESOPHAGEAL
AORTA .
• ANGIOGRAPHY WILL CONFIRM THE COURSE OF AORTA.
• IN SYMPTOMATIC PATIENTS THE RING IS DIVIDED VIA
RIGHT THORACOTOMY APPROACH.

41. LT AORTIC ARCH, RT. DESCENDING AORTA,
RT. DUCTUS(CIRCUMFLEX AORTIC ARCH)
• BRANCHING PATTERN SIMILAR TO EARLIER
• ARCH RETROESOPHAGEAL
• RSCA THE LAST ARCH VESSEL .
• DESCENDING AORTA CONNECTED TO RPA BY LIGAMENTUM-
FORMS VASULAR RING
• EMBRYOLOGY: DISAPPEARANCE OF RT 4TH ARCH BUT WITH LEFT
DISTAL DORSAL AORTA FORMING DEFINITIVE DISTAL ARCH AND
PASSING RETROESOPHAGEALLY TO A DESCENDING AORTA
BEGINNING TO RIGHT OF VERTEBRAL COLUMN.

42. • PLAIN CHEST FILM : LEFT SIDED AORTIC ARCH WITH RIGHT SIDED
UPPER DESCENDING AORTA.
• BARIUM ESOPHAGOGRAPHY : LARGE POSTERIOR INDENTATION
FROM RETROESOPHAGEAL AORTA .
• ANGIOGRAPHY WILL CONFIRM THE COURSE OF AORTA.
• IN SYMPTOMATIC PATIENTS THE RING IS DIVIDED VIA RIGHT
THORACOTOMY APPROACH.

43. The descending aorta is connected by a ductus or ligamentum to the right pulmonary
artery (RPA) forming a vascular ring.

44. LT AORTIC ARCH & ISOLATED RSCA
• RSCA ARISES ONLY FROM RT DUCTUS.
• RIGHT 6TH ARCH PERSISTS WITH DISSOLUTION OF RIGHT 4 TH
ARCH AND RIGHT DORSAL AORTA.
• 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

45. • ANGIOGRAPHY : DELAYED FILLING OF RSCA AFTER AORTIC ROOT
SHOOT .
• WITH PHASE ENCODED VELOCITY MAPPING ,RETROGRADE FLOW IN
THE VERTEBRAL ARTERY CAN BE DETECTED ON MRI.
• SYMPTOMATIC PATIENTS ARE TREATED WITH IMPLANTATION OF
SUBCLAVIAN ARTERY INTO AORTA.

46. LT AORTIC ARCH WITH CERVICAL ORIGIN OF
RT SUBCLAVIAN
• MARKER OF 22Q11 DELETION. (MARKER OF CATCH 22)
• INNOMINATE TRIFURCATES IN THE NECK-RSCA TRAVELS
BACK TO THORAX
• SUBCLAVIAN ARTERY ARISES FROM 3RD ARCH INSTEAD
OF 4 TH .
• 3 RD ARCH BEING MORE CEPHALAD GIVES ORIGIN TO
RSCA IN THE NECK.

48. 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
• 8-10% IN TRANSPOSITION.

49. RIGHT AORTIC ARCH TYPES
• A) RIGHT AORTIC ARCH-MIRROR IMAGE TYPE BRANCHING
• B) RT AORTIC ARCH WITH RETROESOPHAGEAL DIVERTICULUM OF
KOMMERELL
• C) RT ARCH WITH RETROESOPHAGEAL LSCA
• D) RT ARCH WITH LT DESC AORTA AND LT LIGAMENTUM
• E) RIGHT AORTIC ARCH WITH RETROESOPHAGEAL INNOMINATE
ARTERY
• F) RIGHT AORTIC ARCH WITH ISOLATION OF CONTRALATERAL
ARCH VESSEL

50. RIGHT AORTIC ARCH-MIRROR IMAGE TYPE
BRANCHING
• SEQUENCE OF ARCH VESSELS- LT.INNOMINATE, RT CAROTID,
RSCA.
• LIGAMENTUM LT SIDED.
• NO VASCULAR RING.
• ALMOST ALWAYS ASSOCIATED WITH CHD (48% TOF)

52. • DISSOLUTION OF LEFT DORSAL AORTA DISTAL TO THE ORIGIN OF
LEFT 7 TH INTERSEGMENTAL ARTERY
• SO, LEFT 4TH ARCH BECOMES PROXIMAL LEFT SUBCLAVIAN
ARTERY.
• LEFT SIXTH ARCH PERSISTS- LEFT DUCTUS ARISES FROM
UNDERSIDE OF LEFT INNOMINATE ARTERY AND PASSES TO LPA .
• ALTERNATIVELY RIGHT DUCTUS PERSISTS GIVING TRUE MIRROR
IMAGE OF NORMAL.

54. RT AORTIC ARCH WITH RETROESOPHAGEAL
DIVERTICULUM OF KOMMERELL
• SECOND MOST COMMON VASCULAR RING .
• 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
• SHOULD BE DIFFERENTIATED FROM RT AORTIC ARCH
WITH RETROESOPHAGEAL SUBCLAVIAN ARTERY.

55. • SYMPTOMS OF VASCULAR RING WITH RIGHT SIDED AORTIC ARCH ON
PLAIN CHEST FILM SHOULD RAISE THE SUSPICION OF THIS ANOMALY .
• BARIUM STUDY REVEALS A LARGE POSTERIOR INDENTATION ON THE
ESOPHAGUS.
• BRANCHING PATTERN OF ARCH VESSELS ON ECHO.
• ANGIOGRAPHY REVEALS CHARACTERISTIC BRANCHING PATTERN AND
ABRUPT CHANGE IN CALIBRE FROM DIVERTICULUM TO SUBCLAVIAN
ARTERY.
• MRI IDEAL FOR DIAGNOSIS .
• SURGICAL DIVISION OF DUCTUS IN SYMPTOMATIC PATIENTS VIA LEFT
THORACOTOMY.

57. RT ARCH WITH RETROESOPHAGEAL LSCA
• SIMILAR TO PREVIOUS ONE EXCEPT FOR THE ABSENCE OF
RETROESOPHAGEAL DIVERTICULUM.
• SEQUENCE – LT CAROTID, RT CAROTID ,RSCA, RETROESOPHAGEAL
LSCA.
• DUCTUS IS RT SIDED.
• NO VASCULAR RING.
• INVOLUTION OF LEFT 4TH AND 6TH AORTIC ARCHES.
• ASSOCIATED WITH CONOTRUNCAL ANOMALIES .
• HIGHER INCIDENCE OF 22Q11 DELETION.

58. RT ARCH WITH LT DESC AORTA AND LT
LIGAMENTUM
• ALSO KNOWN AS CIRCUMFLEX RIGHT AORTIC ARCH.
• AORTIC ARCH ITSELF CROSSES MIDLINE TO THE LEFT AT THE LEVEL OF T4
VERTEBRAL BODY -CONNECTS TO LT DUCTUS TO FORM VASCULAR RING
• SEQUENCE –LT INNOMINATE, RT CAROTID, RSCA OR LT CAROTID, RIGHT
CAROTID, RIGHT SUBCLAVIAN, FINALLY LEFT SUBCLAVIAN.
• AORTIC ARCH IS RETROESOPHAGEAL AND NOT THE SUBCLAVIAN ARTERY.

59. • EMBRYOLOGY : DISSOLUTION OF EITHER LEFT DORSAL AORTA
DISTAL TO TAKEOFF OF THE LEFT SUBCLAVIAN ARTERY OR THE
LEFT FOURTH ARCH. PERSISTENT LEFT SIXTH ARCH CONNECTS TO
THE LEFT SIDED DORSAL AORTA COMPLETING VASCULAR RING .
• BARIUM STUDY :DOWNWARD SLANTING LEFT OESOPHAGEAL
INDENTATION .
• MOBILISATION OF RETROESOPHAGEAL AORTIC SEGMENT AND
REANASTOMOSIS OF ASC AND DEC AORTA USING TUBE GRAFT
MAY BE NEEDED IN SYMPTOMATIC ADULTS IN ADDITION TO SIMPLE
DIVISION OF LIGAMENTUM.

60. RIGHT AORTIC ARCH WITH
RETROESOPHAGEAL
INNOMINATE ARTERY
• SEQUENCE : RIGHT CAROTID, RIGHT SCA, F/B RETROESOPHAGEAL LEFT
INNOMINATE ARTERY .
• DUCTUS OR LIGAMENTUM CONNECTS LPA WITH BASE OF INNOMINATE A.
COMPLETING VASCULAR RING.
• EMBRYOLOGY : DISSOLUTION OF LEFT BRANCH OF TRUNCOAORTIC SAC AND
LEFT 4 TH ARCH THE LEFT DORSAL AORTA SUPPLIES LEFT 7TH IS ARTERY AND
LEFT 3 RD ARCH.
• CLUE FOR DIAGNOSIS IS SINGLE CAROTID ARTERY ARISING FROM PROXIMAL
AORTA.
• IN SYMPTOMATIC PATIENTS SURGICAL DIVISION OF LIGAMENTUM WITH
IMPLANTATION OF INNOMINATE ARTERY TO ASCENDING AORTA IS REQUIRED.

61. RIGHT AORTIC ARCH WITH ISOLATION OF
CONTRALATERAL ARCH VESSEL
• VESSEL ARISES EXCLUSIVELY FROM PULMONARY ARTERY VIA DUCTUS ARTERIOSUS
BUT WITHOUT CONNECTION TO AORTA.
• 3 FORMS : ISOLATION OF LSCA (M.C.), ISOLATION OF LEFT CAROTID AND ISOLATION
OF LEFT INNOMINATE .
• CHD IN MORE THAN 50% (M.C. TOF).
• PRESENTATION: DIMINISHED PULSE AMPLITUDE OR BLOOD PRESSURE IN LEFT ARM ,
SUBCLAVIAN STEAL SYNDROME, PULMONARY ARTERY STEAL (IF DUCT PATENT)
• THE DIAGNOSIS SHOULD BE SUSPECTED IN PT WITH RIGHT SIDED AORTIC ARCH WITH
DIMINISHED PULSE AMPLITUDE OR BLOOD PRESSURE IN LEFT ARM .
• DELAYED FILLING OF LSCA VIA VERTEBRAL ARTERY DURING AORTIC ARCH SHOOT
CONFIRMS DIAGNOSIS.

62. CERVICAL AORTIC ARCH
• ARCH FOUND ABOVE LEVEL OF CLAVICLE
• TWO CATEGORIES-
• 1.NORMAL BRANCHING PATTERN
• 2.ANOMALOUS SUBCLAVIAN ARTERY AND VASCULAR RING WITH
DESCENDING AORTA CONTRALATERAL TO ARCH OR
RETROESOPHAGEAL DIVERTICULUM.
• 2ND GROUP-DIVIDED ACCORDING TO CAROTID ORIGIN (BICAROTID
TRUNK OR SEPARATE ORIGIN OF EXT & INT CAROTID)
• FIRST GROUP USUALLY HAS LEFT SIDED ARCH WHILE SECOND GROUP
HAS RIGHT SIDED.
• SEPARATE ORIGIN OF VERTEBRAL ARTERY CAN BE SEEN IN BOTH GROUPS.

63. • 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.
• PRESENTATION :
- PULSATILE MASS IN SUPRACLAVICULAR FOSSA OR THE NECK.
- SYMPTOMS OF VASCULAR RING
- SUBCLAVIAN STEAL IN PATIENTS WITH STENOSIS OR ATRESIA OF
LSCA .
• PRESUMPTIVE DIAGNOSIS CAN BE MADE BY NOTATION OF LOSS OF
FEMORAL PULSE DURING BRIEF COMPRESSION OF THE MASS.

64. DOUBLE AORTIC ARCH
• BOTH RT AND LT ARCHES PRESENT.
• BOTH ARCHES CAN BE PATENT OR ONE HYPOPLASTIC OR ATRETIC(USUALLY
LEFT)
• PERSISTENCE OF BOTH RT AND LT 4TH ARCH WHICH JOIN TA SAC TO THEIR
RESPECTIVE DORSAL AORTAE
• ONLY ONE 6TH REMAIN.
• SYMMETRIC ORIGIN OF 4 ARCH VESSELS FROM RESPECTIVE ARCHES WHEN BOTH
PATENT
• RARELY A/W OTHER CHD, WHEN PRESENT-TOF MOST COMMON.
• FORM COMPLETE VASCULAR RINGS.
• WHEN BOTH ARCHES ARE PATENT, RINGS TYPICALLY TIGHT AND PRESENT WITH
STRIDOR IN FIRST WEEK OF LIFE.

66. • DIAGNOSIS CAN BE MADE ON PLAIN CHEST FILM WHEN BOTH ARCHES ARE
PATENT.
• TRACHEAL AIR COLUMN IS INDENTED BY MORE SUPERIOR RIGHT SIDED ARCH
AND INFERIOR LEFT SIDED ARCH.
• CONFIRMATION WITH FURTHER IMAGING IS IMPORTANT TO IDENTIFY ATRETIC
SEGMENT.
• SURGICAL DIVISION OF RING IF SYMPTOMATIC.

67. PERSISTENT FIFTH AORTIC ARCH
• FIRST REPORTED BY VAN PRAAGH IN 1969
• DOUBLE LUMEN AORTIC ARCH IN WHICH BOTH ARCHES APPEAR ON SAME SIDE
OF TRACHEA.
• FREQUENTLY ASSOCIATED WITH CHD; HOWEVER CAN BE INCIDENTAL FINDING
WITHOUT CLINICAL SIGNIFICANCE .
• 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

69. 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

70. ASSOCIATIONS
• TYPE A-AORTICOPULMONARY SEPTAL DEFECT,TGA
• TYPE B- MOST COMMON A/W CONOTRUNCAL ANOMALY
WITH LARGE MALALIGNED VSD WITH SUBAORTIC
OBSTRUCTION, DIGEORGE SYNDROME.
• TYPE C-RARE
• INTERRUPTED RT ARCH SEEN ONLY IN DIGEORGE
SYNDROME.

71. EMBRYOLOGY
• TYPE A-INVOLUTION OF BOTH DORSAL AORTA DISTAL TO 4TH
ARCH AND PROX TO PERSISTENT 6TH ARCH WHICH SUPPLIES
DESCENDING AORTA .
• 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,
ASSOCIATED PROXIMAL 3 RD ARCH AND ENTIRE 4 TH ARCH WITH
PERSISTENCE OF DORSAL AORTA BETWEEN 3RD AND 4 TH ARCH.

73. • PRESENT WITH ACUTE CARDIOVASCULAR COLLAPSE OR HEART FAILURE AFTER
CLOSURE OF DUCTUS IN FIRST DAYS OF LIFE.
• ABSENCE OF ALL LIMB PULSE WITH STRONG CAROTID PULSE SUGGEST TYPE B
WITH ANOMALOUS SUBCLAVIAN.
• 2 D ECHO IS MOST IMPORTANT DIAGNOSTIC TOOL .
• 3 D RECONSTRUCTIVE MRI IS USEFUL FOR IDENTIFYING BRANCHING PATTERN
AND SITE OF INTERRUPTION.

74. • SURGICAL APPROCH DEPENDS ON THE DEGREE OF SUBAORTIC
OBSTRUCTION .
• 1.IF SUBAORTIC DIAMETER > 5 MM  PRIMARY INTRACARDIAC
REPAIR.
• 2.IF SUBAORTIC DIAMETER < 3MM  BYPASS SUBAORTIC REGION –
ASSOCIATE MPA WITH ASCENDING AORTA USING HOMOGRAFT
AUGMENTATION TO COMPLETE AORTIC RECONSTRUCTION.
• AORTIC ARCH IS RECONSTRUCTED BY LIBERAL DISSECTION
AROUND TWO ARCH COMPONENTS WITH DIRECT ANASTOMOSIS
OF TWO ENDS.

75. ANOMALOUS ORIGIN OF THE PULMONARY
ARTERY FROM THE 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 THE
LEFTWARD MIGRATION 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 DEVELOPMENT OF PULMONARY VASCULAR DISEASE.

76. ANOMALOUS ORIGIN OF THE LPA
FROM THE RPA
• LPA ARISES FROM RPA AND PASSES B/W TRACHEA AND ESOPHAGUS-
PULMONARY ARTERY SLING.
• ONLY ANOMALY IN WHICH A MAJOR VASCULAR STRUCTURE PASSES BETWEEN
TRACHEA AND ESOPHAGUS.
• TRACHEAL COMPRESSION-SEVERE RESPIRATORY DISTRESS AND STRIDOR,MILDER
FORMS MAY EXISTS
• ISOLATED ANOMALY, RARELY A/W TOF
• ANTERIOR INDENTATION ON BARIUM SWALLOW.
• BRONCHOSCOPY NEEDED PRIOR TO SURGERY TO RULE OUT COMPLETE
CARTILAGENOUS RING

77. SURGICAL IMPLICATIONS
• SYSTEMIC TO PULMONARY SHUNTS ARE BEST CARRIED OUT USING
SIDE WITH INNOMINATE ARTERY.
• KNOWLEDGE OF SIDEDNESS OF AORTA MAY BE VERY USEFUL IN
THE REPAIR OF EOSOPHAGEAL ATRESIA OR TRACHEO-ESOPHAGEAL
FISTULA.
• ANATOMICAL VARIATIONS OR ANOMALIES IN BRACHIOCEPHALIC
ARTERIES MY POSE DIFFICULTY IN CAROTID ARTERY STENTING AS
WELL AS MAY INCREASE COMPLICATION RATES..

78. SUMMARY
• AORTIC ARCH ANOMALIES AND VASCULAR RINGS CAN BE
INTERPRETED ON THE BASIS OF EMBRYOLOGY
• WITH THE DEVELPOMENT OF MRI AND CT, 3-D RECONSTRUCTION
IS POSSIBLE.
• INTERVENTION REQUIRED ONLY WHEN SYMPTOMATIC OR WHEN
A/W OTHER CARDIAC ANOMALIES.