Aortic Arch Abnormalities

NHS.

Norman H Silverman MD, D Sc.(Med.). FACC, FAHA.

Stanford University & Lucile Packard Children’s Hospital

IPOK

.

E mail: norm.silverman@stanford.edu

www.MD1World.com

Abnormalities • Supravalvar Aortic Stenosis• The Aortic Root in Marfan’s Syndrome

• Interruption• Hypoplasia

• High Aortic Arch • Arch Laterality• Coarctation

• Vascular Rings

Examination Technique

• A variety views should be used including :-• Suprasternal coronal and sagittal imaging,• Parasternal and• Subcostal views of the aorta and its relations.• The Imaging should be complemented by

various Doppler modalities including Color flow imaging.

Normal Aortic Arch Echo

Normal Aortic ArchSSN Sagittal

AAo

RPA

IV

RBr

D Ao

Aortic Arch Laterality• Transducer alignment

– The arch direction follows the plane of the array• Innominate Artery

– The innominate artery always bifurcates on the opposite side to the aortic arch.

– Lack of bifurcation may indicate that there is an anomalous subclavian artery

• Tracheal Rings or Esophagus – The descending aorta can be recognized by the examiner

either to the left (in left aortic arch) or to the right (in right aortic arch)

Innominate Artery Shows Arch Position

NHS.

Right Innominate, therefore left arch

Left innominate, therefore right arch

Arch Laterality

A Left Arch

A

R Arch Trachea

Tracheal Rings

Arch Laterality

Right Arch

Left Arch

Routine Arch Sweep

LA

IV

LSA

DAoRPA

TAO

AAo

THYMUS

LCA

Normal Aorta: Color Imaging

Supravalvar Aortic Stenosis

Supravalvar Aortic Stenosis

William’s Syndrome

William’s Syndrome

Supravalvar Aortic Stenosis

L Ax

Ao

LA

RV

LV

Supravalvar Aortic Stenosis

Ao

LVRV

Ao

Subcost.Coronal

P L Ax

Bicuspid Aortic Valve & Multiple Dissections

Aortic Dissection, Coarctation & Bicuspid Aortic Valve

Bicuspid Aortic Valve: Post Stenotic DilatationAAo

LV

AAo

Marfan’s Syndrome

Marfan’s Syndrome

Mariska Kemna

Marfan’s Syndrome: Dilated Aortic Root

P Sax.RV

LA

RA

Ao

DAo

LXRV

Ao

LALA

Marfan’s Syndrome: Dilated Aortic Root

Aortic Regurgitation

Marfan’s Syndrome: Dilated Aortic Root

Marfan’s Syndrome: Dilated Aortic Root

Coarctation

Coarctation

Courtesy Diane Spicer

Coarctation

Pathological Varieties of Coarctation &Techniques of Display

NHS.

DUCTUS SLING IN COARCTATION

Doppler ∆ ofCoarctation

Coarctation

PA Shelf

DAo

Duct Beak

A Ao

NHS.

 Color Angiogram in Coarctation

RPA

DAO

Coarctation

Tr Ao Arch

Innominate Artery

L Subclavian

HYPOPLASTIC ISTHMUS

Ductus & ? Coarctation: On Prostaglandin

NHS.

MPA

Duct

Isthmus

? CoarctDAo

LPALA

Coarctation of the Aorta

NHS.

TAO

DAO

PA

OFF PGE1On PGE1

Taussig- Bing Malformation and Coarctation

PA

LA

IV

AAo

TAO

Coarct

D Ao

The Diversity of Coarctation

Coarctation-MRI 3 D Reconstruction

Courtesy of Dr. KerenHasbani

Coarctation: End - to - Side Anastomosis

SSN Cor.

Suture Line

IV

PA

D Ao

Fifth Aortic Arch

PMW ‘07

Interrupted Aortic Arch: Associations

• Ventricular septal defect• Catch 22 Syndrome • Truncus arteriosus• Aortopulmonary Window• Taussig Bing Malformation

Aortic Arch Interruption

C B A

Found with:- 22 Q-Truncus ArteriosusTaussig Bing Aortopulmonary Window VSD

Aortic Arch Interruption

Type B InterruptionAAo

PAD

LSA

Type B Interruption: Ductus & Descending Aorta

MPAAAo

LSA

Duct O AD

LA

NHS.

MPA

RL D

DA

O

LA

Interrupted Aortic Arch Sagittal Plane Imaging

INTERRUPTED ARCH TYPE B

AAo

Duct

LSA

D Ao

In. VnIn. A

Interrupted Arch: 2 Different Doppler Signals

NHS.

a

Truncus with Interrupted Aortic Arch

RVLV

*

A Ao

Tr

LPAD

The VSD in Interruption & Coarctation

Ao

LVRV

A4Ch.

P Lx

RV

Ao

LA

LV

In Coarctation and interruption of the aortic arch, all the woe, lies below!Richard Van Praagh.

Arch Embryology

Arch Embryology

Right Aortic Arch

Left Arch: Cervical Arch and Right Ductus

High ( Cervical ) Arch

Right PA

High ( Cervical ) Arch

Right PA

RAA- LPDAALSCA

DAA

BronchoscopyRT Mainstem Bronchus Compression

LT RT

Coronal Scan

Double Aortic Arch

R L

AAO

Double Aortic Arch

Fetal Double Aortic Arch

Double Aortic Arch

L Duct, Right Ao Arch

Interrupted Aortic Arch, R Desc. Ao & R Duct- Another Bronchial Vise

CP1281387-6

Right Arch with Aberrant Left Subclavian

Diverticulum of Kommerell's

Left ductus

Left subclavian

RING

CP1281387-6

Left ductus From innominate

Right ArchMirror Image Branching

Right Arch Mirror-image Branching and No Ring

NO RING

Right Aortic Arch – Aberrant L subclavian artery and L arterial duct

CP1281387-9

Left Aortic Arch with Aberrant Right Subclavian

Aberrant

RT SCA

Left Duct with Right Aortic Arch

EJCTS 2002

10 Reoperations

7 Primary operations

Resection of Kommerell’s diverticulum and left subclavian artery transfer for recurrent symptoms after vascular ring division

C.L. Backer, N. Hillman, C. Mavroudis, L.D. Holinger

Kommerell’s Diverticulum

Aortic diverticulum

Kommerell’s Diverticulum

Left Ductus: Right Arch

NHS.

Potentially Isolated L subclav.Art

BILATERAL DUCTS WITH ABSENT CENTRAL PA ‘S

Bilateral Ductus

Bilateral Ducts & Absent Central PA’s

Right Arch and Duct

Left Innominate and Duct

MAPCA’s

D A OLA

S Cost Sagittal

S SostCoronal

P Sx

Pulmonary Artery SlingFront

Back

CT Bronchial Tomography

Pulmonary artery Sling ECHO

LPA

PA Sling

Pulmonary Artery Sling

LPA

RPA

Pulmonary Artery Sling

AO

MPA

PSX

RPALPA

AOMPA

RPA

LPA

Thank You!