Mark Ferguson, M.D.

Seattle Children’s Hospital

University of Washington

Congenital Aortic Anomalies

16 mo with chronic cough

16 mo with chronic cough

Which vessel configuration is LEAST likely based on the

preceding images?

1. Right aortic arch with an aberrant left subclavian artery

2. Pulmonary sling

3. Double aortic arch

4. Left arch with an aberrant right

subclavian artery

Question 1

Which vessel configuration is LEAST likely based on the

preceding images:

1. Right aortic arch with an aberrant left subclavian artery

2. Pulmonary sling

3. Double aortic arch

4. Left arch with an aberrant right

subclavian artery

Answer 1

Double Aortic Arch

Double Aortic Arch

During embryonic vascular development, which aortic/branchial

arch contributes to the normal left-sided aortic arch?

1. II arch

2. III arch

3. IV arch

4. VI arch

Question 2

During embryonic vascular development, which aortic/branchial

arch contributes to the normal left-sided aortic arch?

1. II arch

2. III arch

3. IV arch

4. VI arch

Answer 2

Aortic Arch Development

Third arches: carotid arteries

Fourth arches: aortic arch (AA) on the left side, BCA on right

Sixth arches: right & left PA’s and the ductus arteriosus (DA) Seventh intersegmentals: subclavian arteries First, second, and fifth arches involute.

Based on Edwards’ hypothetical embryonic double aortic arch model

Normal Arch Development

Double Arch Development

Anatomy:

• double arches encircle the esophagus and

trachea (most common vascular ring)

• various forms, most commonly right arch is

dominant with a more superior apex and

descending aorta on the left

Epidemiology:

• vascular rings comprise 1% of surgically

managed cardiovascular malformations

(about half of those are double arches)

• no gender or race predilection

Double Aortic Arch

Clinical presentation:

• respiratory difficulties like stridor but also

including apparent life-threating events

• dysphagia, vomiting, choking, usually >2 yrs of age

Associations:

• usually without other cardiovascular anomalies

• 20% have chromosome band 22q11 deletion

Double Aortic Arch

Imaging:

• Chest Radiographs - may show indentation of trachea

or indeterminate arch sidedness

• Esophagram - bilateral and posterior indentation of

esophagus

• CT - anatomy, determine dominant arch for surgical

planning (contralateral thoracotomy),

evaluate airway compression

• MRI - anatomy, as CT

Double Aortic Arch

Right Aortic Arch with Aberrant Left Subclavian Artery

Right arch w/ ab LSC

interruption of the dorsal segment of the left arch

between the LCCA and LSCA with regression of the

right ductus arteriosus

• left ligamentum arteriosum can form a vascular ring

• Kommerell’s diverticulum - remnant of left dorsal

aortic root

• can be associated with a midline descending aorta

• Two major regions of potential airway compression

1. level of arch and ab subclavian artery

2. level of carina or main bronchi

Right Aortic Arch w/ ab LSC

9 yo with headaches

Aortic Coarctation

Focal Long Segment w/ Collaterals

Question 3

Which of the following is a characteristic of aortic coarctation?

1. Reverse figure-of-three sign on frontal chest radiograph

2. Associated with berry aneurysms of the circle of Willis

3. First and second rib notching secondary to collateral vessels

4. Right ventricular hypertrophy

Answer 3

Which of the following is a characteristic of aortic coarctation?

1. Reverse figure-of-three sign on frontal chest radiograph

2. Associated with berry aneurysms of the circle of Willis

3. First and second rib notching secondary to collateral vessels

supplied by costocervical trunk

4. Right ventricular hypertrophy

3

Pathophysiology:

• defect in vessel media, posterior infolding which

may be circumferential

• located near the ductus arteriosus

• can have tubular hypoplasia of arch

• post-stenotic dilation

• collateral vessels (intercostal and internal mammary)

Epidemiology:

• 5-10% of all congenital cardiac lesions

• untreated - < 20% survive to age 50 yrs

• males > females up to 2:1

Aortic Coarctation

Clinical presentation (depends on severity):

• infancy - CHF, acidosis, poor

perfusion of lower body

• post infancy - asymptomatic, murmur, htn,

HA, leg cramps, cold feet

Associations:

• Turner’s syndrome (XO)

• Bicuspid Aortic Valve

• VSD

• PDA

• Berry aneurysms of the circle of Willis

Aortic Coarctation

Imaging:

• Chest Radiographs - “3” sign, rib notching, edema, CM

• Esophagram - reverse “3” sign

• CT - anatomy (site, severity, morphology, collaterals)

• MRI

anatomy

gradient (peak velocities)

collateral flow using phase contrast techniques

evaluate for bicuspid AV, VSD, or LVH

Aortic Coarctation

6 yo with dysphagia

6 yo with dysphagia

Cervical Arch with Aneurysm

• perhaps formation of aortic arch

from third rather than fourth arch or

lack of caudal migration of 4th arch

• rare anomaly but with 20%

complicated by aneurysm

• often asymptomatic, can have

vascular ring-like symptoms or

supraclavicular pulsatile mass

6 day old with abnormal echo

Which type of interrupted aortic arch is demonstrated?

1. Type A

2. Type B

3. Type C

4. Type D

Question 4

Which type of interrupted aortic arch is demonstrated?

1. Type A

2. Type B

3. Type C

4. Type D

Answer 4

Interrupted Aortic Arch

33-42% Type A 53-65% Type B 1-4% Type C

Classification by Celoria and Patton

Interrupted Aortic Arch

Anatomy:

• complete discontinuity between ascending and

descending aorta

• simple or complex

simple - VSD and PDA

complex - truncus, TGA, double outlet RV

Epidemiology:

• 1% of congenital heart disease

• 25% have DiGeorge syndrome

Clinical presentation:

• heart failure (particularly with closure of ductus

arteriosus), cyanosis, poor peripheral pulses

Imaging:

• Chest Radiographs - CM with increased pulm

vascularity

and edema in critically ill newborn

midline

trachea and inconspicuous aortic knob

• CT - anatomy, determine type

• MRI - anatomy, as CT

Interrupted Aortic Arch

17 yo with scoliosis

5 cm

Which is often associated with Marfan syndrome?

1. Coagulopathy

2. Arterial tortuosity

3. Mitral valve prolapse

4. Coronary artery aneurysm

Question 6

Answer 6

Which is often associated with Marfan syndrome?

1. Coagulopathy

2. Arterial tortuosity - Loeys-Dietz feature

3. Mitral valve prolapse

4. Coronary artery aneurysm - Kawasaki’s feature

Marfan Syndrome

• Definition: Inherited connective tissue disorder with an

autosomal dominant transmission

• Incidence: 1 per 5000

• 25% represent new mutations

• Fibrillin protein dysfunction which prevents proper microfibril

formation.

• Premature medial degeneration with risk of aortic dilation and

subsequent dissection or rupture

Marfan Syndrome

Imaging:

screening and surveillance with MRA

evaluate for progressive aortic enlargement and

complications

References:

Edwards JE. Anomalies of the derivatives of the aortic arch system. Med Clin North Am

(1948) vol. 32 pp. 925 - 949.

Hernanz-Schulman. Vascular rings: a practical approach to imaging diagnosis. Pediatr

Radiol (2005) vol. 35 (10) pp. 961-79

Donnelly et al. Aberrant subclavian arteries: cross-sectional imaging findings in infants and

children referred for evaluation of extrinsic airway compression. AJR Am J Roentgenol

(2002) vol. 178 (5) pp. 1269-74

Farsak et al. Cervical aortic arch with aneurysm formation. Eur J Cardiothorac Surg (1998)

vol. 14 (4) pp. 437-9

Kimura-Hayama et al. Uncommon congenital and acquired aortic diseases: role of

multidetector CT angiography. Radiographics : a review publication of the Radiological

Society of North America, Inc (2010) vol. 30 (1) pp. 79-98

Yoo SJ, MacDonald C, Babyn P, eds. Chest Radiographic Interpretation in Pediatric Cardiac

Patients. New York, NY: Thieme Medical Publishers, Inc., 2010.

Ho V and Reddy G, eds. Cardiovascular Imaging. St. Louis, MO: Elsevier, 2011

Celoria GC, Patton RB. Congenital absence of the aortic arch. Am Heart J 1959;58:407–

413.

Collett RW, Edwards JE. Persistent truncus arteriosus: a classification according to

anatomic types. Surg Clin North Am 1949;29:1245–1270.