This document discusses various types of vascular rings that can occur during embryonic development when the aortic arch arteries and branches do not regress properly. It describes double aortic arch as the most common type where both right and left aortic arches persist. Other types include right aortic arch with an aberrant left subclavian artery or mirror-image branching. Clinical presentations involve respiratory and feeding symptoms. Diagnosis involves imaging like CXR, barium swallow, CT/MRI and angiography. Surgical treatment is indicated for symptomatic patients to divide the vascular ring.

1. VASCULAR RINGS
& SLINGS
DR. NGUYEN HOANG LINH CHI
CARDIOVASCULAR DEPARTMENT
VIETNAM NATIONAL HOSPITAL OF PEDIATRICS

2. EMBRYOLOGY
 Develops during 3rd -8th
week of the fetal life
 Ventral aorta partly fuse 
Aortic sac
 Unfused parts remain as Rt
& Lt horns of the sac
 The first arteries to appear in
the embryo  Right & left
primitive aorta
 Continuous with endocardial
tube
 Each one – dorsal & ventral
portion

3. EMBRYOLOGY
 Six pairs of arterial arches appear,
connecting the dorsal & ventral
aorta
 All are not present at the same
time
 Selective regression & persistance
of these arch vessels forms the
major arteries of head, neck &
thorax

4. EMBRYOLOGY
 The 1st, 2nd, 5th arches disappear
- 1st arch : dissappeared, small
portion  maxillary artery
- 2nd arch : dissappeared, remain
portion  hyoid + stapedial
arteries
- 5th arch : never forms/form
incompletetly  regresses

5. EMBRYOLOGY
 3rd arch  Common carotid artery
 4th arch :
- Right : proximal part of RSA
- Left: part of aortic arch between LCA
& LSA
 6th arch  Pulmonary arteries &
Ductus arteriosus
- Right side:
+ Proximal part  proximal segment
of RPA
+ Distal portion  dissapears
- Left side:
+ Proximal part  proximal segment
of LPA
+ Distal part : forms ductus arterious

6. EMBRYOLOGY
 Aortic arch
+ Proximal segment from aortic
sac
+ Middle segment from the Lt 4th
arch arch
+ Distal segment from the Lt
dorsal aortic

7. EMBRYOLOGY
 Brachiocephalic A  Rt horn of
the aortic sac
 Rt subclavian :
+ Rt 4th arch artery
+ Rt dorsal aorta
+ 7th intersegmental A
 Lt subclavian :
7th intersegmental A

8. NORMAL ANATOMY

9. VASCULAR RING
DEFINITION
Vascular ring is a congenital
anomaly in which the aortic
arch and its branches
completely or incompletely
encircle and compress the
trachea or esophagus or both
INCIDENCE:
 Based on surgical case series
 True prevalence : ascertain ( ~ 1-
3% CHD)
- Double aortic arch : most
common
- Rt Aortic arch (RAA) & aberrant
LtSA

10. VASCULAR RING
CLASSIFICATION
 Complete Vascular Ring
- Double aortic ring
- Rt aortic arch (RAA) & retroesophageal component
+ Retroesophageal LSA & ligamentum/ductus arteriosum
+ Mirror-image branching & retroesophageal ligamentum arteriosum
+ Retroesophageal Lt brachiocephalic artery
- Lt aortic arch (LAA) & Rt DAo & Rt PDA/ligamenttum arteriosus
- Cervical aortic arch complex
 Incomplete Vascular Ring
- Lt aortic arch & Retroesophageal RSA
- Tracheal compression brachiocephalic or Lt common carotid artery
- Ductus arteriosus sling
- Malrotation of heart & PDA
 Pulmonary artery Sling

11. VASCULAR RING
KEY FEATURES
 Arch location
 Branching of aorta
 Compression of airway
 Diverticulum of Kommerel
 dEscending aorta

12. VASCULAR RING

13. VASCULAR RING
CLASSIFICATION
 Complete Vascular Ring
- Double aortic ring
- Rt aortic arch (RAA) & retroesophageal component
+ Retroesophageal LSA & ligamentum arteriosum
+ Mirror-image branching & retroesophageal ligamentum arteriosum
+ Retroesophageal Lt brachiocephalic artery
- Lt aortic arch (LAA) & Rt DAo & Rt PDA/ligamenttum arteriosus
- Cervical aortic arch complex
 Incomplete Vascular Ring
- Lt aortic arch & Retroesophageal RSA
- Tracheal compression b brachiocephalic or Lt common carotid
artery
- Ductus arteriosus sling
- Malrotation of heart & PDA
 Pulmonary artery Sling

14. DOUBLE AORTIC ARCH
•Most common vascular ring (40%)
• Both of embryonic right and left arches persist
•Ascending aorta arises normally  leaves the
pericardium  divides 2 branches (Lt & Rt arch)
 join posteriorly to Dao
• Lt arch :
-pass anterior, left of trachea  join by ductus
/ligamentum arteriosus
- gives 2 vessels: LCA , LSA
• Rt arch:
-pass posterior, right of esophageus  join
-left-side Dao
-Gives 2 vessels : RCA, RSA
• Right arch dominant is most common (75%) ,
•Left arch dominant (25%), 2 arch is equal (5%)
•Associated anomalies : uncommon (TOF,

15. DOUBLE AORTIC ARCH
CLINICAL PRESENTATIONS
RESPIRATORY SYMPTOMPSs FEEDING SYMPTOMS
- Inspiratory stridor ( onset <3 moth)
- “ Noisy breathing ” or wheezing
-Chronic cough
-Recurrent respiratory infections
-Hoarse cry
-ALTE/apnea
-Gagging or choking
-Recurrent emesis
-Dysphagia ( solid foods, in older
children )
-Failure to thrive
PHYSICAL EXAM
-Often normal
-Poor weight gain ( if severe compression)
-Pulmonary exam : wheezing, stridor, dyspnea, retraction

16. DOUBLE AORTIC ARCH
DIAGNOSIS
 Chest X-ray
 Barium constrat esophagrams
 Bronchography
 Echocardiography
 Magnetic resonance imaging (MRI)
 Computed tomography
 Angiography

17. Chest X-ray

18. Barium constrat esophagrams
Figure 51-7 , p1843,Kirklin, Barratt-Boyes-Cardiac Surgery 2013

19. Echocardiography

20. Computed tomography

21. Magnetic resonance imaging (MRI)

22. Angiography

23. Bronchography
STAGING
-Percentage is evaluated by using
ETT of different sizes the largest
ETT that can be place with 20cm
pressure is evaluated against a scale
developed by Myers and Cotton:
+ Grade 1: Obstruction of 0-50% of
the lumen
+ Grade 2: Obstruction of 51-70% of
the lumen
+ Grade 3: Obstruction of 71-99% of
the lumen
+ Grade 4: Obstruction of 100% ( no
visible lumen)

24. DOUBLE AORTIC ARCH
Figure 51-9 , p1846,Kirklin, Barratt-Boyes-Cardiac Surgery 2013
SURGICAL TREATMENT

25. VASCULAR RING
CLASSIFICATION
 Complete Vascular Ring
- Double aortic ring
- Rt aortic arch (RAA) & retroesophageal component :
+ Retroesophageal LSA & ligamentum/ductus arteriosum
+ Mirror-image branching & retroesophageal ligamentum arteriosum
+ Retroesophageal Lt brachiocephalic artery
+ RAA & Retroesophageal LSA
- Lt aortic arch (LAA) & Rt DAo & Rt PDA/ligamenttum arteriosus
- Cervical aortic arch complex
 Incomplete Vascular Ring
- Lt aortic arch & Retroesophageal RSA
- Tracheal compression brachiocephalic or Lt common carotid artery
- Ductus arteriosus sling
- Malrotation of heart & PDA
 Pulmonary artery Sling

26. RAA + ABERRANT LEFT SUBCLAVIAN ARTERY
 Second most common vascular ring
(30%)
 Regression of left aortic arch
segment between LCA & LSCA (4th
arch)  LSCA originates as last
branch from the aortic arch  LSA
pass posterior, left of esophagus.
 Left ductus ligament originates from
bulbous dilation at the base of LSA
(diverticulum of Kommerell) &
attaches to proximal LPA the ring
compresses esophagus + trachea
 DAo can be left/right side
 Usually an isolated anomaly

27. RAA+ ABERRANT LEFT SUBCLAVIAN ARTERY

28. RAA+ ABERRANT LEFT SUBCLAVIAN ARTERY
BARIUM ESOPHAGRAM
Figure 51-7 , p1843,Kirklin, Barratt-Boyes-Cardiac Surgery 2013

29. RAA+ ABERRANT LEFT SUBCLAVIAN ARTERY
 AORTOGRAM:
Figure 51-8 , p1845,Kirklin, Barratt-Boyes-Cardiac Surgery 2013

30. CT/MRI

31.  SURGICAL TREATMENT
RAA+ ABERRANT LEFT SUBCLAVIAN ARTERY
Figure 51-10 , p1847,Kirklin, Barratt-Boyes-Cardiac Surgery 2013

32. VASCULAR RING
CLASSIFICATION
 Complete Vascular Ring
- Double aortic ring
- Rt aortic arch (RAA) & retroesophageal component
+ Retroesophageal LSA & ligamentum arteriosum
+ Mirror-image branching & retroesophageal ligamentum arteriosum
+ Retroesophageal Lt brachiocephalic artery
+ RAA & Retroesophageal LSA
- Lt aortic arch (LAA) & Rt DAo & Rt PDA/ligamenttum arteriosus
- Cervical aortic arch complex
 Incomplete Vascular Ring
- Lt aortic arch & Retroesophageal RSA
- Tracheal compression brachiocephalic or Lt common carotid artery
- Ductus arteriosus sling
- Malrotation of heart & PDA
 Pulmonary artery Sling

33. RAA with Mirror-image Branching &
Retroesophageal Ligamentum Arteriosus
 Regression of left dorsal aorta between the left 7th
intersegmental artery (LSA) & left 6th arch (ductus)

34. VASCULAR RING
CLASSIFICATION
 Complete Vascular Ring
- Double aortic ring
- Rt aortic arch (RAA) & retroesophageal component
+ Retroesophageal LSA & ligamentum arteriosum
+ Mirror-image branching & retroesophageal ligamentum arteriosum
+ Retroesophageal Lt brachiocephalic artery
- Lt aortic arch (LAA) & Rt DAo & Rt PDA/ligamenttum arteriosus
- Cervical aortic arch complex
 Incomplete Vascular Ring
- Lt aortic arch & Retroesophageal RSA
- Tracheal compression brachiocephalic or Lt common carotid artery
- Ductus arteriosus sling
- Malrotation of heart & PDA
 Pulmonary Vascular Sling

35. RAA with RETROESOPHAGEAL LEFT
INNOMINATE ARTERY
Left arch regresses between right arch and LCA

36. ANOMALOUS LEFT PULMONARY ARTERY
“PULMONARY VASCULAR SLING”
• Left PA arises extrapericardially from
posterosuperior wall of RPA & courses
behind the trachea , in front of the
esophagus to enter the hilum of the left
lung + compresses the right main bronchus
• RPA direct continuation of the
pulmonary trunk .
• Left lung hilum is lower than normal in
relation to the pulmonary trunk
•Ductus/ligamentum arteriosus follows a
normal course from pulmonary trunk RPA
to anomalous LPA  join the DAo 
Ring-Sling complex

37. PULMONARY ARTERY SLING
 DIAGNOSIS :
 CXR (AP and lateral)
- Tracheal narowing and/or displacement( lateral)
- Aortic arch location
- Tracheal position : abnormal deviation to the left (due
to the aorta coursin over the right main bronchus OR
Mildline position with double aortic arch
- Atelectasis, hyperinflation, or pneumonia may be present

38. PULMONARY ARTERY SLING
www.uhrad.com/pedsarc/peds056a.jpg
CXR

39. PULMONARY ARTERY SLING
BARIUM ESOPHAGRAM
-Most important and reliabe diagnostic tool
-Posterior (+ anterior) compression of sophagus on lateral

40. PULMONARY ARTERY SLING
 ECHOCARDIOGRAPHY

41. PULMONARY ARTERY SLING
 CT/MRI : identify vascular structures and anatomy of
tracheobronchial

42. PULMONARY ARTERY SLING
ANGIOGRAPHY : considered “gold standard”, but rarely
needed for diagnosis

43. PULMONARY ARTERY SLING
 BRONCHOSCOPY:
- Use as a diagnostic tool is controversial
- Recommended for diagnosis of a vascular sling rule out
concomitant tracheal rings ( and useful for abberant
innominate artery )

44. PULMONARY ARTERY SLING
 TREATMENT
- Asymptomatic patient need no surgical treatment,
even when anomalies are found incidentally
- Medical management is recommended for infants with
mild symptoms
- Respiratory distress, history of recurrent pulmonary
infections, apneic spells, FTT are indications for
surgical intervention

45. PULMONARY ARTERY SLING
p1853,Kirklin, Barratt-Boyes-Cardiac Surgery 2013

46. PULMONARY ARTERY SLING
p1855,Kirklin, Barratt-Boyes-Cardiac Surgery 2013

47. LAA + Retroesophageal RSCA

48. TRACHEAL COMPRESSION BY INNOMINATE
ARTERY

49. TRACHEAL COMPRESSION BY INNOMINATE
ARTERY
Figure 51-12 , p1849,Kirklin, Barratt-Boyes-Cardiac Surgery 2013