VASCULAR RINGS AND SLINGS TYPES HAEMODYNAMICS PRESENTATION AND DIAGNOSIS.pptx

1. VASCULAR RINGS AND SLINGS
TYPES, HAEMODYNAMICS,
PRESENTATION AND DIAGNOSIS.
MODERATOR – PROF. SACHIN TALWAR
DISCUSSANT – PROF. MILIND. P. HOTE

2. VASCULAR RINGS - INTRODUCTION
“ A 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.”
Coined by Robert Gross in his paper in the New England Journal
of Medicine from 1945.

4. CLASSIFICATION OF VASCULAR RINGS
• COMPLETE VASCULAR RING
– Double Aortic Arch
– Right Arch with retroesophageal component:
• Mirror Image branching with retro esophageal ligamentum
arteriosum
• Retroesophageal left subclavian artery with ligamentum
arteriosum
• Retroesophageal left brachiocephalic artery.
– Left Aortic Arch and right Descending Aorta with right
ligamentum arteriosum and or patent ductus arteriosus.
– Cervical Aortic Arch Complex

5. CLASSIFICATION OF VASCULAR RINGS
• INCOMPLETE VASCULAR RING
– Left Aortic Arch and retroesophageal right subclavian
artery.
– Tracheal compression by brachiocephalic or left
common carotid artery
– Ductus Arteriosus sling
– Malrotation of the Heart with patent ductus
arteriosus.

7. EMBRYOLOGY
DAY 18 DAY 22

11. CLINICAL PRESENTATION AND DIAGNOSIS.
• Classical presentation of a child with a symptomatic Vascular Ring :
– Noisy Breathing
– “Seal Bark” Cough.
• Other frequent symptoms :
- Wheezing
- Recurrent Upper Respiratory Tract Infections
- Dyspnea On Exertion.
- Dysphagia
• Less frequent but serious
- Apnoea
- Severe Respiratory Distress requiring intubation and Mechanical Ventilation.
Dysphagia usually in Older children, frequently to Solids> Liquids.
“Child is last to leave the table at dinner time”

12. Backer CL, Mavroudis C, Rigsby CK, Holinger LD (2005) Trends in vascular ring surgery. J Thorac
Cardiovasc Surg 129:1339–1347

13. COMPLETE VASCULAR RINGS :
DOUBLE AORTIC ARCH
• Ascending aorta arises normally, but
as it leaves the pericardium it divides
into two branches, a left and right
aortic arch, that join posteriorly to
form the descending aorta.
– Rt.dominant arch-75%.
– Lt.dominant arch-20%.
– Balanced-5%.
• Associated anomalies: TOF and TGA.

14. 4 VESSELS SIGN

15. RIGHT DOMINANT– 75% LEFT DOMINANT - 20%

19. summary

20. RIGHT ARCH – EDWARDS CLASSIFICATION
TYPE DEFINITION PREVALENCE
I Mirror Image
Aortic Arch
59%
II Aberrant Left
Subclavian
Artery
39.5%
III Isolated Left
Subclavian
Artery
1.5%

21. TYPE I : RIGHT AORTIC ARCH WITH MIRROR IMAGE BRANCHING
– Most common, up to 59% of all right-sided
arches.
– Interruption of the dorsal segment of the left
arch between the left subclavian artery and
descending aorta.
– In this variant, the left brachiocephalic trunk
arises first, followed by the right common
carotid and right subclavian arteries
– Usually associated with
*Tetralogy of Fallot *Truncus arteriosus
*Tricuspid atresia *TGA

22. TYPE II : RIGHT ARCH WITH ABERRANT LEFT SUBCLAVIAN ARTERY
• 39.5% of all right-sided arches.
• Associated with a Kommerell diverticulum
• Occurs from interruption of the dorsal segment of
the left arch between the left common carotid
and left subclavian arteries.
• Rarely symptomatic and is therefore usually
incidental, although it can rarely cause
esophageal and/or tracheal compression.
• Rarely associated with other cardiovascular
abnormalities

23. KOMMERELL’S DIVERTICULUM
• Kommerell's diverticulum - Aneurysmal
dilatation of the descending aorta at the origin
of an aberrant subclavian artery.
• Can be seen in both right- and left-sided aortic
arches.
• First described by Burckhard F Kommerell, a
German Radiologist, in 1936 as a pulsatile
mass posterior to the esophagus causing its
compression as seen on barium swallow.
• Embryologic remnant of the left fourth aortic
arch.
• The major complications of a Kommerell
diverticulum are rupture, dissection or
aneurysm.

26. A significant Kommerell diverticulum is defined as an
aneurysmal bulging of the base of the subclavian artery
which is more than 1½ to 2 times the size of the
subclavian artery.
Can be an independent cause of the compression of the
posterior portion of the esophagus and trachea.
Should there be a significant Kommerell diverticulum,
resection of the diverticulum and transfer of the left
subclavian artery to the left carotid artery is
recommended

27. TYPE III : RIGHT ARCH WITH ISOLATED LEFT SUBCLAVIAN ARTERY
– Rarest type: 0.8%
– Results from interruption of the
left arch at two levels, with one
level between the left common
carotid and left subclavian
arteries and the other level distal
to the attachment of the left
ductus.
– May be associated with
congenital subclavian steal
syndrome and vertebrobasilar
insufficiency

28. RIGHT AORTIC ARCH
• These children present later in life than patients
with double aortic arch.
• Symptoms develop between the ages of 1-6
months.
• Ring is anatomically “looser”, as it is partially
formed by the low pressure pulmonary artery
and the ligamentum which does not have blood
flow.

29. LEFT AORTIC ARCH AND RIGHT DESCENDING AORTA
Vascular rings are likely in this
combination, where in, with a
right sided PDA or ligamentum
arteriosum, a vascular ring is
formed.

30. *Persistence of the Rt. or Lt. 3rd
branchial arch and regression of the 4th
branchial arch.
*Consists of Cervical position of the
apex of the aortic arch.
*Separate origin of the contralateral
CCA.
*Retroesophageal DTA coursing
contralaterally to the arch and
anomalous origin of the subclavian
artery from DTA.
CERVICAL AORTIC ARCH COMPLEX

31. Rare aortic arch anomaly
Characterized by an elongated, high-
lying aortic arch extending at or above
the level of the medial ends of
the clavicles.
• Usually asymptomatic. Symptomatic
patients may present with :
– palpable supraclavicular mass
– dysphagia
– wheezing
– coughing
– hoarseness
– stridor
– choking
– apneic spells
– chest pain
– recurrent pulmonary infections

32. INCOMPLETE VASCULAR RINGS
LEFT AORTIC ARCH AND RETROESOPHAGEAL RT.SCA:
• 0.5% of Incidence.
• Retroesophageal Rt.SCA arising as the 4th branch of
an otherwise normal Aortic arch was once thought
to be a cause of dysphagia lusoria or “difficulty
swallowing due to a trick of nature”.
• Rarely,a Rt.Ligamentum Arteriosum passing from
the retroesophageal Rt. SCA to Rt. PA forms a
vascular ring that is symptomatic.

34. These vessels are drawn taut
across the anterior wall of
the trachea, a potential but
uncommon cause of
respiratory obstruction.
Presumably due to
originating more posteriorly
from the aortic arch than
usual.
TRACHEAL COMPRESSION BY BRACHIOCEPHALIC OR
LT.CCA

35. Ductus Arteriosus Sling:
Binet and colleagues described an infant with
respiratory obstruction in which an anomalous
vessel (presumed to be the ductus arteriosus)
originated from the Rt.PA, crossed to the left
between the esophagus and trachea, and joined
the descending aorta adjacent to the origin of a
retroesophageal Rt.SCA.

39. SEVERE MALROTATION OF HEART WITH PDA:
• Compression of the lower trachea can occur with a normal left arch
when there is severe malrotation of the heart into the right chest in
association with agenesis or hypoplasia of the right lung.
• Scherer and Westcott, in May 1972 described a patient with
dextrocardia and normal lungs in whom the pulmonary trunk lay
anterior to the trachea and somewhat to the right.
• Compression was relieved by dividing the patent ductus arteriosus.

41. CLINICAL FEATURES AND DIAGNOSTIC
CRITERIA
• Symptoms and Signs:
 Relate to the consequences of tracheal and esophageal compression.
 Presentation is usually < 6 m of life and often <1st m.
 Inspiratory stridor , expiratory wheeze and tachypnea at birth.
 Hoarse cry ,Persistent barking cough is frequently present.
 Episodes of apnea, severe cyanosis, and unconsciousness.
 Recurrent respiratory infections
 Difficulty in swallowing liquids, with episodes of choking .
 Dysphagia for solids is common (most severe cases are operated on
before the babies are old enough to be offered solid food).

42.  In adults symptomatic vascular rings
manifesting in adults are rare.
 Two thirds have symptoms :in which 63%
were respiratory and Dysphagia in 33%.
 Vascular rings occurring in adults may mimic
chronic asthma.

43. Licari, A., Manca, E., Rispoli, G. A., Mannarino, S., Pelizzo, G., & Marseglia, G. L.
(2015). Congenital vascular rings: A clinical challenge for the pediatrician. Pediatric
Pulmonology, 50(5), 511–524. doi:10.1002/ppul.23152

44. INVESTIGATIONS
Chest Radiograph
Most common place to start the evaluation.
Establishes location of the arch. (Normal Left,
Right or Indeterminate – likely Double).

46. ARCH LATERALITY

47. Esophagography:
Video esophagography at
the time of
cineangiography is
optimal because it
permits a detailed study
showing the pulsatile
nature of the obstruction
and trachea.
With double aortic arch,
the esophagram shows
left- and right-sided
indentations.

48. • Bronchoscopy: rarely done, Identifies sites of
tracheal compression and shows its pulsatile
nature.
• 2D-Echo:Useful in neonates and infants, and is
critically important for identifying associated
cardiac anomalies.
• CECT with 3D reconstruction provides greater
anatomic details.
• MRI: Diagnostic and delineates severity of
tracheal narrowing.

49. • Aortography: Rarely necessary, performed to
assess associated congenital cardiac anomalies.
• Can establish a complete double aortic arch
and sites of narrowing in arches.
• Cannot distinguish between a double arch with
an atretic segment and a right aortic arch with
a retroesophageal component.

50. • Natural History:
• Account for 1% to 2% of CHD’s.
• Symptoms first appearing after age 6 months are
less severe and rarely progressive.
• Symptoms are of borderline severity, they usually
disappear as the child grows.
• Generally, symptoms are milder and of later
onset, in right aortic arch with retroesophageal
component than in those with double aortic arch.

51. • Indications of Operation:
• All patients with important obstructed airway
symptoms.
• Should not be delayed, because hypoxic and
apneic spells may occur and to prevent further
damage to the trachea and bronchi.
• Not indicated if symptoms are mild or absent.

52. VASCULAR SLING
• Congenital anomaly in which the LPA arises
from the RPA extrapericardially, courses to the
left behind the tracheal bifurcation and in front
of the esophagus to reach the left lung hilum,
and forms a sling around the trachea.
• LPA Slinging around the trachea

54. • The anomalous LPA is frequently slightly
smaller than normal.
• The ligamentum arteriosum passing
backward directly superior to the left main
bronchus and anomalous LPA to join the
descending aorta.
• It may participate with the anomalous LPA in
forming a vascular ring

55. Tracheobronchial and Pulmonary Abnormalities:
 The trachea near the bifurcation is usually
narrowed as a result of posterior compression
by the anomalous LPA.
 This mainly affects the origin of the right main
bronchus and trachea just above the carina.

56. • When the right main bronchus is selectively
narrowed, there is hyperinflation of the right
lung .
• At birth, if obstruction of the right main
bronchus is severe, it may cause retention of
fetal fluid in the right lung.
• Other Cardiovascular Anomalies: Most
common are LSVC, ASD, PDA and VSD.

57. • CLINICAL FEATURES AND DIAGNOSTIC CRITERIA:
 Symptoms: Relate to the consequences of tracheal
and esophageal compression.
• CXR: Gives important clues
 In newborns, initial CXR may show retention of
fetal fluid in the right lung, evidenced by a uniform
opacity without an air bronchogram.
 Once the fetal fluid has been resorbed or
suctioned off, the lung will appear hyperinflated.

58. Retained fetal
lung liquid.
Note the
increased
interstitial
markings and
fluid in the
interlobar
fissure.

59. • Left lung hilum is lower than normal in relation
to position of the pulmonary trunk, and unequal
aeration of the lungs frequently is present.
• There is mediastinal density between the
trachea and esophagus on the lateral view.
• Esophagography: Anterior indentation of the
esophagus in the lateral view just above the
level of the carina.

60. • Noninvasive Imaging:
• Diagnosis can be made by 2D ECHO,CECT or
MRI.
• A tracheogram or a tracheal CT study can be
done to evaluate the additional zones of
narrowing that influence surgical
management.
• Best if done in conjunction with video
esophageal studies.

61. AXIAL
ARTERIAL
PHASE

62. AXIAL
ARTERIAL
PHASE

63. • Bronchoscopy: Required in all patients to
evaluate tracheal abnormalities.
• Cardiac Catheterization and Angiography:
can confirm diagnosis and demonstrating
cardiovascular anomalies.
 LPA is visualized best in a cranially tilted
frontal view.

64. THANK YOU