1. Vascular Rings & Sling Seoul National University Hospital
Department of Thoracic & Cardiovascular Surgery

2. Vascular Rings Introduction 1. Definition 2. History
Anomalies of the great arteries that compress the trachea
and/or esophagus
2. History
Hommel : Description double aortic arch in 1737
Wolman : Compression syndrome by double arch in 1939
Gross : 1st. surgical correction of double aortic arch in 1945
* Frequent vascular ring
Double aortic arch
Right aortic arch with retroesophageal Lt. subclavian artery
& ligamentum arteriosum
Right aortic arch with retroesophageal ligamentum arteriosum

3. Edwards’ Hypothetic Double Aortic Arch
Vascular Rings
Classification
Edwards’ Hypothetic Double Aortic Arch

4. Anomalies of the Aorta Position, course, composition
A. Double aortic arch
B. Double lumen aortic arch
C. With Lt. aortic arch & Lt. upper desc. aorta
1. Aberrant right subclavian artery
2. Other aberrant brachiocephalic arteries
a. Aberrant brachiocephalic arteries without compression of trachea
b. Aberrant innominate or left common carotid arteries causing
compression of the trachea
3. Ductus arteriosus sling
4. Subclavian steal
D. Circumflex retroesophageal component
E. Right aortic arch
1. Without retroesophageal component
2. With retroesophageal component

5. Pulmonary Artery System
Congenital anomalies
Anomalous origin of left pulmonary
artery (“vascular sling”)
B. Unilateral absence of pulmonary artery
C. Origin of one pulmonary artery from the
ascending aorta

6. Abnormalities of the Aorta
Length, size, or continuity
A. Cervical aorta
B. Hypoplasia of the aorta
C. Pseudocoarctation of the aorta
D. Complete interruption of aortic arch

7. Embryonic development
Vascular Rings
Embryonic development

8. Vascular Rings & Slings
Pathophysiology
Maldevelopment of the embryonic aortic arches produces vascular rings/sling that may partially or completely encircle the trachea and/or esophagus, producing compressive symptoms.
Tracheoesophageal compressive symptoms include respiratory distress, recurrent pulmonary infections, and dysphagia.

9. Vascular Rings & Slings
Clinical feature
1. Symptoms and Signs
* Presentation within 1st 6months of life
* Expiratory wheeze, inspiratory stridor
* Tachypnea, respiratory infection
2. Laboratory Findings
* X-Ray : tracheal deviation, compression
* Esophagogram : most useful, indentation
* Bronchogram : rarely done
* Aortography
3. Natural History
* Incidence : 1~2% of CHD
* Symptom before 6 months : severe, and fatal before 1 year
* Symptom after 6 months : less & disappear or become mild

10. Double Aortic Arch Morphology
The left arch is often narrower than the right.
Uncommonly right arch may be smaller in its distal part, but it is rarely atretic.
Associated cardiovascular anomalies are not common,but include TOF & TGA.
Vascular ring is present.

11. Double Aortic Arch
Morphology

12. Right Aortic Arch
Types

13. Double Aortic Arch
Pathology
Pathology

14. Right Aortic Arch Morphology
1. Without retroesophageal component (Type I)
* There is not a vascular ring
* Common in TOF & truncus arteriosus
2. With retroesophageal component
1) Mirror image branching & restroesophageal LA (Type II)
* vascular ring is present, but rare
2) Restroesophageal anomalous Lt. subclavian artery & LA (Type III)
* common type of vascular ring
* loose, compression is uncommon
* associated anomalies are rare
3) Retroesphageal anomalous Lt innominate artery (Type IV)

15. Branching configuration
Right Aortic Arch
Branching configuration
A, Paired dorsal aorta showing the position of the seventh intersegmental artery before cephalad migration. B, Cephalad migration with regression of the left dorsal aorta. C, Incomplete cephalad migration resulting in LSA arising from ascending aorta.

16. Left Aortic Arch Morphology 1. Anomalous Rt. subclavian artery
* Dysphagia lusorium
* Rarely Rt. ligamentum arteriosum (vascular ring)
2. Lt. aortic arch and Rt. descending aorta
* Combination with Rt PDA or DA, vascular ring is present
* Associated CHD is more common
3. Ductus arteriosus sling
* Anomalous vessel from Rt. pulmonary artery origin crossed to
Lt between esophagus & trachea to join descending aorta
4. Abnormal course of innominate or Lt. carotid artery
5. Severe malrotation of the heart

17. Left Aortic Arch
Aberrant RSCA

18. Left ligamentum arteriosum
Right Aortic Arch
Left ligamentum arteriosum

19. Left ligamentum arteriosum
Right Aortic Arch
Left ligamentum arteriosum

20. Tracheal compression by innominate artery
Vascular Compression
Tracheal compression by innominate artery
Vascular Compression

21. Left Aortic Arch
Retroesophageal RSCA

22. Vascular Rings Operative treatment
1. Indication : patients with significant obstructive symptoms
2. Technique of operation
1) Double aortic arch : Division at the junction (Lt approach)
2) Right aortic arch : Dissection complete and division of
ligamentum arteriosum (Lt approach)
3) Left aortic arch : Dissection complete and division right
ligamentum arteriosum (Rt approach)
3. Result
* Low hospital mortality
* Late survival and relief of symptoms are both excellent
unless other associated anomalies
* Rarely aneurysm formation

23. Kommerell's Diverticulum
Presentation
Approximately 40% of RAA cases are associated with an aberrant subclavian artery originating from a diverticulum, so-called Kommerell's diverticulum, which is formed by a remnant of the left dorsal aorta
Most patients with RAA are asymptomatic, however, RAA and aortic diverticulum can be confused with acquired aortic diseases, leading to unnecessary surgical intervention
Although rare, atherosclerotic changes of ASA and KA can lead to dissection or aneurysmal dilatation

24. Kommerell's Diverticulum
Characteristics
A rare condition that occurs in association with a left aortic arch with aberrant right subclavian artery or a right aortic arch with aberrant left subclavian artery
Burckhard F. Kommerell reported an aortic diverticulum in a patient who had LAA and ARSA in 1936
The aneurysmal diverticulum of the descending aorta at the origin of ARSA is called Kommerell's diverticulum
Anatomically, in a patient with RAA and ALSA, the left common carotid artery arises from the anterior of the ascending aorta, followed by the right common carotid artery, the right subclavian artery, and the ALSA that arises from Kommerell's diverticulum

25. Kommerell's Diverticulum
CT angiography
Computed tomographic angiography viewed from back shows Kommerell diverticulum (arrow) and dilatation of presumed remnant of origin of left subclavian artery (arrowhead).

26. Kommerell's Diverticulum
Surgical indications
Indications were that the diameter of Kommerell's diverticulum was more than 50 mm in patients who had symptoms associated with Kommerell's diverticulum
Surgical techniques
Total arch replacement
Replacement of the descending aorta with aberrant subclavian artery–carotid artery transposition
Open stent grafting with the extra-anatomic bypass
Ligation of the aberrant subclavian artery, and reconstruction of the descending aorta

27. Vascular Sling Introduction Definition History
Left pulmonary artery arises anomalously from the right pulmonary artery extrapericardially, form a sling around the trachea.
History
Glaevecke & Doehle : 1stly recognized in 1897
Contro : Term vascular sling in 1958
Potts : 1st successful operation in 1954

28. Vascular Slings Morphology
1. Left pulmonary artery is slightly smaller than the right.
2. Rarely right upper lobe artery may come off left pulmonary
artery near its origin.
3. Tracheobronchial and pulmonary abnormalities
* Trachea near bifurcation is usually narrowed.
* 50% show narrowing of trachea secondary
to complete cartilage ring.
* Hypoplasia, hyperinflation, atelectasis
4. Other cardiovascular anomalies
* 50% of cases are associated, left SVC, ASD, PDA, VSD
5. Noncardiovascular anomalies
* relatively frequent, a wide variety

29. Pulmonary Artery Sling
Morphology

30. Pulmonary Artery Sling
Clinical features
1. Symptoms and signs
* 90% have significant symptom, present at birth or soon after.
* Wheezing, stridor, choking, tachypnea, cyanosis
2. Laboratory findings
* X-Ray : bowing and deviation of airway
* Esophagogram : anterior indentation in lateral view
* Tracheogram : used with or without bronchoscopy
3. Natural History
* Significant obstruction : most die before 1 year of age
* Late presentation : survival is possible without surgery,
obstruction become less with growth

31. Pulmonary Artery Sling
Operative treatment
1. Indication
* If there are symptoms & signs of respiratory obstruction.
* When symptoms are mild, surgery is not recommended
2. Technique
* Left posterolateral thoracotomy without CPB
* Median sternotomy with CPB
3. Result
* Survival : 50~60%
* Relief of obstruction : prognosis is poor with diffuse
anatomic tracheal stenosis

32. Pulmonary Artery Sling
Operation