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Vascular Rings & Slings

Learning Objectives

Core Knowledge & Clinical Reasoning

  • [ ] Recognize clinical presentation of vascular rings (stridor, dysphagia, respiratory symptoms)
  • [ ] Differentiate complete from incomplete vascular rings
  • [ ] Identify CXR findings suggesting vascular ring (right aortic arch)
  • [ ] Understand anatomy of common vascular ring types

Management Decisions

  • [ ] Determine appropriate imaging workup for suspected vascular ring
  • [ ] Recognize indications for surgical repair
  • [ ] Anticipate post-operative course including tracheomalacia

Communication & Counseling

  • [ ] Explain vascular ring anatomy to families in understandable terms
  • [ ] Counsel on expected surgical outcomes and recovery timeline

Systems-Based Practice

  • [ ] Coordinate multidisciplinary evaluation (cardiology, pulmonology, ENT, surgery)
  • [ ] Ensure appropriate post-operative airway monitoring

Key Concept

Vascular Ring = Vascular structures encircling trachea and/or esophagus

Presents with airway and feeding symptoms from extrinsic compression

Classification

Complete Vascular Rings

Completely encircle trachea and esophagus

Type Anatomy Frequency
Double aortic arch Both left and right arches present Most common complete ring
Right arch + aberrant left subclavian Right arch, left subclavian from descending aorta, left ligamentum Second most common

Incomplete Rings (Partial Compression)

Type Anatomy Compression
Innominate artery compression Normal arch, innominate arises leftward Anterior tracheal
Pulmonary artery sling Left PA arises from right PA Right mainstem bronchus
Aberrant right subclavian Left arch, RSCA from descending aorta Posterior esophageal (often asymptomatic)

Double Aortic Arch

Anatomy

  • Both right and left aortic arches present
  • Usually right arch dominant (larger)
  • Arches join posteriorly to form descending aorta
  • Trachea and esophagus encircled

Clinical Presentation

Age Symptoms
Neonate/Infant Stridor (biphasic), feeding difficulties
Older child Dysphagia, recurrent respiratory infections

Classic triad: 1. Stridor (worse when supine, with feeds) 2. Dysphagia 3. Respiratory distress with feeding

Imaging Findings

  • CXR: Right-sided aortic arch, possible tracheal narrowing
  • Barium swallow: Bilateral esophageal indentation (diagnostic clue)
  • CT/MR angiography: Gold standard - shows both arches

Right Aortic Arch with Aberrant Left Subclavian

Anatomy

  • Right aortic arch (crosses right mainstem bronchus)
  • Left subclavian arises from descending aorta (Kommerell diverticulum)
  • Left ligamentum arteriosum completes the ring posteriorly

Key Point

Ring is formed by: Right arch + aberrant LSCA + left ligamentum

Must divide ligamentum to release ring

Associations

  • 22q11 deletion (DiGeorge) - 25% have right arch
  • TOF - ~25% have right arch
  • Truncus arteriosus

Pulmonary Artery Sling

Anatomy

  • Left pulmonary artery arises from RIGHT PA
  • LPA courses between trachea and esophagus
  • Compresses right mainstem bronchus and distal trachea

Associations

  • Complete tracheal rings (50%) - long-segment tracheal stenosis
  • May require tracheal reconstruction (slide tracheoplasty)

Presentation

  • More severe respiratory symptoms than typical vascular ring
  • Wheezing (often misdiagnosed as asthma)
  • Recurrent pneumonia (right-sided)

Imaging

  • CXR: Asymmetric aeration, right lung hyperinflation or atelectasis
  • CT angiography: Anomalous LPA course between trachea and esophagus
  • Bronchoscopy: Pulsatile posterior compression of trachea

Innominate Artery Compression

Anatomy

  • Normal aortic arch anatomy
  • Innominate artery arises more leftward than usual
  • Compresses anterior trachea as it crosses

Presentation

  • Biphasic stridor
  • "Dying spells" - reflex bradycardia with compression
  • Usually mild, may improve with growth

Management

  • Most resolve spontaneously by age 2-3 years
  • Surgery (aortopexy) only if severe symptoms
  • Aortopexy: Suspend ascending aorta/innominate to sternum

Aberrant Right Subclavian Artery (ARSA)

Anatomy

  • Left aortic arch (normal)
  • Right subclavian arises from descending aorta
  • Courses posterior to esophagus (retroesophageal)

Clinical Significance

  • Usually asymptomatic (no complete ring)
  • "Dysphagia lusoria" - rare dysphagia in adults
  • Most common aortic arch anomaly (~0.5-1% of population)
  • Association with Down syndrome (~30%)

When to consider repair

  • Symptomatic dysphagia unresponsive to conservative measures
  • Kommerell diverticulum with aneurysm risk

Diagnostic Approach

Step 1: Clinical Suspicion

  • Stridor (biphasic, worse supine/with feeds)
  • Feeding difficulties, choking
  • Recurrent respiratory symptoms
  • Failure to respond to asthma treatment

Step 2: Chest X-ray

  • Right aortic arch = suspect vascular ring
  • Tracheal deviation or narrowing

Step 3: Barium Swallow (if available)

  • Posterior esophageal indentation (all rings)
  • Bilateral indentation = double arch
  • Anterior indentation = pulmonary sling

Step 4: CT or MR Angiography

  • Gold standard for surgical planning
  • Shows arch anatomy, dominance, ligamentum position
  • Airway assessment (3D reconstruction)

Step 5: Bronchoscopy (optional)

  • Direct visualization of airway compression
  • Pulsatile compression = vascular
  • Assess for tracheomalacia

Surgical Management

Indications

  • Symptomatic vascular ring (stridor, feeding difficulty)
  • Recurrent respiratory infections
  • Failure to thrive
  • Apnea/cyanotic spells

Surgical Approach

Type Procedure
Double aortic arch Divide smaller (usually left) arch + ligamentum
Right arch + aberrant LSCA Divide ligamentum (+ Kommerell if aneurysmal)
Pulmonary artery sling LPA reimplantation to main PA (requires CPB)
Innominate compression Aortopexy

Key Points

  • Most repairs via left thoracotomy or VATS
  • CPB not required for most vascular rings
  • Pulmonary sling often needs sternotomy + CPB

Post-Operative Expectations

Immediate

  • Stridor may persist (tracheomalacia from chronic compression)
  • Gradual improvement over weeks to months

Complications

  • Tracheomalacia - most common persistent issue
  • Recurrent laryngeal nerve injury (hoarseness)
  • Chylothorax (thoracic duct injury)

Long-term

  • Excellent outcomes - most asymptomatic by 6-12 months
  • Stridor typically resolves as trachea remodels
  • No activity restrictions in most patients

Board Pearls

Pearl: Right aortic arch on CXR + stridor = suspect vascular ring

Barium swallow shows posterior esophageal compression

Pearl: Double aortic arch is most common COMPLETE ring

Divide the smaller (usually left) arch + ligamentum

Pearl: Pulmonary artery sling - LPA from RPA

50% have complete tracheal rings requiring slide tracheoplasty

Pearl: Vascular ring repair usually does NOT require bypass

Exception: Pulmonary artery sling requires CPB

Pearl: Tracheomalacia causes persistent post-op stridor

Usually resolves over months as trachea remodels

Self-Assessment

Q1: A 3-month-old presents with biphasic stridor worse with feeds. CXR shows a right-sided aortic arch. What is the most likely diagnosis?

Answer **Answer**: Vascular ring (most likely right aortic arch with aberrant left subclavian OR double aortic arch) **Rationale**: Right aortic arch + stridor/feeding difficulties is classic for vascular ring. The two most common are double aortic arch and right arch with aberrant left subclavian. CT angiography will define the anatomy.

Q2: A 4-month-old with "asthma" unresponsive to bronchodilators has recurrent right lower lobe pneumonia. Echo shows the left pulmonary artery arising from the right PA. What associated finding occurs in 50%?

Answer **Answer**: Complete tracheal rings (long-segment tracheal stenosis) **Rationale**: Pulmonary artery sling (LPA from RPA) is associated with complete tracheal rings in ~50% of cases. These patients may require slide tracheoplasty in addition to LPA reimplantation.

Q3: After successful double aortic arch repair, a patient continues to have stridor at 2 weeks post-op. What is the most likely cause?

Answer **Answer**: Tracheomalacia **Rationale**: Chronic vascular compression causes tracheomalacia (softening of tracheal cartilage). After ring division, the stridor typically persists but gradually improves over weeks to months as the trachea remodels and strengthens.

Key Guidelines

2020 Society of Thoracic Surgeons Expert Consensus Statement on Vascular Rings Ann Thorac Surg. 2020 Surgical indications and techniques for vascular ring repair

References

  • Backer CL, et al. Ann Thorac Surg. 2019;107:296-304
  • STS/EACTS Congenital Heart Surgery Database
  • Defined indications for vascular ring surgery