What is the approach for a double aortic arch operation?

Double Aortic Arch Operation: Surgical Approach and Management

Surgical division of the smaller, non-dominant arch is the recommended treatment for double aortic arch, which provides excellent outcomes with low mortality and significant symptomatic relief in most patients. 1, 2

Diagnostic Evaluation

Before surgical intervention, comprehensive imaging is essential:

• CT or MRI is the first-line imaging technique for detailed anatomical assessment 3, 4
• Barium esophagography to visualize esophageal compression 2, 5
• Bronchoscopy to evaluate tracheal compression 2, 5
• Echocardiography to identify any associated cardiac anomalies 1

Surgical Approach

Preoperative Planning

• Determine the dominant arch (right-dominant in approximately 70-75% of cases) 2
• Identify the smaller arch for division
• Evaluate for associated cardiac or non-cardiac anomalies (present in up to 18% of cases) 2

Surgical Technique

1. Approach:

   • Left posterolateral thoracotomy is the most common approach (used in 86% of cases) 1
   • Right thoracotomy or median sternotomy may be used in specific anatomical variants 1

2. Procedure:

   • Division of the smaller, non-dominant aortic arch (usually the left arch)
   • Complete division of any ligamentous connections
   • Preservation of recurrent laryngeal nerve
   • Release of all adhesions causing tracheoesophageal compression

3. Special Considerations:

   • When the aortic arch extends into the proximal descending thoracic aorta, an elephant trunk or frozen elephant trunk procedure should be considered 3
   • For complex cases with atretic segments, careful identification of all vascular structures is critical 6

Postoperative Management

Immediate Postoperative Care

• Monitor for potential complications:
  • Chylothorax (occurs in 9-13.8% of cases) 1, 2
  • Respiratory complications
  • Recurrent laryngeal nerve injury

Follow-up Protocol

• Early postoperative imaging (CT or MRI) within 1 month 3
• Annual imaging for the first 2 years, then every 5 years if findings are stable 3
• Regular assessment for persistent respiratory symptoms (present in up to 54% of patients) 2

Outcomes and Prognosis

• Mortality: Surgical mortality is very low (0-2.5%) 1, 2
• Symptom Resolution: Complete improvement in 75-80% of patients, partial improvement in most others 1
• Long-term Complications:
  • Persistent respiratory symptoms (54%) 2
  • Tracheal stenosis (14%) 2
  • Tracheomalacia (7%) 2
  • Persistent gastrointestinal symptoms (6%) 2

Special Considerations

• Timing of Surgery: Early intervention is recommended once diagnosis is confirmed, even in oligosymptomatic patients, to prevent complications 5
• High-Risk Groups: Patients with tracheomalacia or associated asthma may have persistent symptoms and require additional interventions 1
• Adult Patients: Even adults with longstanding symptoms should be considered for surgical repair, as resolution of symptoms can still be achieved 5

Common Pitfalls to Avoid

1. Delayed Diagnosis: Despite typical symptoms in early childhood, diagnosis may be missed or delayed 5
2. Incomplete Division: Failure to completely divide all fibrous connections can result in persistent symptoms
3. Injury to Adjacent Structures: Careful dissection is needed to avoid damage to the recurrent laryngeal nerve, esophagus, and trachea
4. Inadequate Follow-up: Long-term surveillance is important as tracheomalacia and other airway issues may persist despite successful vascular repair

Double aortic arch repair is a well-established procedure with excellent outcomes when performed by experienced surgeons. The key to success lies in proper preoperative imaging, meticulous surgical technique, and appropriate long-term follow-up.