What are the treatment guidelines for a patient with a double aortic arch?

Treatment Guidelines for Double Aortic Arch

Surgical division of the non-dominant arch (typically the left) is the definitive treatment for double aortic arch and should be performed in all symptomatic patients, ideally before 12 months of age to prevent irreversible tracheal and esophageal damage. 1, 2

Clinical Presentation and Diagnosis

Respiratory symptoms are the hallmark of double aortic arch and drive the need for intervention:

• Respiratory distress, stridor, and noisy breathing occur in 77-91% of patients 2
• Gastrointestinal symptoms (choking with feeds, dysphagia) present in 40% of cases 2
• Most patients present within the first 5-6 months of life, with the majority requiring surgery before 12 months of age 1, 2

Diagnostic workup should include:

• Barium swallow to demonstrate bilateral esophageal compression 1
• Bronchoscopy to visualize extrinsic tracheal compression 1
• Echocardiography as initial imaging modality 3
• CT angiography with 3D reconstruction for definitive anatomic delineation 3

Surgical Approach

The surgical strategy depends on arch dominance:

• Division of the non-dominant arch (typically the left anterior arch) is performed in the vast majority of cases 1, 2
• The right posterior arch is dominant in 72% of patients 2
• Division of the left arch was performed in 16 of 17 patients in one series 1

Critical technical considerations:

• Kommerell's diverticulum must be resected if present (found in approximately 25% of cases) to prevent recurrent dysphagia from persistent compression 1
• Vascular suspension procedures are frequently necessary (required in 76% of patients in one series) to further release the trachea and esophagus from compression 1
• Complete mobilization of the vascular ring is essential to relieve compression 2

Outcomes and Prognosis

Surgical outcomes are excellent with contemporary techniques:

• Operative mortality is essentially zero in modern series (96% survival at 5 years) 2
• No mortality was reported in a 17-patient series 1
• Reoperation is rarely required (only 1 patient in an 81-patient series) 2
• No late aortic arch obstruction develops after proper repair 2

Common postoperative complications:

• Chylothorax occurs in approximately 9% of cases 2
• Persistent respiratory symptoms are frequent (54% at follow-up) due to pre-existing tracheomalacia or compression-related airway maldevelopment 2
• Tracheal stenosis documented in 14% and tracheomalacia in 7% postoperatively 2

Management of Persistent Symptoms

For severe persistent tracheal obstruction after initial repair:

• Anterior aortopexy is effective for children with severe tracheomalacia or residual tracheal compression causing failure to extubate or severe respiratory distress 4
• This provides a simple, safe treatment option for the minority of patients with severe residual symptoms 4

Critical Pitfalls to Avoid

• Delaying surgery beyond 12 months of age increases the risk of irreversible airway damage from chronic compression 1, 2
• Failing to resect Kommerell's diverticulum when present leads to recurrent dysphagia 1
• Inadequate vascular suspension may result in persistent tracheoesophageal compression despite arch division 1
• Underestimating associated cardiac anomalies (present in 18% of cases) requires comprehensive preoperative cardiac evaluation 2