What is the treatment approach for a tortuous aortic arch?

Treatment Approach for Tortuous Aortic Arch

The treatment approach for tortuous aortic arch should be guided by the presence of symptoms, aortic diameter measurements, and associated complications, with surgical intervention recommended for symptomatic patients or when specific size thresholds are reached. 1

Initial Evaluation

• Dedicated thoracic aortic imaging with CT or MRI is essential for all patients with tortuous aortic arch to assess anatomy and identify any associated aneurysms 1
• Key measurements include the aortic diameter at multiple levels and assessment of any associated diverticulum 1
• Evaluate for symptoms that may be related to the tortuous arch, including:
  • Hoarseness (from recurrent laryngeal nerve involvement) 1
  • Chest pain, pressure, fatigue, or neck/jaw/back pain 1
  • Dysphagia or dyspnea from compression of adjacent structures 2

Management Algorithm Based on Symptoms and Size

Asymptomatic Patients

• For isolated tortuous aortic arch with normal diameter (<4.0 cm):

  • Regular imaging surveillance with CT or MRI at 12-month intervals 2
  • Optimal cardiovascular risk management and medical treatment to reduce major adverse cardiovascular events 2

• For tortuous aortic arch with aneurysm 4.0-5.4 cm:

  • More frequent imaging surveillance with CT or MRI at 6-month intervals 2
  • Consider beta-blockers for blood pressure control and to reduce shear stress on the aortic wall 2

• For tortuous aortic arch with aneurysm ≥5.5 cm in low-risk surgical candidates:

  • Surgical intervention is recommended 2
  • Growth rate ≥0.5 cm/year is also an indication for intervention even if below size threshold 2

Symptomatic Patients

• Open surgical intervention is recommended for patients with symptoms attributable to the tortuous aortic arch who are at low or intermediate operative risk 2
• Symptoms warranting intervention include:
  • Recurrent chest or back pain not attributable to other causes 2
  • Compression symptoms affecting adjacent structures 2
  • Signs of impending rupture 3

Surgical Approaches

• For isolated tortuous aortic arch requiring intervention:

  • Open surgical replacement of the affected segment is the standard approach 4
  • Requires cardiopulmonary bypass with some degree of hypothermia for brain protection 2

• For tortuous arch with aneurysmal disease extending to adjacent segments:

  • Hemiarch replacement when disease extends from the ascending aorta 2
  • Consider elephant trunk or frozen elephant trunk procedure if disease extends into the proximal descending thoracic aorta 2

• For high-risk surgical patients:

  • Branched thoracic endovascular aortic repair (b-TEVAR) may be considered in specialized centers 5
  • Extra-anatomic bypass (e.g., ascending-to-infrarenal abdominal aorta bypass) may be an alternative in cases of extreme tortuosity unsuitable for endovascular repair 6

Perioperative Considerations

• Brain protection strategies during open arch surgery:

  • Profound hypothermia alone, direct antegrade perfusion of brachiocephalic arteries, or retrograde perfusion using cold oxygenated blood 2
  • Consider axillary or subclavian artery bypass to reduce stroke risk 2

• Postoperative management:

  • Early postoperative imaging (within 1 month) to assess surgical result 2
  • Annual imaging for the first 2 years, then every 5 years if findings remain stable 2

Common Pitfalls and Caveats

• Tortuous aortic arch may be associated with other congenital anomalies or connective tissue disorders that require additional evaluation and management 7
• Endovascular approaches are limited by the complex anatomy of the tortuous arch and may not be suitable for all patients 5
• Surgical risk assessment is critical, as mortality for elective arch surgery has decreased significantly in experienced centers to <1% 2
• Long-term follow-up imaging is essential even after successful repair to monitor for development of new aortic pathology 2