What is the treatment for extensive aortic vascular involvement, particularly in the aortic arch and descending aorta, with significant dilatation?

Treatment for Extensive Aortic Vascular Disease Involving the Aortic Arch and Descending Aorta

Open surgical repair is recommended for patients with extensive aortic vascular disease involving the aortic arch and descending aorta, with a two-stage surgical approach being the optimal treatment strategy for this complex presentation. 1

Assessment of Current Aortic Dimensions

The patient's measurements show:

• Sinus of Valsalva: 3.7 cm (normal)
• Ascending aorta: 3.8 x 4 cm (borderline dilated)
• Aortic arch: 3 cm (normal)
• Descending aorta: 2.9 x 2.8 cm (normal)

While these measurements do not meet the traditional size criteria for intervention (typically ≥5.5 cm for the aortic arch), the mention of "extensive aortic vascular" involvement suggests pathology beyond simple dilatation, possibly including dissection or other structural abnormalities.

Treatment Algorithm

1. Immediate Management

• Complete imaging workup with CT angiography or MRI of the entire aorta if not already done
• Aggressive blood pressure control with beta-blockers as first-line therapy 1
• Transfer to a high-volume aortic center with a multidisciplinary aortic team 1

2. Definitive Surgical Management

For extensive disease involving both the arch and descending aorta, a two-stage approach is recommended:

First Stage:

• Open surgical repair via median sternotomy to address the aortic arch
• Options include:
  • Hemiarch replacement if disease is limited to proximal arch 1
  • Total arch replacement for more extensive arch involvement 1
  • Elephant trunk or frozen elephant trunk procedure to facilitate the second-stage repair 1

Second Stage:

• Endovascular repair (TEVAR) of the descending thoracic aorta using the elephant trunk as a landing zone 1, 2
• Alternatively, open repair via left thoracotomy if endovascular approach is not feasible 2

This staged approach has demonstrated acceptable morbidity and mortality rates in patients with extensive aortic disease 2.

Special Considerations

Endovascular Options

• For high-risk surgical patients, a hybrid approach combining surgical debranching of arch vessels followed by endovascular stenting may be considered 1
• Novel techniques using personalized aortic arch stent grafts may allow a complete endovascular approach in select cases 1

Genetic Considerations

• If genetic aortic disease is suspected (e.g., Marfan syndrome), open surgical repair is preferred over endovascular techniques 1, 3
• Lower threshold for intervention (≥5.0 cm) should be considered in patients with genetic aortic disorders 1

Postoperative Management

• Lifelong imaging surveillance of the entire aorta
• Aggressive blood pressure control with target systolic BP <120 mmHg 3
• Continuation of beta-blockers and consideration of ARBs (e.g., losartan) 3

Common Pitfalls to Avoid

• Delaying referral to a specialized aortic center
• Focusing only on the most dilated segments while neglecting the entire aortic pathology
• Inadequate blood pressure control during perioperative period
• Failure to recognize that even after successful repair, patients remain at risk for complications in the residual aorta

The complexity of extensive aortic disease involving both the arch and descending aorta necessitates a carefully planned surgical approach by an experienced aortic team to optimize outcomes and reduce mortality risk.