Aortic treatment in connective tissue disease

Area of Science:

• Cardiovascular Surgery
• Genetics
• Vascular Medicine

Background:

• Connective tissue diseases (CTD) disrupt matrix remodeling, increasing risks of arterial aneurysms, dissections, and ruptures.
• Open surgical repair is the standard for CTD patients but often requires multiple operations.
• Endovascular techniques offer potential benefits, especially in high-risk patients or as a bridge to open repair.

Purpose of the Study:

• To discuss the management of aortic diseases in CTD patients.
• To evaluate the role of open surgery versus emerging endovascular treatments.
• To provide an overview of published series on this topic.

Main Methods:

• Review of current literature on open and endovascular aortic repair in CTD.
• Discussion of staged hybrid procedures combining open and endovascular techniques.
• Analysis of considerations for future interventions, including landing zone creation.

Main Results:

• Endovascular treatment is generally restricted to selected high-surgical-risk CTD patients.
• Staged hybrid procedures aim to reduce morbidity, mortality, and spinal cord ischemia.
• A "graft-to-graft" approach is recommended when feasible, requiring careful surgical planning.

Conclusions:

• CTD-related aortic disease management requires a multidisciplinary approach in high-volume centers.
• Endovascular therapy is a viable option for high-risk CTD patients.
• Staged hybrid procedures represent an evolving strategy to optimize outcomes and avoid re-operations.