[Modern aortic surgery in Marfan syndrome--2011]

Area of Science:

• Cardiovascular Surgery
• Genetics
• Connective Tissue Diseases

Background:

• Marfan syndrome is a hereditary connective tissue disorder affecting multiple systems, notably the cardiovascular system.
• Aortic root aneurysms in Marfan syndrome pose a significant risk of acute aortic dissection (AADA) and rupture, limiting life expectancy.
• Accurate diagnosis using the revised Ghent nosology and genetic analysis is crucial due to overlapping symptoms with other syndromes.

Purpose of the Study:

• To evaluate surgical techniques for managing aortic root aneurysms in Marfan syndrome.
• To compare the Bentall operation with the valve-sparing reimplantation technique (David operation).
• To highlight the importance of specialized centers and long-term follow-up for Marfan syndrome patients.

Main Methods:

• Review of surgical outcomes for aortic root and arch replacement in Marfan syndrome.
• Comparison of the Bentall operation (mechanical valve conduit) with the David operation (valve-sparing reimplantation).
• Description of surgical techniques including moderate hypothermic circulatory arrest and antegrade cerebral perfusion for aortic arch replacement.

Main Results:

• The Bentall operation achieves complete resection of pathological aortic tissue but requires lifelong anticoagulation.
• The David operation demonstrates favorable long-term durability of the reconstructed aortic valve, avoiding anticoagulation.
• The David operation is increasingly preferred for aortic root aneurysms, with ongoing technical improvements enhancing longevity.

Conclusions:

• Prophylactic aortic root replacement is essential in Marfan syndrome to prevent catastrophic aortic events.
• The David operation is a viable and often preferred alternative to the Bentall procedure for aortic root aneurysms.
• Specialized Marfan centers and close post-operative surveillance are critical for optimal patient management and early detection of aortic changes.