Aortic Root Surgery in Marfan Syndrome: Medium-Term Outcome in a Single-Center Experience

Area of Science:

• Cardiovascular Surgery
• Genetics
• Thoracic Surgery

Background:

• Marfan syndrome (MFS) is a genetic disorder affecting connective tissue, notably the aorta.
• Aortic root complications, including aneurysm and dissection, are significant causes of morbidity and mortality in MFS.
• The Ghent criteria provide a standardized diagnostic framework for Marfan syndrome.

Purpose of the Study:

• To analyze surgical outcomes of aortic root procedures in Marfan syndrome patients.
• To evaluate the efficacy and risks of both aortic valve (AV)-replacing and AV-sparing root surgeries.
• To contribute data to the Marfan registry regarding surgical interventions and long-term results.

Main Methods:

• Retrospective analysis of data from 59 Marfan syndrome patients meeting Ghent criteria.
• Inclusion of patients who underwent aortic root surgery between April 2004 and February 2012.
• Application of Kaplan-Meier survival analysis to assess outcomes and freedom from reoperation.

Main Results:

• 59 MFS patients underwent 67 aortic root operations; 36% AV-replacing and 64% AV-sparing.
• No early mortalities were recorded; 5-year survival was 91.2%.
• AV-sparing surgery was associated with a higher incidence of moderate/severe AV regurgitation (21%) and reintervention.

Conclusions:

• Aortic root surgery in MFS patients at experienced centers has low operative morbidity and mortality.
• AV-sparing root surgery may increase the risk of AV regurgitation and subsequent reoperations.
• Close clinical follow-up is crucial for detecting and managing complications, particularly AV regurgitation, after aortic root surgery in MFS.