Surgical Outcome of Abdominal Aortic Aneurysm Replacement in Patients with Connective Tissue Disorders under 30 Years of Age.

Objectives

Abdominal aortic aneurysm (AAA) in patients <30 years old is relatively rare. We retrospectively analyzed patients <30 years who received an AAA replacement. Materials: Among 3,003 patients who received an AAA replacement during the last 40 years, 10 patients <30 years old were retrospectively reviewed. All patients suffered from a connective tissue disease: eight from Marfan syndrome and two from Loeys-Dietz syndrome. Five patients had a history of cardiovascular surgery. Aortic pathologies were a dissection type in eight patients and a non-dissection type in two. All patients received a graft replacement of infrarenal AAA, with a bifurcated graft in six patients and a straight graft in four.

Results

Except for cases that were urgent and emergent, rapid aneurysm expansion was noted in all cases. Mean AAA diameter at surgery was 46.7±9.2 mm. No hospital mortality was recorded. Eight patients required 10 additional cardiovascular surgeries: two root replacements, two total arch replacements, two descending aortic replacements, and four thoracoabdominal replacements.

Conclusion

AAA replacement in patients <30 years is safe. In younger patients with a connective tissue disease, AAA should be included in the routine medical check-up, and earlier surgical indication should be considered for its rapid expansion.