Objectives: Pre-emptive strategies to manage the aortic complications of Marfan syndrome have resulted in improved life expectancy yet, secondary to the variation of phenotypic expression, anticipating the risk and nature of future aortic events is challenging. We examine rates of new aortic events and reinterventions in a Marfan cohort following initial aortic presentation.
Methods: Retrospective cohort study of Marfan patients with aortic pathology presenting to our institution 1998-2018. Patients were grouped according to index event: aortic dissection or root aneurysm. Patients with aortic dissection were classified according to Debakey criteria. Incidence of new aortic events and frequency of reintervention were analysed.
Results: One hundred and twenty-six aortic procedures were performed in 74 Marfan patients with a median follow-up of 7 years. Forty-seven patients had an index event of root aneurysm and 27 had aortic dissection. Following operative intervention in the aneurysm group, 7 patients developed Debakey III dissections raising the overall number of patients who developed dissection within this cohort to 34. Reinterventions were more frequent in the dissection group with full replacement of the native aorta in 5 patients.
Conclusions: After operative intervention on the proximal aorta, a proportion will develop distal pathology. A greater focus on factors contributing to future events, such as mapping genotypes to clinical course, may lead the way for targeted operative techniques and surveillance.
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