Marfan Syndrome: a Significant Predictor of Recurrent Aortic Dissection
Key findings
- Marfan syndrome is a highly significant predictor of recurrent aortic dissection (AD)
- Patients with recurrent AD are more likely to have atherosclerosis than patients with initial AD
Approximately 5% of individuals suffering an acute aortic dissection (AD) will experience a recurrent AD, according to the largest systematic study of hospital patients with recurrent AD. More than 20% of individuals who have a recurrent AD are also diagnosed with Marfan syndrome, a rare genetic disease.
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Researchers, including Mark E. Lindsay, MD, PhD, and Eric Isselbacher, MD, identified 204 cases of recurrent AD from 3,828 AD cases in the International Registry of Acute Aortic Dissection. The study was published in the journal Circulation.
The average age of patients with recurrent AD was slightly lower at 59 years compared to 62 years of age for patients with an initial AD.
Despite recurrent AD patients being younger at presentation, they experienced more atherosclerosis than patients with initial AD--32% versus 24%. Also, more recurrent AD patients had a history of cardiovascular surgery, including aortic valve replacement, coronary artery bypass grafting, and aortic aneurysm of dissection repair.
When the researchers compared recurrent AD patients who had a type A dissection versus a type B dissection, they found type A dissection patients were not as likely to undergo surgical aortic repair. However, type B dissection patients were more than twice as likely to undergo surgical aortic repair--28% versus 11%.
The researchers found Marfan syndrome to be a highly significant predictor of recurrent AD, with a hazard ratio of 8.6. The proportion of patients with Marfan syndrome who had a recurrent AD was significantly greater than the proportion of patients with an initial AD --21.5% versus 3.1%. Patients with Marfan syndrome were much younger than patients without, with a mean age of 42 years versus 63 years.
To determine the anatomic details of recurrent AD, the researchers conducted a subanalysis of 63 patients with recurrent AD from Massachusetts General Hospital, Brigham and Women’s Hospital, and the University of Michigan Health System. They found that these patients presented at any age, but younger patients were more likely to have Marfan syndrome.
The researchers recommended that formal cardiovascular genetics consultation and gene testing be considered for any patient presenting with recurrent AD.
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