Abstract 20187: Type a Aortic Dissection in Bicuspid Aortic Valve and Marfan Syndrome Patients: Differences and Similarities
Background: Bicuspid aortic valve (BAV) and Marfan Syndrome (MFS) are associated with deleterious changes in the aortic media that predispose patients to Type A acute aortic dissection (TAAAD). Little comparison has been done regarding how TAAAD manifests itself in these different diseases.
Methods: Type A patients enrolled in the International Registry of Acute Aortic Dissection with recorded history of either condition were compared (N=2320). BAV was noted in 93 patients (4.0%) and MFS in 84 (3.6%).
Results: MFS patients presented younger (36.7±11.7 v 52.7±16.1, p<0.001). BAV patients were more commonly of white race (95.6% v 85.4%, p=0.033). Regarding patient history, BAV was more often associated with hypertension (60.4% v 28.9%, p<0.001), atherosclerosis (24.7% v 2.4%, p<0.001), and aortic valve stenosis or insufficiency (41.6% v 19.3%, p=0.002). Conversely, patients with MFS more frequently demonstrated prior aortic dissection (15.9% v 4.3%, p=0.019), prior aneurysm or dissection surgery (19.0% v 5.5%, p=0.006), and family history of aortic disease (44.1% v 3.2%, p<0.001).
Patients with MFS were more likely to present with pain (96.4% v 86.8%, p=0.030); no other differences in presenting symptoms were noted. Annulus, root, sinotubular junction, arch, and descending aortic diameters were similar between groups. The ascending aorta was larger in BAV patients (median diameter 5.5 cm (4.8-6.4) v 4.8 (4.0-6.0), p=0.008). BAV patients were more likely to have periaortic hematoma at presentation (23.3% v 9.1%, p=0.024). Surgery was performed frequently in both groups (91.4% BAV v 90.5% MFS, p=NS) with BAV patients undergoing more aortic valve replacement (59.7% v 37.7%, p=0.008). In-hospital mortality was similar (16.1% BAV v 13.1% MFS, p=NS), and no other differences were seen in in-hospital complications or 5-year survival estimates. MFS patients were much more likely to require a subsequent aortic intervention on 5-year Kaplan-Meier analysis (0% in BAV v 51.4% MFS; p=0.003).
Conclusions: Differences in patient history and ascending aortic size were noted between groups, while presentation, management and outcomes were similar. Notably, patients with MFS were much more likely to require an aortic operation 5 years post-discharge.
Author Disclosures: M. Di Eusanio: None. H. Siddiqi: None. E. Bossone: None. L. Pape: None. T.G. Gleason: None. K.M. Harris: None. T. Myrmel: None. A.C. Braverman: None. T.M. Sundt: None. D.G. Montgomery: None. E.M. Isselbacher: None. K.A. Eagle: None. C.A. Nienaber: None. A. Evangelista: None. R. Fattori: None.
- © 2014 by American Heart Association, Inc.