Abstract 11459: Is the Outcome of Surgery for Aortic Dissection Worse in Patients With Cystic Medial Necrosis Combined With Genetically Diagnosed Connective Tissue Disease?
Introduction: A retrospective analysis was performed to determine the impact of genetically diagnosed connective tissue disease (CTD) on the early and late outcomes of surgical treatment for aortic dissection in patients having aortic disease associated with cystic medial necrosis (CMN).
Patients and Methods: Between 2003 and 2013, 298 patients (56% male, 64.0±15.7 years) underwent initial surgical treatments for type A aortic dissection in 179 or type B in 119. In the surgical spacemen, CMN was present in 141 patients (47.3%). Of them, 43 patients (53% male, 37±12.8 years) undertook genetic examinations, which showed positive in 30 patients (CTD); FBN1 in 19, TGFBR2 in 4, ACTA2 in 3, MYH11 in 1, SMAD3 in 1, TGFB2 in 1, and COL1A1 in 1. In the other 13 patients (non-CTD), they showed negative. The aortic dissection was type A in 13 (43%, acute 7, chronic 4) and type B in 17 (1, 16) in CTD, while it was type A in 10 (77%, acute 9, chronic 1) and type B in 3 (0, 3) in non-CTD. The initial surgeries were for Ao. arch in 14 including 9 with simultaneous root repair, descending Ao. in 9, thoracoabdominal Ao. in 3, and abdominal Ao. in 4 in CTD, while they were for arch in 10 including 1 with root repair, descending Ao. in 1, and thoracoabdominal Ao. in 2 in non-CTD. The early and late outcomes of initial aortic surgery were compared between the CTD and non-CTD patient-groups. The follow-up rate was 100% for 57.1 ± 43.0 months.
Results: There were no early and late deaths. New aortic dissection on the other aortic sites occurred at 13% only in CTD. Furthermore, the freedom from reoperation rate at 5/10 years was significant lower 20/27% in CTD, compared with 54/69% in non-CTD (P= 0.03/P= 0.02).
Conclusions: In patients with CMN combined with genetically diagnosed CTD, new aortic dissection often occurred and the freedom from aortic reoperation was significantly worse. Closer follow-up and proper surgical treatments with adequate timing are mandatory for such a high-risk patient-group.
Author Disclosures: T. Fujiyoshi: None. K. Minatoya: None. Y. Ikeda: None. H. Ishibashi-Ueda: None. T. Morisaki: None. H. Morisaki: None. H. Sasaki: None. H. Tanaka: None. T. Oda: None. M. Seike: None. H. Ogino: None.
- © 2014 by American Heart Association, Inc.