Abstract 17960: Aortic Root Surgery in Patients With Marfan Syndrome in the Current Era: Analysis of Surgical Technique and Outcome in 59 Patients
Background: There are few data on outcome of aortic root surgery in patients (pts) meeting strict diagnostic criteria for Marfan syndrome (MFS) in the current era.
Methods: Pts with MFS referred for aortic surgery root at our clinic were included. There were 59pts diagnosed with MFS by modified Ghent criteria by genetics (10pts), family history (32pts) and/or diagnostic features of the systemic score.
Results: Between 2004 to 2012, 59 MFS pts (42men;71%) referred for aortic root replacement underwent 68 cardiac operations (7 had 2 and 2 had 3). The primary reason for surgery included aortic root aneurysm in 51pts (aortic root >45mm), aortic regurgitation in 15, acute dissection in 4, and mitral regurgitation due to mitral valve (MV) prolapse in 7. Age at surgery was 36±13 years. Preoperative aortic root was 50±6 mm (23±4 mm/m2 BSA, in 8 pts >28mm/m2). Among 59 initial operations, 20 (34%) had composite replacement of AV and ascending aorta (AVR), and 39 (66%) had AV sparing root replacement (AVS) procedures (37 David type and 2 Yacoub type operations). 5 pts having AVS procedures had simultaneous AV repair. Additional simultaneous procedures included:9 pts (15%) aortic arch repair due to aneurysmal dilatation, in 3 pts MV repair, and 1 pt MV replacement. Early mortality was 0%. At a mean follow-up 4.6±3.2 years, 2 deaths occurred: a 38 year old man died 4.5 years (sudden cardiac death, ejection fraction 49%) and a 42 year old man died with 1.5 years (urosepsis) after surgery. 8pts (14%) required 11 reoperations; 6 of the 8pts had initial AVS procedure (5 David, 1 Yacoub). Among the 11 reoperations, 5 had subsequent AVR (severe AR), CABG (1pt), infected prosthetic AV (1pt) and ascending aortic replacement (1 pt), AV replacement for AR plus MV repair, composite homograft for recurrent graft infection (1pt) and surgery for descending aortic dissection and later for thoracoabdominal aneurysm (1pt).
Conclusion: Prophylactic aortic root replacement in MFS pts with AVS or AVR performed at an experienced center carries excellent survival. Preoperative aortic root size is rarely >28 mm/m2. Reoperations are most often required after initital AVS procedures; AVS procedures can fail and further identification of risk factors are essential for optimal patient selection.
- © 2012 by American Heart Association, Inc.