Abstract 13178: Bicuspid Aortic Valve and Marfan Syndrome: Two Strikes?
Objective: To determine the morbidity associated with a diagnosis of bicuspid aortic valve (BAV) and Marfan syndrome (MFS) versus MFS alone.
Methods: We performed a retrospective cross-sectional analysis in adults with MFS vs. those with MFS plus BAV within the registry of G enetically T riggered T horacic A ortic Aneurysms and Related C onditions (GenTAC). Cases that met the new Ghent criteria were considered.
Results: In the GenTAC cohort, 651 patients met the new Ghent criteria for MFS and 33 (5%) had a dual diagnosis of BAV-MFS. More MFS men than women had BAV-MFS (23 M 70% vs. 10 W, 30%, p<0.005). Estimated prevalence of BAV in patients with MFS within the cohort was 5%. There were no differences between groups in age, history of hypertension, diabetes, medication use, smoking, or history of aortic dissection. The proportion of the population with history of aortic root replacement was similar between the two groups (Table). BAV-MFS subjects were younger at aortic replacement (24.9 years ± 8.7) compared to MFS only subjects (32.7 years ± 12.9; < 0.03). There were no differences related to type of surgery, number of surgeries, length of hospital stay or complications. There were no differences in quality of life measures. While the number of women was small, there were no outcome measures that differed by sex.
Conclusions: Among patients with MFS within the GenTAC registry, the 5% prevalence of BAV is greater than the ~1% prevalence seen in the general population. Gender distribution of BAV-MFS is similar to BAV, i.e. a marked male predominance. Those with BAV-MFS were younger at aortic replacement than those with MFS alone; however, there were no differences in valve dysfunction, type of aortic surgery, surgical complications or quality of life. Dual diagnosis of BAV-MFS is a marker of early initial aortic surgery without increased perioperative morbidity or mortality.
Table 1: Aortic surgery by type and indication
- © 2013 by American Heart Association, Inc.