Marfan syndrome with a giant noncoronary sinus of Valsalva aneurysm.

Abstract

Once presented in 2011 by Dayi SU et al. our case is a 30-year-old woman with Marfan syndrome who had undergone aortic valve replacement due to severe aortic regurgitation secondary to dilatation of the aortic root in 2000 and supracoronary aortic replacement due to Stanford type A dissection in 2002 with subsequent dilatation of sinus of Valsalva. When described by Dayi SU et al. the size of the aneurysm was noted to be 6x6.5x6.5 cm in dimensions and it was formed from noncoronary sinus of Valsalva. At this time conservative followup was found to be the appropriate therapy because high risk of redo surgery. Three years later the same patient was observed in echocardiography laboratory with a rapid progression of the same aneurysm of sinus of Valsalva to 8.3x9.3x8.2 cm in size (Figure 1A). Moderate mitral and tricuspid regurgitation with prolapse of valves and normal function of mechanical aortic valve were observed. Computed tomography angiography also confirmed enlargement of the aneurysm to 91.9x91.8 in size (Figures 1B, C, and D). Herein, we would like to draw attention to the clinical presentation and progression of Marfan syndrome.