Decrease in aortic distensibility after an extended aortic reconstruction for Marfan's syndrome as a cause of...

Two cases of Marfan's syndrome underwent a reconstruction of the abdominal aorta and descending thoracic aorta. A replacement of the abdominal aorta with visceral arteries as well as a wrapping of the descending thoracic aorta was performed in case 1 while a reconstruction of the descending thoracic aorta and infra-renal abdominal aorta was done in case 2. After the reconstruction, both cases developed acute aortic dissection DeBakey type I. Another reconstruction of the ascending aorta was then urgently performed. Cardiac catheterization after the second operation in case 1 revealed that the distensibility of the aorta had disappeared at the location of the vascular prosthesis while it had also decreased at the wrapped portion, and the maximum dp/dt of the ascending aorta also increased. Increases in the pulse pressure and pulse rate after the first operation were observed in both cases. These hemodynamic changes, which were produced by a decreased distensibility of the reconstructed aorta, increased the mechanical stress to the native aortic wall, and may have been one of the causes of acute aortic dissection DeBakey type I occurring after reconstruction with a prosthesis.