Total aortic replacement in a patient with mega aorta syndrome--a case report

A 64-year-old woman without manifestations of Marfan syndrome was referred for the close investigation of a pulsatile abdominal mass. Computed tomographic scans and magnetic resonance images revealed aneurysmal dilatation of the entire aorta, including the ascending aorta and extending to the bifurcation of the aorta, as well as tortuousness of the thoracoabdominal and abdominal aorta. Digital subtraction angiography also showed aneurysmal dilatation of the entire aorta and trivial aortic regurgitation. However, aortic annular dilatation was not found by echocardiography or aortography. The entire aorta was replaced in two stages. First, graft replacement of the ascending aorta, except for the sinus segment, and the aortic arch was performed using an elephant trunk technique under hypothermic cardiopulmonary bypass with selective cerebral perfusion. Twelve weeks later, the remaining aorta, including the descending aorta and extending to the common iliac artery on the right side, and to the common femoral artery on the left side, was replaced with a partial cardiopulmonary bypass using femoral artery and vein cannulation. We believe that patients with mega aorta syndrome are best treated by total aortic replacement. The results in the present case indicate that the elephant trunk technique is useful for extensive aortic replacement in stages, and greatly facilitates the second stage operative procedures.