The Ross procedure, which includes removal of the malformed aortic valve an
d replacement of a pulmonary autograft in the aortic position, has increase
d the number of available treatment options. Recently, dilatation of the au
tograft pulmonary root after the Ross procedure has been reported as a comp
lication. We report a patient with bicuspid aortic valve malformations and
aortic annulus dilatation, who had a saccular-form, true-type, aneurysm in
the pulmonary autograft seven months after the Ross procedure. These change
s have not been described so far as complication. Pathologically, marked mu
coid degeneration was noted in the tunica media of the aneurysm, as well as
in the original aortic root. These findings may suggest similar pathologic
al characteristics between the aorta and pulmonary arteries. Hence, the sur
gical risks accompanying vascular characteristics in patients with congenit
al aortic valve malformations should be considered.