One-year-old boy who previously had modified Van Praagh procedure for interruption of the aortic arch (Celoria-Patton type A) and double inlet left ventricle with ventriculoarterial discordance was admitted for progressive cyanosis. He underwent reconstruction of the left pulmonary artery and additional left common carotid-left pulmonary artery shunt. During this procedure, severe hypotension of the lower extremities and heart failure occurred for unknown cause. He died on the 7 postoperative day for lung bleeding with disseminated intravascular coagulopathy. Autopsy revealed dissection of the pseudointima and obstruction of the prosthetic graft (interposed between the main pulmonary artery and the descending aorta). This complication seems very rare but a great care should be taken when a prosthetic graft is manipulated later.