A case of severe cyanosis with hepatic cirrhosis in a five-year-old girl who had undergone corrective surgery of congenital biliary atresia previously was reported. She had severe cyanosis, clubbed fingers and toes, polycytemia and squatting on exercise. Intracardiac shunt was ruled out by two-dimensional echocardiography. Contrast echocardiography. Contrast echocardiogram and lung per fusion scanning indicates the presence of intrapulmonary right-to-left shunt. The cardiac catheterization and pulmonary wedge angiography were done at age of five years, but intracardiac right-to-left shunt and congenital anomarous pulmonary vessels were not demonstrated. From those findings, we conclude that the cause of her cyanosis was intrapulmonary right-to-left shunt with multiple dilatation of pulmonary cappilary. This is a first case of cyanosis due to intrapulmonary right-to-left shunt complicating postoperative hepatic cirrhosis in congenital biliary atresia.
