Cystic fibrosis manifested as focal biliary cirrhosis in a newborn infant with congenital heart disease

A 3 day old male infant, meconium stained at birth, presented with respiratory distress. Hypoplastic left heart syndrome was diagnosed clinically and documented at autopsy. Unsuspected cystic fibrosis was demonstrated by the histologic presence of severe focal biliary cirrhosis with spreading fibrosis in the liver; minimal lesions in the pancreas and intestines were compatible with a diagnosis of cystic fibrosis. The abundant hepatic fibrosis is unusual in an infant of this age indicating its in utero origin. This case is also unusual because of the fortuitous association of cystic fibrosis and a congenital cardiac malformation.