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

A 3-day-old 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. 