The Liver in Congenital Heart Disease: Effects of Infantile Coarctation of the Aorta and the Hypoplastic Left Heart Syndrome in Infancy

To define the occurrence of severe liver injury complicating congestive heart failure in the neonatal period as manifested by centrolobular and midzonal hepatic necrosis, liver sections from 137 cases of congenital heart disease dying before 1 month of age were reviewed. A striking incidence of hepatic necrosis was found in association with infantile coarctation of the aorta and the hypoplastic left heart syndrome. Necrosis was present in 37.6% (27 of 72) of cases of these two hemodynamically related groups of defects but was found in association with only 6.1% (4 of 65) of all other cardiac defects. In 22.6% of patients manifesting necrosis, clinical bleeding occurred which was at least in part related to this hepatic injury. All of those cases in which bleeding occurred were encompassed by the group of infantile coarctation and the hypoplastic left heart syndrome.