Two patients with Adams-Oliver syndrome and congenital cardiac malform
ations are described. A literature review revealed a 13.4% occurrence
of congenital cardiac malformations in individuals with Adams-Oliver s
yndrome, suggesting that cardiac anomalies are a frequent manifestatio
n of this syndrome. All patients with Adams-Oliver syndrome (aplasia c
utis congenita and terminal digital anomalies) should be evaluated for
cardiac anomalies.