[Records of congenital heart disease in infants under one year in information systems on birth, hospitalization and death in Rio de Janeiro State, Brazil, 2006-2010].

Objective: to describe the occurrence of cases of congenital heart disease in infants under one year recorded in health information systems of Rio de Janeiro State, from 2006 to 2010.

Methods: descriptive study with data from the Information System on Live Births (Sinasc), Hospital Information System of the Brazilian National Health System (SIH/SUS) and Mortality Information System (SIM).

Results: a total of 345 records were found on Sinasc, there were 1,089 hospitalized children (SIH/SUS) and 1,121 deaths (SIM) of children under one year of age whose underlying cause was congenital heart disease; the prevalence of congenital heart disease was 3.18/10,000 live births; heart diseases were the main causes of death in the group of congenital malformations, with a mortality coefficient of 1.03/1,000 live births.

Conclusion: there was underreporting of cases of congenital heart disease on Sinasc, demonstrating the difficulty of early diagnosis.