Introduction

Our aim was to describe the epidemiological profile of patients with congenital heart disease (CHD) followed up in the paediatric cardiology outpatient clinic at a tertiary hospital in the city of Rio de Janeiro (RJ), Brazil.

Material and methods

Data were collected from 4050 medical records from April 1987 to January 2020, and the prevalence of CHD was calculated based on the type and sex in this cohort. The data were expressed as percentages. Patients aged > 18 years at the time of initial diagnosis and those with acquired heart disease were excluded.

Results

Male sex comprised approximately 58% of the total CHD carriers. Among the left-right shunt CHDs, ventricular septal defect was the most prevalent (33%), followed by atrial septal defect (18%), and then patent ductus arteriosus (10.5%). Among the cyanogenic CHDs, tetralogy of Fallot was the most frequent (approximately 5% of all CHDs).

Conclusions

The study showed the prevalence of the main CHD in a tertiary referral hospital. We emphasize the importance of knowledge of the epidemiological profile of CHDs in the training of professionals involved in the diagnosis and appropriate treatment of these anomalies.