Down's syndrome is the most frequent chromosomic anomaly. Fifty percent are associated with a congenital heart disease. Life expectancy and quality of life are increasing since 15 years with improving surgical techniques.
Patients and methods: This study presents 73 patients with Down's syndrome who underwent a surgical repair of congenital heart disease between 1992 and 2002. Among them, 37 (50.6%) had a complete atrio-ventricular septal defect (AVSD) and 36 an other anomaly.
Results: Five children died in the post operative period: all had an AVSD. Two late deaths occurred due to extra cardiac causes during the follow up. Two children who underwent a repair of AVSD had to be reoperated. The majority of the children are doing well, without any cardiac symptom. Thirty-eight percent of patients with AVSD repair have a middle mitral or tricuspid regurgitation and three had a massive mitral or tricuspid regurgitation. One child presents neurological sequelae related to surgery.
Conclusion: Congenital heart diseases in Down's syndrome can be repaired with a limited risk of death. Final results are good for many children with significant improvement of the quality of life and life expectancy.