Prevalence of Congenital Heart Diseases in children and adolescentes with Down Syndrome followed up in a pediatric cardiology reference service

INTRODUCTION: Down Syndrome (DS) is a genetic syndrome with a high prevalence worldwide and associated with numerous comorbidities, which require greater attention and medical care. Among them, Congenital Heart Defects (CHD) stand out in the population with DS, both for their high prevalence (40-65%) and for their influence on the quality and life expectancy of this population. METHODOLOGY: This is a retrospective cross-sectional observational clinical study which aim to identify the prevalence of different CHD in the DS population attended at a pediatric cardiology outpatient clinic and to analyze possible factors related to prognosis. There were no exclusion criteria, considering that only patients diagnosed with DS with CHD were included. RESULTS: We analyzed 154 patients, 75 males and 79 females, with statistical differences regarding age, weight and length at admission being smaller in females. Weight at the time of surgery was associated with mortality, being lower in patients who died. Total atrioventricular septal defect (AVSD) was the most prevalent CHD and the only congenital heart disease that was statistically associated with mortality. Conclusion: The most frequent CHD was AVSD, according to the main literature data. Total AVSD and surgical weight were associated with mortality.