Congenital heart disease, gastrointestinal defect, and low birth weight as the contributing factors for three-year survival rates among Down syndrome children in Indonesia

Background: Down syndrome is the most common congenital chromosomal anomaly and occurs in about 1-10:1.000 live births globally. Various reports stated an increasing survival rate because of advanced medical and surgical care. The highest mortality in Down syndrome children takes place in the first three years of life with its comorbidities being congenital heart disease and gastrointestinal defect. Low birth weight was also more common in Down syndrome children compared to normal children and was one of the contributing factors to higher mortality. This study aims to examine three-year survival rates among children with Down syndrome. Methods: We included all medical records with Down syndrome children in Dr. Sardjito Hospital, Yogyakarta, Indonesia during 2013 to 2016. We excluded all medical records with inadequate data. Three-year survival rates were analyzed using Kaplan-Meier and hazard ratio was analyzed using Cox regression. Results: The 1-year, 2-years and 3-year survival rates in Down syndrome children were 80.1%, 72.4%, and 70.8% respectively. Overall, 45% of births with Down syndrome had congenital heart disease, 11% had a gastrointestinal defect, and 9% had both congenital heart disease and gastrointestinal defect. Three-year survival rates in Down syndrome children with congenital heart disease was 61.4% (78/127) and 81.7% (89/109) (p=0.001). Three-year survival with gastrointestinal defect was 47.8% (22/46) and without was 76.3% (145/190) (p<0.001). Furthermore, three-year survival with low birth-weight was 64.6% (42/65) and without was 71.6% (111/155) (p=0.328). Conclusion: Congenital heart disease and gastrointestinal defect lower the survival rate in Down syndrome children.