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1, 95% CI, 57.9-134.1), patent ductus arteriosus (OR = 56.9, 95% CI, 40.1-80.8), tetralogy of fallot (OR = 42.1, 95% CI, 19.3-92.3), or atrioventricular septal defect (OR = 510.0, 95% CI, 126.7-999. were higher than those of age- and sex-matched controls. The risk of death in patients with DS was significantly higher than that of age- and sex-matched controls [hazard ratio (HR) =41.7, 95% CI 20.0-87.0]. Conclusion In South Korea, the DS population prevalence was 4.03 per 100,000 persons in 2015. The subjects with DS were more likely