Skip to main content Skip to main navigation menu Skip to site footer

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

  • Tiona Romauli Simamora ,
  • Suryono Yudha Patria ,
  • Setya Wandita ,


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.



  1. Al-Biltagi M. Down syndrome from Epidemiologic Point of View. Ecronicon Pediatr. 2015;2(1):82–91.
  2. Glasson EJ, Sullivan SG, Hussain R, Petterson BA, Montgomery PD, Bittles AH. The changing survival profile of people with Down’s syndrome: Implications for genetic counselling. Clin Genet. 2002;62(5):390–3.
  3. O’Leary L, Hughes-McCormack L, Dunn K, Cooper SA. Early death and causes of death of people with Down syndrome: A systematic review. J Appl Res Intellect Disabil. 2018;31(5):687–708.
  4. Bull MJ. Health supervision for children with Down syndrome. Pediatrics. 2011 Aug;128(2):393–406.
  5. Leonard S, Bower C, Petterson B, Leonard H. Survival of infants born with Down’s syndrome: 1980-96. Paediatr Perinat Epidemiol. 2000;14(2):163–71.
  6. Glasson EJ, Jacques A, Wong K, Bourke J, Leonard H. Improved Survival in Down Syndrome over the Last 60 Years and the Impact of Perinatal Factors in Recent Decades. J Pediatr. 2016;169:214-20.e1.
  7. Irving CA, Chaudhari MP. Cardiovascular abnormalities in Down’s syndrome: Spectrum, management and survival over 22 years. Arch Dis Child. 2012;97(4):326–30.
  8. Morris JK, Garne E, Wellesley D, Addor MC, Arriola L, Barisic I, et al. Major congenital anomalies in babies born with Down syndrome: A EUROCAT population-based registry study. Am J Med Genet Part A. 2014;164(12):2979–86.
  9. Stoll C, Dott B, Alembik Y, Roth MP. Associated congenital anomalies among cases with Down syndrome. Eur J Med Genet. 2015;58(12):674–80.
  10. Cleves MA, Hobbs CA, Cleves PA, Tilford JM, Bird TM, Robbins JM. Congenital defects among liveborn infants with Down syndrome. Birth Defects Res Part A - Clin Mol Teratol. 2007;79(9):657–63.
  11. Brodwall K, Greve G, Leirgul E, Klungsøyr K, Holmstrøm H, Vollset SE, et al. The five-year survival of children with Down syndrome in Norway 1994-2009 differed by associated congenital heart defect and extracardiac malformations. Acta Paediatr. 2018;107(5):845–53.
  12. Dimopoulos K, Kempny A. Patients with down syndrome and congenital heart disease: Survival is improving, but challenges remain. Heart. 2016;102(19):1515–7.
  13. Clark TG, Bradburn MJ, Love SB, Altman DG. Survival Analysis Part I: Basic concepts and first analyses. Br J Cancer. 2003;89:232–8.
  14. Makhov AS, Madvedev IN. Functional characteristics of children with Down syndrome and possibilities of their correction with the help of athletic activity in Russia. Bali Medical Journal. 2019;8(2): 587-591.

How to Cite

Tiona Romauli Simamora, Suryono Yudha Patria, & Setya Wandita. (2022). Congenital heart disease, gastrointestinal defect, and low birth weight as the contributing factors for three-year survival rates among Down syndrome children in Indonesia. Indonesia Journal of Biomedical Science, 16(2), 65–69.




Search Panel

Tiona Romauli Simamora
Google Scholar
IJBS Journal

Suryono Yudha Patria
Google Scholar
IJBS Journal

Setya Wandita
Google Scholar
IJBS Journal