Association between down syndrome and in-hospital death among children undergoing surgery for congenital heart disease a us population-based study

Circulation: Cardiovascular Quality and Outcomes

Volumn 7, Issue 3, 2014, Pages 445-452

  Evans, Jacqueline M ^a   Dharmar, Madan ^a   Meierhenry, Erin ^b   Marcin, James P ^c   Raff, Gary W ^a  

^a Section of Critical Care Medicine   (United States)

^b UNIVERSITY OF CALIFORNIA   (United States)

^c UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

Congenital; Death; Heart defects; Pediatrics

Indexed keywords

ARTICLE; CHILD; CONGENITAL HEART DISEASE; DOWN SYNDROME; FEMALE; GLENN SHUNT; HEART SURGERY; HEART VENTRICLE SEPTUM DEFECT; HUMAN; MAJOR CLINICAL STUDY; MALE; MORTALITY; PREMATURITY; PRIORITY JOURNAL; RISK ASSESSMENT; SYSTEMIC PULMONARY SHUNT; TREATMENT OUTCOME; ADOLESCENT; COHORT ANALYSIS; COMPLICATION; HEART DEFECTS, CONGENITAL; INFANT; NEWBORN; PRESCHOOL CHILD; RISK; UNITED STATES;

ADOLESCENT; CARDIAC SURGICAL PROCEDURES; CHILD; CHILD, PRESCHOOL; COHORT STUDIES; DOWN SYNDROME; FEMALE; HEART DEFECTS, CONGENITAL; HOSPITAL MORTALITY; HUMANS; INFANT; INFANT, NEWBORN; MALE; RISK; UNITED STATES;

EID: 84903588772     PISSN: 19417713     EISSN: 19417705     Source Type: Journal    
DOI: 10.1161/CIRCOUTCOMES.113.000764     Document Type: Article

References (41)

1. Irving CA, Chaudhari MP. Cardiovascular abnormalities in Down's syndrome: spectrum, management and survival over 22 years. Arch Dis Child. 2012;97:326-330.
2. Weijerman ME, van Furth AM, Vonk Noordegraaf A, van Wouwe JP, Broers CJ, Gemke RJ. Prevalence, neonatal characteristics, and first-year mortality of Down syndrome: a national study. J Pediatr. 2008;152:15-19. (Pubitemid 350265948)
3. Bittles AH, Bower C, Hussain R, Glasson EJ. The four ages of Down syndrome. Eur J Public Health. 2007;17:221-225. (Pubitemid 46536733)
4. Tennant PW, Pearce MS, Bythell M, Rankin J. 20-year survival of children born with congenital anomalies: a population-based study. Lancet. 2010;375:649-656.
5. Loane M, Morris JK, Addor MC, Arriola L, Budd J, Doray B, Garne E, Gatt M, Haeusler M, Khoshnood B, Klungsøyr Melve K, Latos-Bielenska A, McDonnell B, Mullaney C, O'Mahony M, Queisser-Wahrendorf A, Rankin J, Rissmann A, Rounding C, Salvador J, Tucker D, Wellesley D, Yevtushok L, Dolk H. Twenty-year trends in the prevalence of Down syndrome and other trisomies in Europe: impact of maternal age and prenatal screening. Eur J Hum Genet. 2013;21:27-33.
6. Shin M, Besser LM, Kucik JE, Lu C, Siffel C, Correa A; Congenital Anomaly Multistate Prevalence and Survival Collaborative. Prevalence of Down syndrome among children and adolescents in 10 regions of the United States. Pediatrics. 2009;124:1565-1571.
7. Freeman SB, Taft LF, Dooley KJ, Allran K, Sherman SL, Hassold TJ, Khoury MJ, Saker DM. Population-based study of congenital heart defects in Down syndrome. Am J Med Genet. 1998;80:213-217. (Pubitemid 28533530)
8. Wells GL, Barker SE, Finley SC, Colvin EV, Finley WH. Congenital heart disease in infants with Down's syndrome. South Med J. 1994;87:724-727. (Pubitemid 24218226)
9. Marino B. Congenital heart disease in patients with Down's syndrome: anatomic and genetic aspects. Biomed Pharmacother. 1993;47:197-200. (Pubitemid 23295767)
10. de Rubens Figueroa J, del Pozzo Magaña B, Pablos Hach JL, Calderón Jiménez C, Castrejón Urbina R. [Heart malformations in children with Down syndrome]. Rev Esp Cardiol. 2003;56:894-899.
11. Al-Hay AA, MacNeill SJ, Yacoub M, Shore DF, Shinebourne EA. Complete atrioventricular septal defect, Down syndrome, and surgical outcome: risk factors. Ann Thorac Surg. 2003;75:412-421. (Pubitemid 36184481)
12. Lange R, Guenther T, Busch R, Hess J, Schreiber C. The presence of Down syndrome is not a risk factor in complete atrioventricular septal defect repair. J Thorac Cardiovasc Surg. 2007;134:304-310. (Pubitemid 47088564)
13. Formigari R, Di Donato RM, Gargiulo G, Di Carlo D, Feltri C, Picchio FM, Marino B. Better surgical prognosis for patients with complete atrioventricular septal defect and Down's syndrome. Ann Thorac Surg. 2004;78:666-672; discussion 672. (Pubitemid 38980794)
14. Song MS, Hu A, Dyamenahalli U, Dyhamenahali U, Chitayat D, Winsor EJ, Ryan G, Smallhorn J, Barrett J, Yoo SJ, Hornberger LK. Extracardiac lesions and chromosomal abnormalities associated with major fetal heart defects: comparison of intrauterine, postnatal and postmortem diagnoses. Ultrasound Obstet Gynecol. 2009;33:552-559.
15. Frid C, Drott P, Lundell B, Rasmussen F, Annerén G. Mortality in Down's syndrome in relation to congenital malformations. J Intellect Disabil Res. 1999;43(pt 3):234-241. (Pubitemid 29271631)
16. Freeman SB, Bean LH, Allen EG, Tinker SW, Locke AE, Druschel C, Hobbs CA, Romitti PA, Royle MH, Torfs CP, Dooley KJ, Sherman SL. Ethnicity, sex, and the incidence of congenital heart defects: a report from the National Down Syndrome Project. Genet Med. 2008;10:173-180. (Pubitemid 351416813)
17. Alexi-Meskishvili V, Ishino K, Dähnert I, Uhlemann F, Weng Y, Lange PE, Hetzer R. Correction of complete atrioventricular septal defects with the double-patch technique and cleft closure. Ann Thorac Surg. 1996;62:519-524; discussion 524.
18. Günther T, Mazzitelli D, Haehnel CJ, Holper K, Sebening F, Meisner H. Long-term results after repair of complete atrioventricular septal defects: analysis of risk factors. Ann Thorac Surg. 1998;65:754-759; discussion 759.
19. Masuda M, Kado H, Tanoue Y, Fukae K, Onzuka T, Shiokawa Y, Shirota T, Yasui H. Does Down syndrome affect the long-term results of complete atrioventricular septal defect when the defect is repaired during the first year of life? Eur J Cardiothorac Surg. 2005;27:405-409. (Pubitemid 40312346)
20. Morris CD, Magilke D, Reller M. Down's syndrome affects results of surgical correction of complete atrioventricular canal. Pediatr Cardiol. 1992;13:80-84.
21. Tweddell JS, Litwin SB, Berger S, Friedberg DZ, Thomas JP, Frommelt PC, Frommelt MA, Pelech AN, Lewis DA, Fedderly RT, Mussatto KA, Kessel MW. Twenty-year experience with repair of complete atrioventricular septal defects. Ann Thorac Surg. 1996;62:419-424. (Pubitemid 26259683)
22. Atz AM, Hawkins JA, Lu M, Cohen MS, Colan SD, Jaggers J, Lacro RV, McCrindle BW, Margossian R, Mosca RS, Sleeper LA, Minich LL; Pediatric Heart Network Investigators. Surgical management of complete atrioventricular septal defect: associations with surgical technique, age, and trisomy 21. J Thorac Cardiovasc Surg. 2011;141:1371-1379.
23. Minich LL, Tani LY, Pagotto LT, Hawkins JA, McGough EC, Shaddy RE. Size of ventricular structures influences surgical outcome in Down syndrome infants with atrioventricular septal defect. Am J Cardiol. 1998;81:1062-1065. (Pubitemid 28183678)
24. Fudge JC Jr, Li S, Jaggers J, O'Brien SM, Peterson ED, Jacobs JP, Welke KF, Jacobs ML, Li JS, Pasquali SK. Congenital heart surgery outcomes in Down syndrome: analysis of a national clinical database. Pediatrics. 2010;126:315-322.
25. Seifert HA, Howard DL, Silber JH, Jobes DR. Female gender increases the risk of death during hospitalization for pediatric cardiac surgery. J Thorac Cardiovasc Surg. 2007;133:668-675. (Pubitemid 46274775)
26. Marelli A, Gauvreau K, Landzberg M, Jenkins K. Sex differences in mortality in children undergoing congenital heart disease surgery: a United States population-based study. Circulation. 2010;122(11 suppl):S234-S240.
27. Jenkins KJ, Gauvreau K, Newburger JW, Spray TL, Moller JH, Iezzoni LI. Consensus-based method for risk adjustment for surgery for congenital heart disease. J Thorac Cardiovasc Surg. 2002;123:110-118.
28. Jenkins KJ, Gauvreau K. Center-specific differences in mortality: preliminary analyses using the Risk Adjustment in Congenital Heart Surgery (RACHS-1) method. J Thorac Cardiovasc Surg. 2002;124:97-104.
29. Jacobs JP, Jacobs ML, Lacour-Gayet FG, Jenkins KJ, Gauvreau K, Bacha E, Maruszewski B, Clarke DR, Tchervenkov CI, Gaynor JW, Spray TL, Stellin G, O'Bien SM, Elliott MJ, Mavroudis C. Stratification of complexity improves the utility and accuracy of outcomes analysis in a Multi-Institutional Congenital Heart Surgery Database: Application of the Risk Adjustment in Congenital Heart Surgery (RACHS-1) and Aristotle Systems in the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database. Pediatr Cardiol. 2009;30:1117-1130.
30. Campbell RM, Adatia I, Gow RM, Webb GD, Williams WG, Freedom RM. Total cavopulmonary anastomosis (Fontan) in children with Down's syndrome. Ann Thorac Surg. 1998;66:523-526. (Pubitemid 28410689)
31. Garlet TR, Parisotto EB, de Medeiros Gda S, Pereira LC, Moreira EA, Dalmarco EM, Dalmarco JB, Wilhelm Filho D. Systemic oxidative stress in children and teenagers with Down syndrome. Life Sci. 2013;93:558-563.
32. Shott SR. Down syndrome: common otolaryngologic manifestations. Am J Med Genet C Semin Med Genet. 2006;142C:131-140. (Pubitemid 44298195)
33. Bertrand P, Navarro H, Caussade S, Holmgren N, Sánchez I. Airway anomalies in children with Down syndrome: endoscopic findings. Pediatr Pulmonol. 2003;36:137-141. (Pubitemid 36959553)
34. Harrison AM, Cox AC, Davis S, Piedmonte M, Drummond-Webb JJ, Mee RB. Failed extubation after cardiac surgery in young children: prevalence, pathogenesis, and risk factors. Pediatr Crit Care Med. 2002;3:148-152.
35. D'Alto M, Mahadevan VS. Pulmonary arterial hypertension associated with congenital heart disease. Eur Respir Rev. 2012;21:328-337.
36. Gowda S, Bhat D, Feng Z, Chang CH, Ross RD. Pulmonary vein stenosis with Down syndrome: a rare and frequently fatal cause of pulmonary hypertension in infants and children. Congenit Heart Dis. 2013. DOI: 10.1111/chd.12088.
37. Wada N, Takahashi Y, Ando M, Park IS, Sasaki T. Single ventricle repair in children with Down's syndrome. Gen Thorac Cardiovasc Surg. 2008;56:104-108. (Pubitemid 351414883)
38. Gupta-Malhotra M, Larson VE, Rosengart RM, Guo H, Moller JH. Mortality after total cavopulmonary connection in children with the down syndrome. Am J Cardiol. 2010;105:865-868.
39. Furukawa T, Park IS, Yoshikawa T, Nishimura T, Takahashic Y, Ando M, Wada N. Outcome of univentricular repair in patients with Down syndrome. J Thoracic Cardiovasc Surg. 2013;146:1349-1352.
40. Hickey PA, Gauvreau K, Jenkins K, Fawcett J, Hayman L. Statewide and national impact of California's Staffing Law on pediatric cardiac surgery outcomes. J Nurs Adm. 2011;41:218-225.
41. Welke KF, Diggs BS, Karamlou T, Ungerleider RM. Comparison of pediatric cardiac surgical mortality rates from national administrative data to contemporary clinical standards. Ann Thorac Surg. 2009;87:216-222; discussion 222.