Congenital Heart Surgical Admissions in Patients with Trisomy 13 and 18: Frequency, Morbidity, and Mortality

Congenital heart defects are common among patients with trisomy 13 and 18; surgical repair has been controversial and rarely studied. We aimed to assess the frequency of cardiac surgery among admissions with trisomy 13 and 18, and evaluate their associations with resource use, complications, and mortality compared to admissions without these diagnoses. We evaluated congenital heart surgery admissions of ages < 18 years in the 1997, 2000, 2003, 2006, and 2009 Kids’ Inpatient Database. Bivariate and multivariate analyses examined the adjusted association of trisomy 13 and 18 on resource use, complications, and inpatient death following congenital heart surgery. Among the 73,107 congenital heart surgery admissions, trisomy 13 represented 0.03% (n = 22) and trisomy 18 represented 0.08% (n = 58). Trisomy 13 and 18 admissions were longer; trisomy 13: 27 days vs. 8 days, p = 0.003; trisomy 18: 16 days vs. 8 days, p = 0.001. Hospital charges were higher for trisomy 13 and 18 admissions; trisomy 13: $160,890 vs. $87,007, p = 0.010; trisomy 18: $160,616 vs. $86,999, p < 0.001. Trisomy 18 had a higher complication rate: 52% vs. 34%, p < 0.006. For all cardiac surgery admissions, mortality was 4.5%; trisomy 13: 14% and trisomy 18: 12%. In multivariate analysis, trisomy 18 was an independent predictor of death: OR 4.16, 95% CI 1.35–12.82, p = 0.013. Patients with trisomy 13 and 18 represent 0.11% of pediatric congenital heart surgery admissions. These patients have a 2- to 3.4-fold longer hospital stay and double hospital charges. Patients with trisomy 18 have more complications and four times greater adjusted odds for inpatient death.