22q11.2 Deletion Status Influences Resource Utilization in Infants Requiring Repair of Tetralogy of Fallot and Common Arterial Trunk.

22q11.2 deletion syndrome leads to both cardiac and non-cardiac developmental defects. We aimed to study the impact of 22q11.2 deletion syndrome on in-hospital outcomes in children undergoing surgical repair for tetralogy of Fallot (TOF) and truncus arteriosus (TA). Using the nationally representative Kids Inpatient Database (KID), we analyzed data from in-hospital pediatric patients for the years 2003, 2006, 2009, and 2012. We compared the in-hospital outcomes between those with and those without 22q11.2 deletion syndrome. There were 6126 cases of TOF and 968 cases of TA. 22q11.2 deletion syndrome were documented in 7.2% (n = 441) of the TOF and 27.4% (n  =  265) of the TA group. 22q11.2 deletion did not significantly increase the risk of mortality in either group: [OR = 1.98 (95% CI 0.99-3.94), adjusted p  =  0.053] for TOF and OR = 1.07 (95% CI 0.57-1.99), adjusted p = 0.82 for TA. However, the length of hospitalization was longer in the 22q11.2 deletion group by 8.6 days (95% CI 5.2-12), adjusted p < 0.001 for TOF and by 8.15 days (95% CI 1.05-15.25), adjusted p = 0.025 for the TA group. Acute respiratory failure [10.6% vs 5.5%, p < 0.001] and acute renal failure [6.3% vs 2.6%, p < 0.001] were higher in 22q11.2 deletion cohort within the TOF group but not in the TA group. Though survival is not affected, children with 22q11.2 deletion syndrome who undergo surgical repair for TOF and TA use significantly more hospital resources-specifically longer hospital stay and higher hospitalization cost-than those without 22q11.2 deletion syndrome. Prenatal or preoperative testing for 22q11deletion is indicated to make appropriate adjustments in parental, caregiver, and administrative expectations.

Comparison of In-Hospital Outcomes When Repair of Tetralogy of Fallot Is in the Neonatal Period Versus in the Post-Neonatal Period.

Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease (CHD) and optimal timing for total repair of TOF is controversial. We hypothesize that TOF repair in the neonatal period is associated with worse outcomes compared with those who undergo repair later in infancy. We analyzed data using the Kids' Inpatient Database (KID) from 2003 to 2012. We used multivariable logistic regression analyses to compare the in-hospital outcomes between those who underwent total repair of TOF during the neonatal period vs the postneonatal period. There were 6,856 cases of TOF and 7.83% (n = 537) of those underwent repair during the neonatal period. The average mortality in all TOF repair was 2.1% (n = 147). In multiple regression model, compared with repair in postneonatal period, neonatal repair was associated with increased mortality, with adjusted odds ratio of 2.2 (95% confidence interval [CI]: 1.1 to 4.3, p = 0.023). Regarding complications, the neonatal group was associated with higher risk of acute renal failure (8.9% vs 2.3%, p <0.001), need for cardiac catheterization (18.6% vs 8.3%, p <0.001), and ECMO use (4.4% vs1.6%, p <0.001). There was no difference in the rates of arrhythmia, respiratory failure, pulmonary hypertension, or sudden cardiac arrest. Children who underwent repair in the neonatal period had longer hospital stay compared with the postneonatal group (45.5 days [95% CI: 39.3 to 51.7] vs 12.6 days [95% CI: 11.7 to 13.4], p <0.001). Hospital charges were higher for children who underwent repair in the neonatal period compared with those in the postneonatal period. In conclusion, TOF repair in the neonatal period is associated with higher rates of mortality, more postoperative complications, longer hospital stays, and higher hospitalization cost.