Multicenter Evaluation of Pre-operative Feeding in Infants with Ductal Dependent Circulation.

Enteral feeds are often withheld from neonates with ductal dependent cardiac lesions who are receiving prostaglandins. This is despite positive benefits of enteral feeding. We describe a multicenter cohort of these neonates who were fed pre-operatively. We also give a granular description of vital sign measurements and other risk factors prior to feeding. A retrospective chart review was performed at seven centers. Inclusion criteria were full-term neonates under one month of age with ductal dependent lesions receiving prostaglandins. These neonates were fed for at least 24 h during the pre-operative period. Premature neonates were excluded. Using the inclusion criteria, 127 neonates were identified. While being fed, 20.5% of the neonates were intubated, 10.2% were on inotropes, and 55.9% had an umbilical arterial catheter in place. Median oxygen saturations in the six hours prior to feeding were 92.5% in patients with cyanotic lesions, median diastolic blood pressure was 38 mmHg and median somatic NIRS were 66.5%. The median peak daily feeding volume reached was 29 ml/kg/day (IQ range 15.5-96.8 ml/kg/day). One patient developed suspected necrotizing enterocolitis (NEC) in this cohort. Only one adverse event occurred, which was an aspiration thought to be related to feeding, but did not result in intubation or cessation of feeds. NEC was rare among neonates with ductal dependent lesions while receiving enteral nutrition pre-operatively. Umbilical arterial catheters were in place in the majority of these patients. Hemodynamic measures demonstrated a high median oxygen saturation prior to initiation of feeds.

Impact of cochlear abnormalities on hearing outcomes for children with cochlear implants.

OBJECTIVE: Evaluate the impact of cochlear anomalies on hearing outcomes for pediatric patients with cochlear implants. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary care center. SUBJECTS AND METHODS: Charts were retrospectively reviewed for cases where pediatric cochlear implant surgery was performed between 2002 and 2018 at a single, tertiary care institution. Patients were divided into groups based on the presence or absence of radiological cochlear abnormalities, which were further classified as low or high risk anomalies. Hearing outcomes were evaluated by measuring pure tone averages and word recognition scores preoperatively, 3 and 12 months postoperatively, in addition to the most recent test results. RESULTS: There were 154 ears implanted in our cohort of 100 patients. 107 ears had normal cochlear anatomy, 31 had low risk, and 16 had high risk abnormalities. The most common modality of preoperative imaging was CT scan. Postoperative mean pure tone average (PTA) was significantly higher in patients with inner ear anomalies compared to those with normal anatomy. No significant difference in PTA was noted between low versus high risk patients. <50% of patients had word recognition scores available within the first year following surgery. CONCLUSION: Abnormalities of the inner ear significantly influenced hearing outcomes over time following cochlear implant surgery when compared to pediatric patients with normal anatomy. Obtaining hearing testing can be difficult in very young children and therefore future studies are warranted to further investigate the impact that cochlear abnormalities may have on hearing outcomes following cochlear implant surgery.

Chromosomal Abnormalities Affect the Surgical Outcome in Infants with Hypoplastic Left Heart Syndrome: A Large Cohort Analysis.

Patients with hypoplastic left heart syndrome (HLHS) can have associated genetic abnormalities. This study evaluated the incidence of genetic abnormalities among infants with HLHS and the short-term outcomes of this population during the first hospitalization. This is a retrospective analysis of the multi-center Pediatric Heath Information System database of infants with HLHS who underwent Stage I Norwood, Hybrid, or heart transplant during their first hospitalization from 2004 through 2013. We compared clinical data between infants with and without genetic abnormality, among the three most common chromosomal abnormalities, and between survivors and non-survivors. Multivariable analysis was completed to evaluate predictors of mortality among patients with genetic abnormalities. A total of 5721 infants with HLHS were identified; 282 (5%) had associated genetic abnormalities. The three most common chromosomal abnormalities were Turner (25%), DiGeorge (22%), and Downs (12.7%) syndromes. Over the study period, the number of patients with genetic abnormalities undergoing cardiac operations increased without any significant increases in mortality. Infants with genetic abnormalities compared to those without abnormalities had longer hospital length of stay and higher morbidity and mortality. Variables associated with mortality were lower gestational age, longer duration of vasopressor therapy, need for dialysis, and cardiopulmonary resuscitation; and complicated clinical course as suggested by necrotizing enterocolitis, septicemia. Presence of any genetic abnormality in infants with HLHS undergoing cardiac surgery is associated with increased mortality and morbidity. Timely genetic testing, appropriate family counseling, and thorough preoperative case selection are suggested for these patients for any operative intervention.

Epinephrine dosing strategies during pediatric extracorporeal cardiopulmonary resuscitation reveal novel impacts on survival: A multicenter study utilizing time-stamped epinephrine dosing records.

OBJECTIVES: To describe epinephrine dosing distribution using time-stamped data and assess the impact of dosing strategy on survival after ECPR in children. METHODS: This was a retrospective study at five pediatric hospitals of children <18 years with an in-hospital ECPR event. Mean number of epinephrine doses was calculated for each 10-minute CPR interval and compared between survivors and non-survivors. Patients were also divided by dosing strategy into a frequent epinephrine group (dosing interval of ≤5 min/dose throughout the first 30 minutes of the event), and a limited epinephrine group (dosing interval of ≤5 min/dose for the first 10 minutes then >5 min/dose for the time between 10 and 30 minutes). RESULTS: A total of 191 patients were included. Epinephrine was not evenly distributed throughout ECPR, with 66% of doses being given during the first half of the event. Mean number of epinephrine doses was similar between survivors and non-survivors the first 10 minutes (2.7 doses). After 10 minutes, survivors received fewer doses than non-survivors during each subsequent 10-minute interval. Adjusted survival was not different between strategy groups [OR of survival for frequent epinephrine strategy: 0.78 (95% CI 0.36-1.69), p = 0.53]. CONCLUSIONS: Survivors received fewer doses than non-survivors after the first 10 minutes of CPR and although there was no statistical difference in survival based on dosing strategy, the findings of this study question the conventional approach to EPCR analysis that assumes dosing is evenly distributed.

Severe Acute Neurologic Involvement in Children With Hemolytic-Uremic Syndrome.

BACKGROUND AND OBJECTIVES: Acute severe neurologic involvement is the most threatening complication in children with hemolytic-uremic syndrome (HUS). Our primary study objectives were to describe the association between acute neurologic manifestations (ANMs) and in-hospital mortality among children with HUS. METHODS: Using the Pediatric Health Information System database, in this retrospective multicenter cohort study, we identified the first HUS-related inpatient visit among children ≤18 years (years 2004-2018). Frequency of selected ANMs and combinations of ANMs, as well as the rate of mortality, was calculated. Multivariate logistic regression was used to identify the association of ANMs and the risk of in-hospital mortality. RESULTS: Among 3915 patients included in the analysis, an ANM was noted in 10.4% (n = 409) patients. Encephalopathy was the most common ANM (n = 245). Mortality was significantly higher among patients with an ANM compared with patients without an ANM (13.9% vs 1.8%; P < .001). Individuals with any ANM had increased odds of mortality (odds ratio [OR]: 2.25; 95% confidence interval [CI]: 1.29-3.93; P = .004), with greater risk (OR: 2.60; 95% CI: 1.34-5.06; P = .005) among patients with ≥2 manifestations. Brain hemorrhage (OR: 3.09; 95% CI: 1.40-6.82; P = .005), brain infarction (OR: 2.64; 95% CI: 1.10-6.34; P = .03), anoxic brain injury (OR: 3.92; 95% CI: 1.49-10.31; P = .006), and brain edema (OR: 4.81; 95% CI: 1.82-12.71; P = .002) were independently associated with mortality. CONCLUSIONS: In this study, the largest systematic assessment of ANMs among children with HUS to date, we identify differences in in-hospital mortality based on the type of ANM, with increased risk observed for patients with multiple ANMs.