Hospital Outcomes Among Children With Congenital Heart Disease and Adenovirus Pneumonia.

BACKGROUND: The aim of the study is to evaluate the mortality risk factors and hospitalization outcomes of adenovirus pneumonia in pediatric patients with congenital heart disease. METHODS: In this retrospective multicenter cohort study utilizing the Pediatric Health Information System database, we analyzed congenital heart disease patients with adenovirus pneumonia from January 2004 to September 2018, categorizing them into shunts, obstructive lesions, cyanotic lesions and mixing lesions. Multivariate logistic regression analysis was employed to identify mortality risk factors with 2 distinct models to mitigate collinearity issues and the Mann-Whitney U test was used to compare the hospital length of stay between survivors and nonsurvivors across these variables. RESULTS: Among 381 patients with a mean age of 3.2 years (range: 0-4 years), we observed an overall mortality rate of 12.1%, with the highest mortality of 15.1% noted in patients with shunts. Model 1 identified independent factors associated with increased mortality, including age 0-30 days (OR: 8.13, 95% CI: 2.57-25.67, P < 0.005), sepsis/shock (OR: 3.34, 95% CI: 1.42-7.83, P = 0.006), acute kidney failure (OR: 4.25, 95% CI: 2.05-13.43, P = 0.0005), shunts (OR: 2.95, 95% CI: 1.14-7.67, P = 0.03) and cardiac catheterization (OR: 6.04, 95% CI: 1.46-24.94, P = 0.01), and Model 2, extracorporeal membrane oxygenation (OR: 3.26, 95% CI: 1.35-7.87, P = 0.008). Nonsurvivors had a median hospital stay of 47 days compared to 15 days for survivors. CONCLUSION: The study revealed a 12.1% mortality rate in adenoviral pneumonia among children with congenital heart disease, attributed to risk factors such as neonates, sepsis, acute kidney failure, shunts, cardiac catheterization, extracorporeal membrane oxygenation use and a 3-fold longer hospital stay for nonsurvivors compared to survivors.

Streptococcus pneumoniae associated hemolytic uremic syndrome in children.

Introduction: Previous small-scale, single-center investigations of Streptococcus pneumoniae associated hemolytic uremic syndrome (SpHUS) have shown increased disease severity among SpHUS relative to non-SpHUS patients. Our study compares the impact of S. pneumoniae on patient outcomes between SpHUS cases and non-SpHUS controls using the national, multicenter retrospective Pediatric Health Information Systems (PHIS) Database. Methods: Children <18 years of age with a diagnosis of HUS were included. Univariate analyses and multivariable linear and logistic regressions were utilized to assess the impact of S. pneumoniae on mortality, length of stay (LOS), intensive care unit admission (ICU), and mechanical ventilation use. Models were adjusted for demographic and clinical characteristics, including cardiac, neurologic, pulmonary, gastrointestinal, immunologic and renal clinical complications. Results: Of 3,952 index HUS hospitalizations, 231 (5.8%) were due to SpHUS. SpHUS patients had worse outcomes, including longer hospital stays, increased rate of ICU admission, and increased use of mechanical ventilation (p < 0.001 for all). There was a strong positive relationship between clinical complications and adverse outcomes. After adjusting for covariates, SpHUS was associated with an increase in hospital LOS by 3.47 days (p = 0.009) and overall ICU-LOS by 4.21 days (p < 0.001). SpHUS was also associated with increased likelihood of mechanical ventilation (OR: 3.08; p < 0.001), with no increase in ICU admission (p = 0.070) and in-hospital mortality (p = 0.3874). Discussion: Our study highlights that SpHUS patients are at increased risk of multiple adverse outcomes likely due to the summative impact of pneumococcal infection and HUS as well as more frequent clinical complications.

Evaluation of lung ultrasound to detect volume overload in children undergoing dialysis.

BACKGROUND: Lung ultrasound is a well-established technique to assess extravascular lung water, a proxy for volume status, in the adult population. Despite its utility, the data are limited supporting the use of ultrasound to evaluate fluid volume status among pediatric patients. Our study uses a simplified ultrasound protocol to evaluate changes in extravascular lung water, represented by b-lines, among pediatric patients undergoing hemodialysis. METHODS: This prospective single-center study included children from birth to 18 years of age. The number of b-lines per ml/kg of fluid removed was compared prior to, at the midpoint, and following termination of dialysis. An 8-zone protocol was utilized, and b-lines were correlated to hemoconcentration measured by the CRIT-LINE® hematocrit. RESULTS: Six patients with a total of 26 hemodialysis sessions were included in this study. The b-line measurements post-dialysis were 2.27 (p < 0.001; 94%CI -3.31, -1.22) lower relative to pre-dialysis. The number of b-lines was reduced by 1.69 (p < 0.001; -2.58, -0.80) between pre-dialysis and at the midpoint of dialysis and by 0.58 (p = 0.001; -0.90, -0.24) between the midpoint of dialysis and post-dialysis. A 1 mL/kg fluid loss correlated to a decrease in the original b-lines by 0.079. An inverse relationship (r = -0.54; 95% CI: -0.72, -0.34; p < 0.001) was noted between the b-lines and the patients' hematocrit levels. CONCLUSIONS: A simplified 8-zone ultrasound protocol can assess fluid volume change in real time and correlates with hematocrit levels obtained throughout dialysis. This provides a valuable method for monitoring fluid status in volume overloaded patient populations. A higher resolution version of the Graphical abstract is available as Supplementary information.

Norwood Operation: Immediate vs Delayed Sternal Closure.

BACKGROUND: The Norwood operation is a complex neonatal surgery. There are limited data to inform the timing of sternal closure. After the Norwood operation, delayed sternal closure (DSC) is frequent. We aimed to examine the association of DSC with outcomes, with a particular interest in how sternal closure at the time of surgery compared with the timing of DSC. Our outcomes included mortality, length of ventilation, length of stay, and postoperative complications. METHODS: This retrospective study included neonates who underwent a Norwood operation reported in the Pediatric Cardiac Critical Care Consortium registry from February 2019 through April 2021. Outcomes of patients with closed sternum were compared to those with sternal closure prior to postoperative day 3 (early closure) and prior to postoperative day 6 (intermediate closure). RESULTS: The incidence of DSC was 74% (500 of 674). The median duration of open sternum was 4 days (interquartile range 3-5 days). Comparing patients with closed sternum to patients with early sternal closure, there was no statistical difference in mortality rate (1.1% vs 0%) and the median hospital postoperative stay (30 days vs 31 days). Compared with closed sternum, patients with intermediate sternal closure required longer mechanical ventilation (5.9 days vs 3.9 days) and fewer subsequent sternotomies (3% vs 7.5%). CONCLUSIONS: For important outcomes following the Norwood operation there is no advantage to chest closure at the time of surgery if the chest can be closed prior to postoperative day 3.

Preventing Cardiac Arrest in the Pediatric Cardiac Intensive Care Unit Through Multicenter Collaboration.

Importance: Preventing in-hospital cardiac arrest (IHCA) likely represents an effective strategy to improve outcomes for critically ill patients, but feasibility of IHCA prevention remains unclear. Objective: To determine whether a low-technology cardiac arrest prevention (CAP) practice bundle decreases IHCA rate. Design, Setting, and Participants: Pediatric cardiac intensive care unit (CICU) teams from the Pediatric Cardiac Critical Care Consortium (PC4) formed a collaborative learning network to implement the CAP bundle consistent with the Institute for Healthcare Improvement framework; 15 hospitals implemented the bundle voluntarily. Risk-adjusted IHCA incidence rates were analyzed across 2 time periods, 12 months (baseline) and 18 months after CAP implementation (intervention) using difference-in-differences (DID) regression to compare 15 CAP and 16 control PC4 hospitals that chose not to participate in CAP but had IHCA rates tracked in the PC4 registry. Patients deemed at high risk for IHCA, based on a priori evidence-based criteria and empirical hospital-specific criteria, were selected to receive the CAP bundle. Data were collected from July 2018 to December 2019, and data were analyzed from March to August 2020. Interventions: CAP bundle included 5 elements developed to promote increased situational awareness and communication among bedside clinicians to recognize and mitigate deterioration in high-risk patients. Main Outcomes and Measures: Risk-adjusted IHCA incidence rate across all CICU admissions (IHCA events divided by all admissions). Results: The bundle was activated in 2664 of 10 510 CAP hospital admissions (25.3%); admission characteristics were similar across study periods. There was a 30% relative reduction in risk-adjusted IHCA incidence rate at CAP hospitals (intervention period: 2.6%; 95% CI, 2.2-2.9; baseline: 3.7%; 95% CI, 3.1-4.0), but no change at control hospitals (intervention period: 2.7%; 95% CI, 2.3-2.9; baseline: 2.7%; 95% CI, 2.2-3.0). DID analysis confirmed significantly reduced odds of IHCA among all admissions at CAP hospitals compared with control hospitals during the intervention period vs baseline (odds ratio, 0.72; 95% CI, 0.56-0.91; P = .01). DID odds ratios were 0.72 (95% CI, 0.53-0.98) for the surgical subgroup, 0.74 (95% CI, 0.48-1.14) for the medical subgroup, and 0.72 (95% CI, 0.50-1.03) for the high-risk admission subgroup at CAP hospitals after intervention. All-cause risk-adjusted mortality rate did not change after intervention. Conclusions and Relevance: Implementation of this CAP bundle led to significant IHCA reduction across multiple pediatric CICUs. Future studies may determine if this bundle can be effective in other critically ill populations.

Development of consensus recommendations for the management of post-operative chylothorax in paediatric CHD.

OBJECTIVE: A standardised multi-site approach to manage paediatric post-operative chylothorax does not exist and leads to unnecessary practice variation. The Chylothorax Work Group utilised the Pediatric Critical Care Consortium infrastructure to address this gap. METHODS: Over 60 multi-disciplinary providers representing 22 centres convened virtually as a quality initiative to develop an algorithm to manage paediatric post-operative chylothorax. Agreement was objectively quantified for each recommendation in the algorithm by utilising an anonymous survey. "Consensus" was defined as ≥ 80% of responses as "agree" or "strongly agree" to a recommendation. In order to determine if the algorithm recommendations would be correctly interpreted in the clinical environment, we developed ex vivo simulations and surveyed patients who developed the algorithm and patients who did not. RESULTS: The algorithm is intended for all children (<18 years of age) within 30 days of cardiac surgery. It contains rationale for 11 central chylothorax management recommendations; diagnostic criteria and evaluation, trial of fat-modified diet, stratification by volume of daily output, timing of first-line medical therapy for "low" and "high" volume patients, and timing and duration of fat-modified diet. All recommendations achieved "consensus" (agreement >80%) by the workgroup (range 81-100%). Ex vivo simulations demonstrated good understanding by developers (range 94-100%) and non-developers (73%-100%). CONCLUSIONS: The quality improvement effort represents the first multi-site algorithm for the management of paediatric post-operative chylothorax. The algorithm includes transparent and objective measures of agreement and understanding. Agreement to the algorithm recommendations was >80%, and overall understanding was 94%.

Employment Opportunities for Pediatric Critical Care Fellowship Trained Physicians in the United States.

Pediatric Critical Care Medicine (PCCM) training programs and trained fellows in the United State increased steadily without a corresponding increase in population growth. PCCM trainees worry about limited employment prospects. This study aimed to quantify the demand for PCCM trained physicians in the United States by prospectively tracking full-time employment opportunities over 12 months. The number of advertised opportunities identified was low compared with number of fellows likely to be seeking jobs during same time period. If market demand remains stable, there is risk of excess supply if number of newly fellowship-trained PCCM physicians continues to rise.

Comparison of Postoperative, In-Hospital Outcomes After Complete Repair of Tetralogy of Fallot Between 22q11.2 Deletion Syndrome and Trisomy 21.

22q11.2 deletion syndrome (22q11) and trisomy 21 (T21) are frequently associated with tetralogy of Fallot (TOF). We hypothesized that there are differences in postoperative length of stay (LOS) and occurrence of postoperative interventions after complete repair of TOF when comparing children with 22q11 to those with T21. Using the Pediatric Health Information System, we performed a retrospective cohort study of patients who underwent complete repair of TOF from 2004 to 2019. Three groups were identified: 22q11, T21, and controls (those without a coded genetic syndrome). Outcomes were postoperative LOS and composite occurrence (yes/no) of at least one postoperative intervention. Bivariate and multivariate comparisons were made among groups; odds ratios (ORs) with 95% confidence intervals (CIs) were calculated using the control group as the comparator. There were 6924 subjects (n = 493 22q11, n = 455 T21, n = 5976 controls). In bivariate analysis, 22q11 was associated with a longer LOS compared to T21 (OR 2.37 [2.16, 2.60] vs. 1.25 [1.12, 1.39], p < 0.001), and 22q11 more often underwent postoperative intervention (OR 3.42 [CI 2.56, 4.57] vs. 1.38 [CI 0.91, 2.11]; p < 0.001). In multivariate analysis, 22q11 was also associated with longer LOS (adjusted OR 1.35 [1.26, 1.44] vs. 1.12 [1.04, 1.20]; p < 0.001), but there was no difference in the adjusted odds of postoperative intervention. Children with 22q11 are more likely to experience adverse outcomes after repair of TOF compared to those with T21; the differences are most pronounced for LOS.

Utilization Pattern for Eculizumab Among Children With Hemolytic Uremic Syndrome.

Background: Hemolytic uremic syndrome (HUS) is a complex disease with multi-organ involvement. Eculizumab therapy is recommended for treatment of complement mediated hemolytic uremic syndrome (cHUS). However, there are few studies evaluating eculizumab therapy among children with HUS. The primary objectives of the study were to describe and identify factors associated with eculizumab therapy in children with HUS. Design/Methods: This large, retrospective, multi-center, cohort study used the Pediatric Health Information System (PHIS) database to identify the index HUS-related hospitalization among patients ≤18 years of age from September 23, 2011 (Food and Drug Administration approval date of eculizumab) through December 31, 2018. Multivariate analysis was used to identify independent factors associated with eculizumab therapy during or after the index hospitalization. Results: Among 1,885 children included in the study, eculizumab therapy was noted in 167 children with a median age of 3.99 years (SD ± 4.7 years). Eculizumab therapy was administered early (within the first 7 days of hospitalization) among 65% of children who received the drug. Mortality during the index hospitalization among children with eculizumab therapy was 4.2 vs. 3.0% without eculizumab therapy (p = 0.309). Clinical factors independently associated with eculizumab therapy were encephalopathy [odds ratio (OR) = 3.09; p ≤ 0.001], seizure disorder (OR = 2.37; p = 0.006), and cardiac involvement (OR = 6.36, p < 0.001). Conclusion(s): Only 8.9% of children received eculizumab therapy. Children who presented with neurological and cardiac involvement with severe disease were more likely to receive eculizumab therapy, and children who received therapy received it early during their index hospitalization. Further prospective studies are suggested to confirm these findings.

Implementing Pharmacogenomics Testing: Single Center Experience at Arkansas Children's Hospital.

Pharmacogenomics (PGx) is a growing field within precision medicine. Testing can help predict adverse events and sub-therapeutic response risks of certain medications. To date, the US FDA lists over 280 drugs which provide biomarker-based dosing guidance for adults and children. At Arkansas Children's Hospital (ACH), a clinical PGx laboratory-based test was developed and implemented to provide guidance on 66 pediatric medications for genotype-guided dosing. This PGx test consists of 174 single nucleotide polymorphisms (SNPs) targeting 23 clinically actionable PGx genes or gene variants. Individual genotypes are processed to provide per-gene discrete results in star-allele and phenotype format. These results are then integrated into EPIC- EHR. Genomic indicators built into EPIC-EHR provide the source for clinical decision support (CDS) for clinicians, providing genotype-guided dosing.

Risk Factors for Increased Post-operative Length of Stay in Children with Coarctation of Aorta.

Coarctation of the aorta is a relatively common congenital heart disease occurring in 0.4-0.6 per 1000 live births with a low mortality rate. This is a retrospective study, with data abstracted from the Pediatric Health Information System database (PHIS). The study sample included pediatric patients less than or equal to 3 months of age discharged from a PHIS participating hospital between January 1, 2004 and December 31, 2018 who underwent surgical repair of isolated COA. The primary outcome for the study was post-operative hospital length of stay (PH-LOS), and the secondary outcome was in-hospital mortality. Patient demographics, comorbidities, procedures, and outcomes were assessed for statistical differences between eras. A total of 5354 patients were included in the study. The study highlights an increasing trend in PH-LOS and NICU hospital length of stay (NICU-LOS) across the investigated eras. Prematurity (before 37 weeks gestation) was an independent risk factor associated with both longer post-operative length of the stay and higher mortality. In addition, congenital anomalies, respiratory and abdominal surgeries have a significant impact on the post-operative hospital stay. In conclusion, this study is the largest published systematic assessment of PH-LOS in patients with isolated COA repair during infancy to date and identifies independent risk factors of increased PH-LOS.

Cardiac Manifestation among Children with Hemolytic Uremic Syndrome.

OBJECTIVE: The primary objectives of the study were to describe the association between cardiac manifestations and in-hospital mortality among children with hemolytic uremic syndrome. STUDY DESIGN: Using the Pediatric Health Information System database, this retrospective, multicenter, cohort study identified the first hemolytic uremic syndrome-related inpatient visit among children ≤18 years (years 2004-2018). The frequency of selected cardiac manifestations and mortality rates were calculated. Multivariate analysis identified the association of specific cardiac manifestations and the risk of in-hospital mortality. RESULTS: Among 3915 patients in the analysis, 238 (6.1%) had cardiac manifestations. A majority of patients (82.8%; n = 197) had 1 cardiac condition and 17.2% (n = 41) had ≥2 cardiac conditions. The most common cardiac conditions was pericardial disease (n = 102), followed by congestive heart failure (n = 46) and cardiomyopathy/myocarditis (n = 34). The percent mortality for patients with 0, 1, or ≥2 cardiac conditions was 2.1%, 17.3%, and 19.5%, respectively. Patients with any cardiac condition had an increased odds of mortality (OR, 9.74; P = .0001). In additional models, the presence of ≥2 cardiac conditions (OR, 9.90; P < .001), cardiac arrest (OR, 38.25; P < .001), or extracorporeal membrane oxygenation deployment (OR, 11.61; P < .001) were associated with increased risk of in-hospital mortality. CONCLUSIONS: This study identified differences in in-hospital mortality based on the type of cardiac manifestations, with increased risk observed for patients with multiple cardiac involvement, cardiac arrest, and extracorporeal membrane oxygenation deployments.

Impact of Perioperative Tachydysrhythmias on Mortality and Length of Stay in Complete Repair of Tetralogy of Fallot: A Multicenter Retrospective Cohort Study from the Pediatric Health Information System.

INTRODUCTION: Tachydysrhythmias (TDS) frequently occur after complete repair of tetralogy of Fallot (TOF). However, not much is known about the effect of TDS on morbidity and mortality after TOF repair. We sought to assess the associations between TDS and mortality and morbidity after repair of TOF using a multicentre database. MATERIALS AND METHODS: We identified all children aged 0-5 years in the Pediatric Health Information System who underwent TOF repair between 2004 and 2015. Codes for TDS were used to identify cases. Outcome variables were inpatient mortality and total length of stay (LOS). Univariate and multiple logistic and linear regression analyses were used to identify the effects of multiple risk factors, including TDS, on mortality and LOS. RESULTS: A total of 7,749 patients met inclusion criteria, of which 1,493 (19%) had codes for TDS. There was no association between TDS and inpatient mortality. However, TDS were associated with 1.1 days longer LOS and accounted for 2% of the variation observed in LOS. CONCLUSION: After complete repair of TOF, TDS were not associated with mortality and appeared to have only a modest effect on LOS.

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.

Variable Presentation of COVID-19 in Pediatric Patients.

Infection by SARS-CoV-2 has led to disease referred to as coronavirus disease 2019 which, in children has been described as of multisystem inflammatory syndrome in children. Our experience demonstrates 2 distinct presentations of this syndrome which have different clinical courses and may have differing long-term outcomes.

Pediatric Cardiac Critical Care Transport and Palliative Care: A Case Series.

OBJECTIVE: To present our center's experience with terminal extubation in 3 palliative critical care home transports from the Pediatric Cardiac Intensive Unit. DESIGN: All cases were identified from our Cardiovascular intensive care unit ( CVICU). Patients were terminally ill children with no other surgical or medical option who were transported home between 2014 and 2018, for terminal extubation and end-of-life care according to their families' wishes. INTERVENTIONS: The patients were 7, 9 months, and 19 years; and they had very complex and chronic conditions. The families were approached by the CVICU staff during multidisciplinary meetings, where goals of care were established. Parental expectations were clarified, and palliative care team was involved, as well as home hospice was arranged pre transfer. The transfer process was discussed and all the needs were established. All patients had unstable medical conditions, with needs for transport for withdrawal of life support and death at home. Each case needed a highly trained team to support life while in transport. The need of these patients required coordination with home palliative care services, as well as community resources due to difficulty to get in their homes. CONCLUSIONS: Transportation of pediatric cardiac critical care patients for terminal extubation at home is a relatively infrequent practice. It is a feasible alternative for families seeking out of the hospital end-of-life care for their critically ill and technology dependent children. Our single-center experience supports the need for development of formal programs for end-of-life critical care transports.

Central Venous Catheter Utilization and Complications in the Pediatric Cardiac ICU: A Report From the Pediatric Cardiac Critical Care Consortium (PC4).

OBJECTIVES: Current central venous catheter utilization in patients within pediatric cardiac ICUs is not well elucidated. We aim to describe current use of central venous catheters in a multi-institutional cohort and to explore the prevalence and risk factors for central line-associated thrombosis and central line-associated bloodstream infections. DESIGN: Observational analysis. SETTING: Pediatric Cardiac Critical Care Consortium hospitals. PATIENTS: Hospitalizations with at least one cardiac ICU admission from October 2013 to July 2016. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: There were 17,846 hospitalizations and 69% included greater than or equal to one central venous catheter. Central venous catheter use was higher in younger patients (86% neonates). Surgical hospitalizations included at least one central venous catheter 88% of the time compared with 35% of medical hospitalizations. The most common location for central venous catheters was internal jugular (46%). Central venous catheters were in situ a median of 4 days (interquartile range, 2-10). There were 248 hospitalizations (2% overall, 1.8% medical, and 2.1% surgical) with at least one central line-associated thrombosis (271 total thromboses). Thrombosis was diagnosed at a median of 7 days (interquartile range, 4-14) after catheter insertion. There were 127 hospitalizations (1% overall, 1.4% medical, and 1% surgical) with at least one central line-associated bloodstream infection (136 total infections) with no association with catheter type or location. Central line-associated bloodstream infection was diagnosed at a median of 19 days (interquartile range, 8-36) after catheter insertion. Significant risk factors for central line-associated thrombosis and central line-associated bloodstream infection were younger age, greater surgical complexity, and total catheter days. CONCLUSIONS: Utilization of central venous catheters in pediatric cardiac ICUs differs according to indication for hospitalization. Although thrombosis and central line-associated bloodstream infection are infrequent complications of central venous catheter use in cardiac ICU patients, these events can have important short- and long-term consequences for patients. Total central venous catheter line days were the only modifiable risk factor identified. Future study must focus on understanding central venous catheter practices in high-risk patient subgroups that reduce the prevalence of thrombosis and central line-associated bloodstream infection.

Gastrostomy and Tracheostomy After Complete Repair of Tetralogy of Fallot in Children With 22q11.2 Deletion Syndrome.

OBJECTIVES: Caring for a child with gastrostomy and/or tracheostomy can cause measurable parental stress. It is generally known that children with 22q11.2 deletion syndrome are at greater risk of requiring gastrostomy or tracheostomy after heart surgery, although the magnitude of that risk after complete repair of tetralogy of Fallot has not been described. We sought to determine the degree to which 22q11.2 deletion is associated with postoperative gastrostomy and/or tracheostomy after repair of tetralogy of Fallot. DESIGN: Retrospective cohort study. SETTING: Pediatric Health Information System. PATIENTS: Children undergoing complete repair of tetralogy of Fallot (ventricular septal defect closure and relief of right ventricular outflow tract obstruction) from 2003 to 2016. Patients were excluded if they had pulmonary atresia, other congenital heart defects, and/or genetic diagnoses other than 22q11.2 deletion. MEASUREMENTS AND MAIN RESULTS: Two groups were formed on the basis of 22q11.2 deletion status. Outcomes were postoperative tracheostomy and postoperative gastrostomy. Bivariate analysis and Kaplan-Meier analysis at 150 days postoperatively were performed. There were 4,800 patients, of which 317 (7%) had a code for 22q11.2 deletion. There were no significant differences between groups for age at surgery or sex. Patients with 22q11.2 deletion had significantly higher rates of gastrostomy (18% vs 5%; p < 0.001) and higher rates of tracheostomy (7% vs 1%; p < 0.001); there was no difference for mortality. Kaplan-Meier analyses also showed higher rates of gastrostomy (p = 0.024) and tracheostomy (p = 0.037). CONCLUSIONS: The present study establishes rates of postoperative gastrostomy and tracheostomy in children with 22q11.2 deletion after complete repair of tetralogy of Fallot. These data are useful to clinicians for providing families with preoperative counseling.