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Infants with heart disease are at high risk of feeding difficulties and complications. Feeding practices amongst acute care cardiology units are not standardized. This study aims to describe feeding practices for infants at the time of discharge from a Pediatric Acute Care Cardiology Collaborative (PAC3) center and practice variation between centers. Discharge encounters for infants in the PAC3 registry between February 2019 and October 2021 were included. Nutrition type and feeding route at discharge were summarized with descriptive statistics and a modified bump plot. Center variation was assessed using funnel plots with control limits set at the 99.9% confidence interval from the group mean. A total of 15,414 encounters across 24 PAC3 centers were recorded from 8313 unique patients (median encounters 1, range 1-25). Nutrition at discharge consisted of standard formula in 8368 (54%), human milk in 6300 (41%), and elemental formula in 3230 (21%), either alone or in combination. Feeds were fortified to ≥ 24 kcal/oz in 12,359 (80%). Discharge supplemental tube feeding was present in 7353 (48%) encounters with 4643 (63%) receiving continuous feeds, 2144 (29%) bolus feeds, and 566 (8%) a combination. Funnel plots demonstrated variability in nutrition type and feeding route at discharge. Infants with heart disease commonly require high calorie nutrition and supplemental tube feedings at discharge. Feeding strategies at discharge vary widely between PAC3 centers. Collaborative approaches to identify best practices in feeding strategies are needed.

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BACKGROUND: Pain following surgery for cardiac disease is ubiquitous, and optimal management is important. Despite this, there is large practice variation. To address this, the Paediatric Acute Care Cardiology Collaborative undertook the effort to create this clinical practice guideline. METHODS: A panel of experts consisting of paediatric cardiologists, advanced practice practitioners, pharmacists, a paediatric cardiothoracic surgeon, and a paediatric cardiac anaesthesiologist was convened. The literature was searched for relevant articles and Collaborative sites submitted centre-specific protocols for postoperative pain management. Using the modified Delphi technique, recommendations were generated and put through iterative Delphi rounds to achieve consensus. RESULTS: 60 recommendations achieved consensus and are included in this guideline. They address guideline use, pain assessment, general considerations, preoperative considerations, intraoperative considerations, regional anaesthesia, opioids, opioid-sparing, non-opioid medications, non-pharmaceutical pain management, and discharge considerations. CONCLUSIONS: Postoperative pain among children following cardiac surgery is currently an area of significant practice variability despite a large body of literature and the presence of centre-specific protocols. Central to the recommendations included in this guideline is the concept that ideal pain management begins with preoperative counselling and continues through to patient discharge. Overall, the quality of evidence supporting recommendations is low. There is ongoing need for research in this area, particularly in paediatric populations.

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The importance of nutrition in managing critically ill infants with congenital heart disease (CHD) is foundational to optimizing short- and long-term health outcomes. Growth failure and malnutrition are common in infants with CHD. The etiology of growth failure in this population is often multifactorial and may be related to altered metabolic demands, compromised blood flow to the intestine leading to nutrient malabsorption, cellular hypoxia, inadequate energy intake, and poor oral-motor skills. A dearth of high-quality studies and gaps in previously published guidelines have led to wide variability in nutrition practices that are locally driven. This review provides recommendations from the nutrition subgroup of the Neonatal Cardiac Care Collaborative for best evidence-based practices in the provision of nutritional support in infants with CHD. The review of evidence and recommendations focused on 6 predefined areas of clinical care for a target population of infants <6 months with CHD admitted to the ICU or inpatient ward. These areas include energy needs, nutrient requirements, enteral nutrition, feeding practice, parenteral nutrition, and outcomes. Future progress will be directed at quality improvement efforts to optimize perioperative nutrition management with an increasing emphasis on individualized care based on nutritional status, cardiorespiratory physiology, state of illness, and other vulnerabilities.

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While outcomes for neonates with congenital heart disease have improved, it is apparent that substantial variability exists among centers with regard to the multidisciplinary approach to care for this medically fragile patient population. We endeavored to understand the landscape of neonatal cardiac care in the United States. A survey was distributed to physicians who provide neonatal cardiac care in the United States regarding (1) collaborative efforts in care of neonates with congenital heart disease (CHD); (2) access to neonatal cardiac training; and (3) barriers to the implementation of protocols for neonatal cardiac care. Responses were collected from 10/2018 to 6/2019. We received responses from 172 of 608 physicians (28% response rate) from 89 centers. When compared to responses received from physicians at low-volume centers (< 300 annual bypass cases), those at high-volume centers reported more involvement from the neurodevelopmental teams (58% vs. 29%; P = 0.012) and a standardized transition to outpatient care (68% vs. 52%; P = 0.038). While a majority of cardiothoracic surgery and anesthesiology respondents reported multidisciplinary involvement, less than half of cardiology and neonatology supported this statement. The most commonly reported obstacles to multidisciplinary engagement were culture (61.6%) and logistics (47.1%). Having a standardized neonatal cardiac curriculum for neonatal fellows was positively associated with the perception that multidisciplinary collaboration was "always" in place (53% vs. 40%; P = 0.09). There is considerable variation among centers in regard to personnel involved in neonatal cardiac care, related education, and perceived multidisciplinary collaboration among team members. The survey findings suggest the need to establish concrete standards for neonatal cardiac surgical programs, with ongoing quality improvement processes.

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Sedation in the cardiac intensive care unit (CICU) is necessary to keep critically ill infants safe and comfortable. However, long-term use of sedatives may be associated with adverse neurodevelopmental outcomes. We aimed to examine sedation practices in the CICU after the implementation of the Cornell Assessment of Pediatric Delirium (CAPD). We hypothesize the use of the CAPD would be associated with a decrease in sedative weans at CICU discharge. This is a single institution, retrospective cohort study. The study inclusion criteria were term infants, birthweight > 2.5 kg, cardiopulmonary bypass (CPB), and mechanical ventilation (MV) on postoperative day zero. During the study period, 50 and 35 patients respectively, met criteria pre- and post-implementation of CAPD screening. Our results showed a statistically significant increase in the incidence of sedative habituation wean at CICU discharge after CAPD implementation (24% vs. 45.7%, p = 0.036). There was a statistically significant increase in exposure to opiate (56% vs. 88.6%, p = 0.001) and dexmedetomidine infusions (52% vs 80%, p = 0.008), increased likelihood of clonidine use at CICU discharge (OR 9.25, CI 2.39-35.84), and increase in the duration of intravenous sedative infusions (8.1 days vs. 5.1 days, p = 0.04) No statistical difference was found in exposure to fentanyl (42% vs. 58.8%, p = 0.13) or midazolam infusions (22% vs. 25.7%, p = 0.691); and there was no change in benzodiazepine or opiate use at CICU discharge or dosage. The prevalence of delirium in the CAPD cohort was 92%. CAPD implementation in the CICU was associated with changes in sedation practices, specifically an increase in the use of dexmedetomidine, which possibly explains the increased clonidine weans at CICU discharge. This is the first report of the association between CAPD monitoring and changes in sedative practices. Multi-center prospective studies are recommended to evaluate sedative practices, delirium, and its effects on neurodevelopment.

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Hypoalbuminemia is associated with morbidity and mortality in critically ill children. In this multi-centre retrospective study, we aimed to determine normative values of serum albumin in neonates and infants with congenital heart disease, evaluate perioperative changes in albumin levels, and determine if low serum albumin influences post-operative outcomes. Consecutive eligible neonates and infants who underwent cardiac surgery with cardiopulmonary bypass at one of three medical centres, January 2012-August 2013, were included. Data on serum albumin levels from five data points (pre-operative, 0-24, 24-48, 48-72, 72 hours post-operative) were collected. Median pre-operative serum albumin level was 2.5 g/dl (IQR, 2.1-2.8) in neonates versus 4 g/dl (IQR, 3.5-4.4) in infants. Hypoalbuminemia was defined as <25th percentile of these values. A total of 203 patients (126 neonates, 77 infants) were included in the study. Post-operative hypoalbuminemia developed in 12% of neonates and 20% of infants; 97% occurred in the first 48 hours. In multivariable analysis, perioperative hypoalbuminemia was not independently associated with any post-operative morbidity. However, when analysed as a continuous variable, lower serum albumin levels were associated with increased post-operative morbidity. Pre-operative low serum albumin level was independently associated with increased odds of post-operative hypoalbuminemia (OR, 3.67; 95% CI, 1.01-13.29) and prolonged length of hospital stay (RR, 1.40; 95% CI, 1.08-1.82). Lower 0-24-hour post-operative serum albumin level was independently associated with an increased duration of mechanical ventilation (RR, 1.35; 95% CI, 1.12-1.64). Future studies should further assess hypoalbuminemia in this population, with emphasis on evaluating clinically meaningful cut-offs and possibly the use of serum albumin levels in perioperative risk stratification models.

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OBJECTIVES: The transthoracic intracardiac line placed in the right atrium provides a convenient access to the central venous system following cardiac surgery. However, it is associated with risks such as migration and bleeding. We conducted a retrospective study to determine whether position of transthoracic line with respect to site of exit from the chest makes a difference in the rate of complications. METHODS: All infants receiving a transthoracic intracardiac line in the right atrium following cardiac surgery between June 2012 and December 2015 were part of the study. A 3.5-Fr double-lumen umbilical venous catheter was placed directly into the right atrium. The lines exited the thorax either above in the suprasternal notch (upper transthoracic line) or below the diaphragm across the abdominal wall (lower transthoracic line). Patients were analysed for complications such as catheter migration, bleeding upon removal, atrial thrombus, line occlusion, premature removal and failed removal. RESULTS: A total of 131 patients received a transthoracic intracardiac line during the study period. Of the total patients, 88 patients received the upper transthoracic line and 43 patients received the lower transthoracic line. The upper transthoracic line was associated with significantly lower incidence of catheter migration (1% vs 14%) and this held by multivariable logistic regression, adjusting for age and duration of the line (P = 0.003). There was no difference in the rate of other complications including bleeding. CONCLUSIONS: The upper transthoracic line is associated with significantly lower incidence of catheter migration and offers a more optimum position for central access following cardiac surgery.

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BACKGROUND: Prolonged pleural effusion following Fontan operation is common and increases morbidity and hospital length of stay. Vasopressin (VP), a neurohypophysial hormone, has numerous effects on the cardiovascular system. The most notable is increased peripheral vascular resistance, but it may also reduce capillary leakage by tightening endothelial intercellular junctions and reducing capillary hydrostatic pressure We reviewed our experience with the perioperative administration of VP following Fontan operation. METHODS: We retrospectively reviewed the records of 62 consecutive patients who underwent Fontan operation from January 2004 to June 2014. In January 2010, VP was introduced as part of the standard perioperative management of patients undergoing Fontan operation at our center. For this retrospective observational study, patients were grouped according to the use (VP; N = 40) or nonuse (non-VP; N = 22) of VP (0.3-0.5 mU/kg/min) in the perioperative period. The primary end point analyzed was chest tube output. Secondary end points analyzed included fluid balance and length of hospital stay, with groups compared using Mann-Whitney U test. RESULTS: There was no hospital mortality. Median total chest tube output was 22 mL/kg in the VP group and 68 mL/kg in the non-VP group (P < .001). The median total duration of chest tube indwelling time was five days in the VP group and was 11 days in the non-VP group (P < .001). Median fluid balance on first postoperative day was 13 and 38 mL/kg, respectively (P < .001). Median hospital stay for VP and non-VP groups was 9 and 16 days, respectively (P = .002). CONCLUSIONS: The more recent group of patients undergoing Fontan operations, all of whom received VP perioperatively, had less chest tube output and shorter duration of chest tube drainage after the Fontan operation relative to the earlier patient group whose perioperative management did not include VP. They also experienced less positive fluid balance in the early postoperative period and shorter hospital length of stay than the patients from the earlier era.

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UNLABELLED: Introduction The optimal perioperative feeding strategies for neonates with CHD are unknown. In the present study, we describe the current feeding practices across a multi-institutional cohort. METHODS: Inclusion criteria for this study were as follows: all neonates undergoing cardiac surgery admitted to the cardiac ICU for ⩾24 hours preoperatively between October, 2013 and July, 2014 in the Pediatric Cardiac Critical Care Consortium registry. RESULTS: The cohort included 251 patients from eight centres. The most common diagnoses included the following: hypoplastic left heart syndrome (17%), coarctation/aortic arch hypoplasia (18%), and transposition of the great arteries (22%); 14% of the patients were <37weeks of gestational age. The median total hospital length of stay was 21 days (interquartile range (IQR) 14-35) and overall mortality was 8%. Preoperative feeding occurred in 133 (53%) patients. The overall preoperative feeding rates across centres ranged from 29 to 79%. Postoperative feeds started on median day 2 (IQR 1-4); for patients with hypoplastic left heart syndrome postoperative feeds started on median day 4. Postoperative feeds were initiated in 89 (35%) patients before extubation (range across centres: 21-61%). The median cardiac ICU discharge feeding volume was 108 cc/kg/day, varying across centres. The mean discharge weight was 280 g above birth weight, ranging from +100 to 430 g across centres. A total of 110 (44%) patients had discharge feeding tubes, ranging from 6 to 80% across centres, and 40/110 patients had gastrostomy/enterostomy tubes placed. In addition, eight (3.2%) patients developed necrotising enterocolitis - three preoperatively and five postoperatively. CONCLUSION: In this cohort, neonatal feeding practices and outcomes appear to vary across diagnostic groups and institutions. Only half of the patients received preoperative enteral nutrition; almost half had discharge feeding tubes. Multi-institutional collaboration is necessary to determine feeding strategies associated with best clinical outcomes.

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OBJECTIVE: Limited data exist for the use of bumetanide continuous infusions in children. The purpose of this study was to evaluate the use of bumetanide continuous infusions in critically ill pediatric patients. DESIGN: This study was an institutional review board approved, single-center, retrospective chart review of 95 patients. Dosing practices were described by rate (µg/kg/hr), duration (days), and previous diuretic use. Efficacy, determined by ability to achieve negative fluid balance and time to reach negative fluid balance, was assessed at 12, 24, and 48 hours. Safety was evaluated based on prevalence of adverse drug reactions. Adverse drug reactions were predefined as serum potassium concentration less than 3 mEq/L, serum chloride concentration less than 90 mEq/L, serum carbon dioxide concentration greater than 35 mEq/L, and serum creatinine increased greater than 1.5 times baseline and above patient-specific normal range. SETTING: Le Bonheur Children's Hospital, Memphis, TN. PATIENTS: Critically ill patients who are 18 years old or younger and received bumetanide continuous infusions. A total of 95 patients were included. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The mean dose of bumetanide was 5.7 ± 2.2 µg/kg/hr (1-10 µg/kg/hr) with a median duration of 3.3 days (0.3-18.5). The total percentage of patients achieving negative fluid balance by 48 hours was 76% with 54% of patients reaching negative fluid balance within 12 hours. CONCLUSIONS: This study showed that a bumetanide dose of 5.7 µg/kg/hr was effective in achieving negative fluid balance in the majority of critically ill pediatric patients. Additionally, bumetanide appears to be a safe loop diuretic for use as a continuous infusion at the doses described in critically ill pediatric patients.

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This study aimed to assess the impact of integrating a simulation-based education module into an extracorporeal membrane oxygenation (ECMO) curriculum on novice learners and to test the duration of time that skills obtained during this training exercise were retained. The authors hypothesized that multidisciplinary, simulation-based ECMO training would improve comfort and confidence levels among participants. An ECMO training curriculum was developed that incorporated in situ simulation modules to train multidisciplinary health care professionals involved in the management of patients receiving ECMO in the pediatric cardiac intensive care unit (PCICU). During the simulation, a team was assembled similar to the one that would staff the PCICU during a routine workday. Pre- and postparticipation questionnaires were used to determine the effects on the knowledge, ability, and confidence level of the participants. The participants were required to repeat the simulation test within 6-8 months. The study enrolled 26 providers (10 fellow physicians, 12 nurses and nurse practitioners, 4 respiratory therapists). All except one had no previous training in the management of ECMO. Of the 26 participants, 24 passed the initial written and practical tests. One participant failed the written test, whereas another failed the practical test. All the responding participants scored the didactic and scenarios education as useful, at 4 or higher (5 = very useful), in improving their perception of their overall knowledge and their ability to perform the required critical performance criteria on simulated ECMO. The 20 participants who appeared for the 6 month follow-up visit to assess maintenance of competency skills demonstrated success with simulated ECMO emergencies. All four questionnaires were completed by 18 participants. Simulation-based training is an effective method of improving knowledge, ability, and confidence levels among novice ECMO specialists and physician trainees. Further research is needed to assess real-time demonstration of skills retention during ECMO emergencies.

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Findings show that simulation-based team training (SBTT) is effective at increasing teamwork skills. Postpediatric cardiac surgery cardiac arrest (PPCS-CA) is a high-risk clinical situation with high morbidity and mortality. Whereas adult guidelines managing cardiac arrest after cardiac surgery are available, little exists for pediatric cardiac surgery. The authors developed a post-PPCS-CA algorithm and used SBTT to improve identification and management of PPCS-CA in the pediatric cardiovascular intensive care unit. Their goal was to determine whether participation aids in improving teamwork, confidence, and communication during these events. The authors developed a simulation-based training course using common postcardiac surgical emergency scenarios with specific learning objectives. Simulated scenarios are followed by structured debriefings. Participants were evaluated based on critical performance criteria, key elements in the PPCS-CA algorithm, and Team Strategies and Tools to Enhance Performance and Patient Safety (Team STEPPS) principles. Surveys performed before, immediately after, and 3 months after participation evaluated perception of skill, knowledge, and confidence. The study had 37 participants (23 nurses, 5 cardiology/critical care trainees, 5 respiratory therapists, and 4 noncategorized subjects). Confidence and skill in the roles of team leader, advanced airway management, and cardioversion/defibrillation were increased significantly (p < 0.05) immediately after training and 3 months later. A significant increase (p < 0.05) also was observed in the use of Team STEPPS concepts immediately after training and 3 months later. This study showed SBTT to be effective in improving communication and increasing confidence among members of a multidisciplinary team during crisis scenarios. Thus, SBTT provides an excellent tool for teaching and implementing new processes.

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OBJECTIVES: Incisions in the left ventricle have previously been associated with increased mortality and morbidity, particularly in infants. In order to determine whether this assumption is still true in the current era, we reviewed our recent experience with apical left ventriculotomy in neonates and infants. METHODS: The records of five consecutive patients requiring a left ventriculotomy between 2007 and 2010 were reviewed. Weight and age ranged from 2.6 to 16 kilograms and 5 days to 2 years. The diagnoses were three multiple ventricular septal defects, one rhabdomyoma, and one apical aneurysm. The primary end point was left ventricular ejection fraction, with other end points being intensive care unit length of stay, time to extubation, inotrope requirement, arrhythmias, and mitral valve function. RESULTS: There were no early or late deaths. Although lower than their preoperative values, early postoperative ejection fractions were greater than 50% in all patients. Two patients required no inotropes, and 3 required only minimal support. Hospital length of stay was 9 ± 7 days for multiple ventricular septal defect patients, with intensive care unit stays of 2 to 5 days. There were no postoperative arrhythmias requiring pharmacological therapy, and one patient had a significant reduction in mitral insufficiency postoperatively. CONCLUSIONS: Based on our experience, we believe that an apical left ventriculotomy does not significantly impair left ventricular function even in small infants, and is not associated with significant morbidity, based on short-term follow-up. Although the long-term effects are still unknown, early results suggest that a left ventriculotomy may safely be used when alternative approaches are inadequate for complex cardiac defects.

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OBJECTIVE: To explore clinical outcomes and secondary diagnoses present at discharge for infants born with hypoplastic left heart syndrome (HLHS), from a national perspective. METHODS: We examined hospitalizations for infants < or =30 days of age who were born with HLHS, using hospital discharge data from the 1997 Kids Inpatient Database. To explore treatment choices, clinical outcomes, and resource use, we used International Classification of Diseases, 9th Revision, Clinical Modification diagnostic and procedure codes to classify discharges according to type of surgical intervention versus no surgical intervention. To investigate outcomes in more detail, we identified secondary diagnoses noted at discharge, using International Classification of Diseases, 9th Revision, Clinical Modification codes, and stratified results according to type of surgical intervention. RESULTS: Of a total of 550 patients with HLHS, 234 underwent the Norwood procedure, 17 underwent orthotopic heart transplantation, and 106 died in the hospital with no reported surgical intervention. Although we found no demographic variables to be significantly associated with the type of treatment received, discharged patients who died without surgical intervention were significantly more likely to have received care in hospitals identified as small (odds ratio [OR]: 1.5; 95% confidence interval [CI]: 1.03-3.1) or not children's hospitals (OR: 2.02; 95% CI: 1.13-3.6). Secondary diagnoses of cardiac arrest (OR: 2.0; 95% CI: 1.1-3.4) and seizures (OR: 2.6; 95% CI: 1.2-5.5) occurred more frequently in orthotopic heart transplantation cases than in Norwood procedure cases. CONCLUSIONS: These data from a national perspective reflect outcomes of infants with HLHS during a time when rates of initial survival after surgical intervention were considered to be improved. These findings may be useful to clinicians when they are considering and recommending initial medical and surgical strategies currently being proposed for the treatment of HLHS.

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