Bleeding and Thrombosis With Pediatric Extracorporeal Life Support: A Roadmap for Management, Research, and the Future From the Pediatric Cardiac Intensive Care Society: Part 1.

OBJECTIVES: To make practical and evidence-based recommendations on improving understanding of bleeding and thrombosis with pediatric extracorporeal life support and to make recommendations for research directions. DATA SOURCES: Evaluation of literature and consensus conferences of pediatric critical care and extracorporeal life support experts. STUDY SELECTION: A team of 10 experts with pediatric cardiac and extracorporeal membrane oxygenation experience and expertise met through the Pediatric Cardiac Intensive Care Society to review current knowledge and make recommendations for future research to establish "best practice" for anticoagulation management related to extracorporeal life support. DATA EXTRACTION/SYNTHESIS: The first of a two-part white article focuses on clinical understanding and limitations of medications in use for anticoagulation, including novel medications. For each medication, limitations of current knowledge are addressed and research recommendations are suggested to allow for more definitive clinical guidelines in the future. CONCLUSIONS: No consensus on best practice for anticoagulation exists. Structured scientific evaluation to answer questions regarding anticoagulant medication and bleeding and thrombotic events should occur in multicenter studies using standardized approaches and well-defined endpoints. Outcomes related to need for component change, blood product administration, healthcare outcome, and economic assessment should be incorporated into studies. All centers should report data on patients receiving extracorporeal life support to a registry. The Extracorporeal Life Support Organization registry, designed primarily for quality improvement purposes, remains the primary and most successful data repository to date.

Bleeding and Thrombosis With Pediatric Extracorporeal Life Support: A Roadmap for Management, Research, and the Future From the Pediatric Cardiac Intensive Care Society: Part 2.

OBJECTIVES: To make recommendations on improving understanding of bleeding and thrombosis with pediatric extracorporeal life support including future research directions. DATA SOURCES: Evaluation of literature and consensus conferences of pediatric critical care and extracorporeal life support experts. STUDY SELECTION: A team of 10 experts with pediatric cardiac and extracorporeal membrane oxygenation experience and expertise met through the Pediatric Cardiac Intensive Care Society to review current knowledge and make recommendations for future research to establish "best practice" for anticoagulation management related to extracorporeal life support. DATA EXTRACTION/DATA SYNTHESIS: This white paper focuses on clinical understanding and limitations of current strategies to monitor anticoagulation. For each test of anticoagulation, limitations of current knowledge are addressed and future research directions suggested. CONCLUSIONS: No consensus on best practice for anticoagulation monitoring exists. Structured scientific evaluation to answer questions regarding anticoagulation monitoring and bleeding and thrombotic events should occur in multicenter studies using standardized approaches and well-defined endpoints. Outcomes related to need for component change, blood product administration, healthcare outcome, and economic assessment should be incorporated into studies. All centers should report data on patient receiving extracorporeal life support to a registry.

Accurate Prediction of Congenital Heart Surgical Length of Stay Incorporating a Procedure-Based Categorical Variable.

OBJECTIVES: There is increasing demand for the limited resource of Cardiac ICU care. In this setting, there is an expectation to optimize hospital resource use without restricting care delivery. We developed methodology to predict extended cardiac ICU length of stay following surgery for congenital heart disease. DESIGN: Retrospective analysis by multivariable logistic regression of important predictive factors for outcome of postoperative ICU length of stay greater than 7 days. SETTING: Cardiac ICU at Boston Children's Hospital, a large, pediatric cardiac surgical referral center. PATIENTS: All patients undergoing congenital heart surgery at Boston Children's Hospital from January 1, 2010, to December 31, 2015. INTERVENTIONS: No study interventions. MEASUREMENTS AND MAIN RESULTS: The patient population was identified. Clinical variables and Congenital Heart Surgical Stay categories were recorded based on surgical intervention performed. A model was built to predict the outcome postoperative ICU length of stay greater than 7 days at the time of surgical intervention. The development cohort included 4,029 cases categorized into five Congenital Heart Surgical Stay categories with a C statistic of 0.78 for the outcome ICU length of stay greater than 7 days. Explanatory value increased with inclusion of patient preoperative status as determined by age, ventilator dependence, and admission status (C statistic = 0.84). A second model was optimized with inclusion of intraoperative factors available at the time of postoperative ICU admission, including cardiopulmonary bypass time and chest left open (C statistic 0.87). Each model was tested in a validation cohort (n = 1,008) with equivalent C statistics. CONCLUSIONS: Using a model comprised of basic patient characteristics, we developed a robust prediction tool for patients who will remain in the ICU longer than 7 days after cardiac surgery, at the time of postoperative ICU admission. This model may assist in patient counseling, case scheduling, and capacity management. Further examination in external settings is needed to establish generalizability.

Physiologic effects of delayed sternal closure following stage 1 palliation.

BACKGROUND: Following stage 1 palliation, delayed sternal closure may be used as a technique to enhance thoracic compliance but may also prolong the length of stay and increase the risk of infection. METHODS: We reviewed all neonates undergoing stage 1 palliation at our institution between 2010 and 2017 to describe the effects of delayed sternal closure. RESULTS: During the study period, 193 patients underwent stage 1 palliation, of whom 12 died before an attempt at sternal closure. Among the 25 patients who underwent primary sternal closure, 4 (16%) had sternal reopening within 24 hours. Among the 156 infants who underwent delayed sternal closure at 4 [3,6] days post-operatively, 11 (7.1%) had one or more failed attempts at sternal closure. Patients undergoing primary sternal closure had a shorter duration of mechanical ventilation and intensive care unit length of stay. Patients who failed delayed sternal closure had a longer aortic cross-clamp time (123±42 versus 99±35 minutes, p=0.029) and circulatory arrest time (39±28 versus 19±17 minutes, p=0.0009) than those who did not fail. Failure of delayed sternal closure was also closely associated with Technical Performance Score: 1.3% of patients with a score of 1 failed sternal closure compared with 18.9% of patients with a score of 3 (p=0.0028). Among the haemodynamic and ventilatory parameters studied, only superior caval vein saturation following sternal closure was different between patients who did and did not fail sternal closure (30±7 versus 42±10%, p=0.002). All patients who failed sternal closure did so within 24 hours owing to hypoxaemia, hypercarbia, or haemodynamic impairment. CONCLUSION: When performed according to our current clinical practice, sternal closure causes transient and mild changes in haemodynamic and ventilatory parameters. Monitoring of SvO2 following sternal closure may permit early identification of patients at risk for failure.

Training fellows in paediatric cardiology: the Harvard experience.

The Fellowship Program of the Department of Cardiology at Boston Children's Hospital seeks to train academically oriented leaders in clinical care and laboratory and clinical investigation of cardiovascular disease in the young. The core clinical fellowship involves 3 years in training, comprising 24 months of clinical rotations and 12 months of elective and research experience. Trainees have access to a vast array of research opportunities - clinical, basic, and translational. Clinical fellows interested in basic science may reverse the usual sequence and start their training in the laboratory, deferring clinical training for 1 or more years. An increasing number of clinical trainees apply to spend a fourth year as a senior fellow in one of the subspecialty areas of paediatric cardiology. From the founding of the Department to the present, we have maintained a fundamental and unwavering commitment to training and education in clinical care and research in basic science and clinical investigation, as well as to the training of outstanding young clinicians and investigators.

A Pediatric Cardiology Fellowship Boot Camp improves trainee confidence.

Introduction New paediatric cardiology trainees are required to rapidly assimilate knowledge and gain clinical skills to which they have limited or no exposure during residency. The Pediatric Cardiology Fellowship Boot Camp (PCBC) at Boston Children's Hospital was designed to provide incoming fellows with an intensive exposure to congenital cardiac pathology and a broad overview of major areas of paediatric cardiology practice. METHODS: The PCBC curriculum was designed by core faculty in cardiac pathology, echocardiography, electrophysiology, interventional cardiology, exercise physiology, and cardiac intensive care. Individual faculty contributed learning objectives, which were refined by fellowship directors and used to build a programme of didactics, hands-on/simulation-based activities, and self-guided learning opportunities. RESULTS: A total of 16 incoming fellows participated in the 4-week boot camp, with no concurrent clinical responsibilities, over 2 years. On the basis of pre- and post-PCBC surveys, 80% of trainees strongly agreed that they felt more prepared for clinical responsibilities, and a similar percentage felt that PCBC should be offered to future incoming fellows. Fellows showed significant increase in their confidence in all specific knowledge and skills related to the learning objectives. Fellows rated hands-on learning experiences and simulation-based exercises most highly. CONCLUSIONS: We describe a novel 4-week-long boot camp designed to expose incoming paediatric cardiology fellows to the broad spectrum of knowledge and skills required for the practice of paediatric cardiology. The experience increased trainee confidence and sense of preparedness to begin fellowship-related responsibilities. Given that highly interactive activities were rated most highly, boot camps in paediatric cardiology should strongly emphasise these elements.

High-Fidelity Cleft Simulation Maintains Improvements in Performance and Confidence: A Prospective Study.

OBJECTIVE: High-fidelity simulation has a growing role in plastic surgical education. This study tests the hypothesis that cleft lip repair simulation followed by structured debriefing improves performance and self-confidence and that gains are maintained. DESIGN: Prospective, single-blinded interventional study with repeated measures. Trainees performed cleft lip repair on a high-fidelity simulator followed by debriefing, immediately completed a second repair, and returned 3 months later for a third session. Anonymized simulation videos were rated using the modified Objective Structured Assessment of Technical Skills (OSATS) and the Unilateral Cleft Lip Repair competency assessment tool (UCLR). Self-assessed cleft lip knowledge/confidence and procedural self-confidence were surveyed after each simulation. SETTING: Boston Children's Hospital, a tertiary care academic hospital in Boston, MA, USA. PARTICIPANTS: All trainees rotating through the study setting were eligible. Twenty-six participated; 21 returned for follow-up. RESULTS: Significant improvements (p < 0.05) occurred between the first and second simulations for OSATS, UCLR, and procedural self-confidence. Significant improvement occurred between the second and third simulations cleft lip knowledge/confidence. Compared to the first simulation, improvements were maintained at the third simulation for all variables. Training level moderately correlated with score for UCLR for the first simulation (r = 0.55, p < 0.01), deteriorated somewhat with the second (r = 0.35, p = 0.08), and no longer corelated by the third (r = 0.02, p = 0.92). CONCLUSIONS: Objective performance and subjective self-assessed knowledge and confidence improve with high-fidelity simulation plus structured debriefing and improvement is maintained. Differences in procedure-specific performance seen with increasing training level are reduced with simulation, suggesting it may accelerate knowledge and skill acquisition.

A Multimodal Approach to Training Coronavirus Disease (COVID-19) Processes Across Four Intensive Care Units.

Background: Coronavirus disease (COVID-19) required innovative training strategies for emergent aerosol generating procedures in intensive care units. This manuscript summarizes institutional operationalization of COVID-specific training, standardized across four intensive care units. Methods & Results: An interdisciplinary team collaborated with the Simulator Program and OpenPediatrics refining logistics using process maps, walkthroughs and simulation. A multimodal approach to information dissemination, high-volume team training in modified resuscitation practices and technical skill acquisition included instructional videos, training superusers, small-group simulation using a flipped classroom approach with rapid cycle deliberate practice, interactive webinars, and cognitive aids. Institutional data on application of this model are presented. Conclusion: Success was founded in interdisciplinary collaboration, resource availability and institutional buy in.

Transcatheter Palliation With Pulmonary Artery Flow Restrictors in Neonates With Congenital Heart Disease: Feasibility, Outcomes, and Comparison With a Historical Hybrid Stage 1 Cohort.

BACKGROUND: Neonates with complex congenital heart disease and pulmonary overcirculation have been historically treated surgically. However, subcohorts may benefit from less invasive procedures. Data on transcatheter palliation are limited. METHODS: We present our experience with pulmonary flow restrictors (PFRs) for palliation of neonates with congenital heart disease, including procedural feasibility, technical details, and outcomes. We then compared our subcohort of high-risk single ventricle neonates palliated with PFRs with a similar historical cohort who underwent a hybrid Stage 1. Cox regression was used to evaluate the association between palliation strategy and 6-month mortality. RESULTS: From 2021 to 2023, 17 patients (median age, 4 days; interquartile range [IQR], 2-8; median weight, 2.5 kilograms [IQR, 2.1-3.3]) underwent a PFR procedure; 15 (88%) had single ventricle physiology; 15 (88%) were high-risk surgical candidates. All procedures were technically successful. At a median follow-up of 6.2 months (IQR, 4.0-10.8), 13 patients (76%) were successfully bridged to surgery (median time since PFR procedure, 2.6 months [IQR, 1.1-4.4]; median weight, 4.9 kilograms [IQR, 3.4-5.8]). Pulmonary arteries grew adequately for age, and devices were easily removed without complications. The all-cause mortality rate before target surgery was 24% (n=4). Compared with the historical hybrid stage 1 cohort (n=23), after adjustment for main confounding (age, weight, intact/severely restrictive atrial septum or left ventricle to coronary fistulae), the PFR procedure was associated with a significantly lower all-cause 6-month mortality risk (adjusted hazard ratio, 0.26 [95% CI, 0.08-0.82]). CONCLUSIONS: Transcatheter palliation with PFR is feasible, safe, and represents an effective strategy for bridging high-risk neonates with congenital heart disease to surgical palliation, complete repair, or transplant while allowing for clinical stabilization and somatic growth.

Management and outcomes in pediatric patients presenting with acute fulminant myocarditis.

OBJECTIVE: To investigate factors associated with mechanical circulatory support and survival in patients with acute fulminant myocarditis (AFM). STUDY DESIGN: Retrospective cohort of AFM patients admitted to the cardiac intensive care unit during 1996-2008. AFM was defined as distinct onset of symptoms ≤14 days before admission, rapid-onset cardiogenic shock, and normal left ventricular size. Demographic and physiological variables were compared between patients treated with extracorporeal membrane oxygenation (ECMO) and those who were not and between survivors and nonsurvivors. RESULTS: Twenty patients (median age 12.7 years) met inclusion criteria. Seventeen patients (85%) survived to hospital discharge. One underwent heart transplantation. Ten (50%) patients required ECMO, and 7 (70%) of these survived. On admission, patients requiring ECMO had elevated lactate (9 vs 1 mmol/L), creatinine (0.8 vs 0.6 mg/dL), and aspartate aminotransferase (256 vs 35 IU/L) (all P < .05) and a trend towards increased incidence of dysrhythmias (80% vs 30%, P = .07). During hospitalization, non-survivors had higher peak lactate (10 vs 3 mmol/L), creatinine (1.5 vs 0.8 mg/dL), and aspartate aminotransferase (3007 vs 156 IU/L) (all P < .05) compared with survivors. CONCLUSIONS: Patients with AFM with end organ dysfunction or arrhythmias on admission may require mechanical circulatory support. The transplant-free survival rate in this critically ill cohort was excellent (80%).

Outcomes and risk factors for mortality in premature neonates with critical congenital heart disease.

We sought to describe contemporary outcomes and identify risk factors for hospital mortality in premature neonates with critical congenital heart disease who were referred for early intervention. Neonates who were born before 37 weeks' gestation with critical congenital heart disease and admitted to our institution from 2002 to 2008 were included in this retrospective cohort study. Critical congenital heart disease was defined as a defect requiring surgical or transcatheter cardiac intervention or a defect resulting in death within the first 28 days of life. Logistic regression analyses were performed to identify risk factors for mortality before hospital discharge. The study included 180 premature neonates, of whom 37 (21%) died during their initial hospitalization, including 6 (4%) before cardiac intervention and 31 (17%) after cardiac intervention. For the 174 patients undergoing cardiac intervention, independent risk factors for mortality were a 5 min Apgar score ≤ 7, need for preintervention mechanical ventilation, and Risk Adjustment in Congenital Heart Surgery category ≥ 4 or not assignable. Mortality for premature infants with critical congenital heart disease who are referred for early intervention remains high. Patients with lower Apgar scores who receive preintervention mechanical ventilation and undergo more complex procedures are at greatest risk.

The relationship between inflammatory activation and clinical outcome after infant cardiopulmonary bypass.

BACKGROUND: Cardiopulmonary bypass (CPB) induces a systemic inflammatory response. The magnitude and consequences in infants remain unclear. We assessed the relationship between inflammatory state and clinical outcomes in infants undergoing CPB. METHODS: Plasma concentrations of interleukin (IL)-6, IL-8, IL-10, tumor necrosis factor α, IL-1ß, and C-reactive protein (CRP) were measured pre-CPB and immediately post-CPB, and at 6, 12, and 24 hours post-CPB in infants ≤9 months old. Perioperative clinical data were collected prospectively. RESULTS: Diagnoses of 93 patients included transposition of the great arteries (40), tetralogy of Fallot (28), ventricular septal defect (21), truncus arteriosus (2), and complete atrioventricular canal (2). The median age was 37 days (range = 2 to 264). Pre-CPB IL-6 and CRP were higher in younger infants but were not associated with postoperative inflammatory mediator concentrations or measured clinical outcomes. IL-6 increased post-CPB (median 3.2 pg/mL pre-CPB, 24.2 post-CPB, 95.4 at 6 hours, and 90.3 at 24 hours; all P < 0.001). CRP increased post-CPB, peaking at 24 hours (median 27.5 at 24 hours, 0.3 pre-CPB; P < 0.001). IL-10 and IL-8 increased immediately post-CPB. After adjusting for age and diagnosis, postoperative IL-6 and IL-8 correlated with intensive care unit length of stay and postoperative blood product administration and, for IL-8, 24-hour lactate. CONCLUSIONS: Greater preoperative cytokine and CRP production in younger infants did not correlate with postoperative outcomes; correlation between postoperative inflammatory mediator production and clinical course was statistically significant but clinically modest. We conclude that in infants undergoing low-to-moderate-complexity cardiac surgery in a single high-volume center, the contribution of inflammatory mediator production to postoperative morbidity is relatively limited.

Management of suspected myocarditis during critical-care transport.

Myocarditis and malignant dysrhythmias are unusual presentations in pediatric patients. We report a series of 4 patients with myocarditis and arrhythmia who presented to community emergency departments and were transported to a pediatric tertiary-care center. Three of the patients required extracorporeal life support. We discuss considerations for stabilization and transport: airway and ventilation, hemodynamic support, induction and sedation medication choices, transport decisions, and the traits of an ideal receiving center.

Acute coronary artery obstruction following surgical repair of congenital heart disease.

OBJECTIVES: Acute coronary artery obstruction is a rare complication of congenital heart disease surgery but imposes a high burden of morbidity and mortality. Previous case series have described episodes in specific congenital heart lesions or surgical repairs but have not examined the complication in all-comers to congenital heart surgery. We hypothesize that shorter time from a clinically recognized postoperative sentinel event suggestive of coronary ischemia to diagnosis of coronary obstruction is associated with improved clinical outcomes. METHODS: This was a single-center, retrospective review of patients diagnosed with acute coronary artery obstruction by angiography following surgical repair of congenital heart disease between January 2000 and June 2016. RESULTS: In total, 34 patients were identified. The most common procedures associated with coronary artery obstruction were the Norwood procedure, arterial switch operation, and aortic valve repair/replacement. In total, 79% required mechanical circulatory support, 41% died, and 27% were listed for heart transplant. Patients who died or were listed for heart transplant had longer median sentinel-event-to-cardiac-catheterization time (28 [6-168] hours vs 10 [3-56] hours, P = .001), and longer median sentinel-event-to-intervention time (32 [11-350] hours vs 13 [5-59] hours, P = .003). Patients with hypoplastic left heart syndrome were at greater risk of death or transplant listing (odds ratio, 9.23, P = .03). CONCLUSIONS: Time from clinically relevant postoperative sentinel event to diagnosis of coronary artery obstruction by angiography was associated with transplant-listing-free survival. Clinicians should maintain a high index of suspicion for coronary obstruction and consider early catheterization and coronary angiography for patients in whom post-operative coronary compromise is suspected.

Applying Educational Theory to Interdisciplinary Education in Pediatric Cardiac Critical Care.

At the 14th Annual International Meeting for the Pediatric Cardiac Intensive Care Society, the authors presented a simulation workshop for junior multidisciplinary providers focused on cardiopulmonary interactions. We provide an overview of educational theories of particular relevance to curricular design for simulation-based or enhanced activities. We then demonstrate how these theories are applied to curriculum development for individuals to teams and for novice to experts. We review the role of simulation in cardiac intensive care education and the education theories that support its use. Finally, we demonstrate how a conceptual framework, SIMZones, can be applied to design effective simulation-based teaching.

Extracorporeal membrane oxygenation in congenital heart disease.

This review article will discuss the indications for and outcomes of neonates with congenital heart disease who receive extracorporeal membrane oxygenation (ECMO) support. Most commonly, ECMO is used as a perioperative bridge to recovery or temporary support for those after cardiac arrest or near arrest in patients with congenital or acquired heart disease. What had historically been considered a contraindication to ECMO, is evolving and more of the sickest and most complicated babies are cared for on ECMO. Given that, it is imperative for aggressive survellience for long-term morbidity in survivors, particularly neurodevelopmental outcomes.

Simulation training improves team dynamics and performance in a low-resource cardiac intensive care unit.

INTRODUCTION: Although simulation training has been utilized quite extensively in highincome medical environments, its feasibility and effect on team performance in lowresource pediatric Cardiac Intensive Care Unit (CICU) environments has not been demonstrated. We hypothesized that lowfidelity simulationbased crisis resource management training would lead to improvements in team performance in such settings. METHODS: In this prospective observational study, the effect of simulation on team dynamics and performance was assessed in 23 healthcare providers in a pediatric CICU in Southeast Asia. A 5day training program was utilized consisting of various didactic sessions and simulation training exercises. Improvements in team dynamics were assessed using participant questionnaires, expert evaluations, and video analysis of time to intervention and frequency of closedloop communication. RESULTS: In subjective questionnaires, participants noted significant (P < 0.05) improvement in team dynamics and performance over the training period. Video analysis revealed a decrease in time to intervention and significant (P < 0.05) increase in frequency of closedloop communication because of simulation training. CONCLUSIONS: This study demonstrates the feasibility and effectiveness of simulationbased training in improving team dynamics and performance in lowresource pediatric CICU environments, indicating its potential role in eliminating communication barriers in these settings.