Analysis of Perioperative and Long-Term Outcomes Among Presentations of Anomalous Left Coronary Artery from the Pulmonary Artery Diagnosed Beyond Infancy Versus During Infancy.

Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA) typically presents in infancy; however, there are cases of patients who survive the infant period and present later in life. We aimed to characterize patients with late ALCAPA diagnoses and to assess perioperative and functional outcomes. A retrospective chart review of patients who underwent ALCAPA repair between 1996 and 2020 at Boston Children's Hospital was performed. This cohort was divided into early ALCAPA (< 1 year) and late ALCAPA (≥ 1 year) groups. Perioperative data were collected. Longitudinal functional assessments were made by echocardiography, exercise stress test, and cardiac magnetic resonance imaging. The median age of the late ALCAPA group was 7.6 years with 25% (6/24) of patients over 18 years. The late ALCAPA group was more likely to present as an incidental finding (63%) and required less preoperative intervention compared to the early group. On preoperative echocardiogram, the late ALCAPA group had less moderate or severe mitral regurgitation (16.7% vs 62%, p < 0.001) or left ventricular dysfunction (16.7% vs 89%, p < 0.001) compared to the early group. Reoperation was uncommon, and both groups demonstrated almost complete resolution of mitral regurgitation and left ventricular dysfunction over time. There are important differences between late and early ALCAPA subtypes. Revascularization results in excellent outcomes in both early and late groups, but long-term surveillance of ALCAPA patients is warranted as they may have functional deficits after repair.

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.

Echocardiographic predictors of neonatal illness severity in fetuses with critical left heart obstruction with intact or restrictive atrial septum.

BACKGROUND: Neonates with critical left heart obstruction and intact atrial septum (IAS) or restrictive atrial septum (RAS) are at risk for hypoxia within hours of birth and remain a group at high risk for mortality. METHODS: Prenatally diagnosed fetuses with critical left heart obstruction and IAS or RAS with follow-up from January 1, 2005, to February 14, 2017, were included. Primary outcome was a composite measure of severe neonatal illness (pH < 7.15, venous pH < 7.10, bicarbonate < 16 mmol/L, lactic acid > 5 mmol/L, or median oxygen saturation < 60% within 2 hours of birth). RESULTS: Of 68 live born fetuses, 52 (76.5%) had hypoplastic left heart syndrome, 14 (20.5%) had critical aortic stenosis, and two (3%) had complex anatomy with mitral stenosis/atresia. There were 27 (39.7%) fetuses with IAS and 41 (60.3%) with RAS. Severe neonatal illness was present in 36 (52.9%). The strongest discriminators for severe neonatal illness were a pulmonary vein A:R VTI ≤ 2.7 (P < 0.001, AUC 0.93) and larger pulmonary vein diameter (P = 0.025, AUC 0.77). A:R VTI ≤ 2.7 predicted death or transplant (log-rank P = 0.03). CONCLUSIONS: In neonates with hypoplastic left heart syndrome and IAS or RAS, A:R VTI ≤ 2.7 is predictive of severe neonatal instability. This threshold can help guide resource planning, delivery management, and improve fetal intervention criteria.

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.

Revealing Adaptive Management of Environmental Flows.

Managers of land, water, and biodiversity are working with increasingly complex social ecological systems with high uncertainty. Adaptive management (learning from doing) is an ideal approach for working with this complexity. The competing social and environmental demands for water have prompted interest in freshwater adaptive management, but its success and uptake appear to be slow. Some of the perceived "failure" of adaptive management may reflect the way success is conceived and measured; learning, rarely used as an indicator of success, is narrowly defined when it is. In this paper, we document the process of adaptive flow management in the Edward-Wakool system in the southern Murray-Darling Basin, Australia. Data are from interviews with environmental water managers, document review, and the authors' structured reflection on their experiences of adaptive management and environmental flows. Substantial learning occurred in relation to the management of environmental flows in the Edward-Wakool system, with evidence found in planning documents, water-use reports, technical reports, stakeholder committee minutes, and refereed papers, while other evidence was anecdotal. Based on this case, we suggest it may be difficult for external observers to perceive the success of large adaptive management projects because evidence of learning is dispersed across multiple documents, and learning is not necessarily considered a measure of success. We suggest that documentation and sharing of new insights, and of the processes of learning, should be resourced to facilitate social learning within the water management sector, and to help demonstrate the successes of adaptive management.

Adaptive Management of Environmental Flows.

Adaptive management enables managers to work with complexity and uncertainty, and to respond to changing biophysical and social conditions. Amid considerable uncertainty over the benefits of environmental flows, governments are embracing adaptive management as a means to inform decision making. This Special Issue of Environmental Management presents examples of adaptive management of environmental flows and addresses claims that there are few examples of its successful implementation. It arose from a session at the 11th International Symposium on Ecohydraulics held in Australia, and is consequently dominated by papers from Australia. We classified the papers according to the involvement of researchers, managers and the local community in adaptive management. Five papers report on approaches developed by researchers, and one paper on a community-led program; these case studies currently have little impact on decision making. Six papers provide examples involving water managers and researchers, and two papers provide examples involving water managers and the local community. There are no papers where researchers, managers and local communities all contribute equally to adaptive management. Successful adaptive management of environmental flows occurs more often than is perceived. The final paper explores why successes are rarely reported, suggesting a lack of emphasis on reflection on management practices. One major challenge is to increase the documentation of successful adaptive management, so that benefits of learning extend beyond the project where it takes place. Finally, moving towards greater involvement of all stakeholders is critical if we are to realize the benefits of adaptive management for improving outcomes from environmental flows.

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.

Hazard Assessment and Remediation Tool for Simulation-Based Healthcare Facility Design Testing.

OBJECTIVES: To develop an objective, structured observational tool to enable identification and measurement of hazards in the built environment when applied to audiovisual recordings of simulations by trained raters. BACKGROUND: Simulation-based facility design testing is increasingly used to optimize safety of healthcare environments, often relying on participant debriefing or direct observation by human factors experts. METHODS: Hazard categories were defined through participant debriefing and detailed review of pediatric intensive care unit in situ simulation videos. Categories were refined and operational definitions developed through iterative coding and review. Hazard detection was optimized through the use of structured coding protocols and optimized camera angles. RESULTS: Six hazard categories were defined: (1) slip/trip/fall/injury risk, impaired access to (2) patient or (3) equipment, (4) obstructed path, (5) poor visibility, and (6) infection risk. Analysis of paired and individual coding demonstrated strong overall reliability (0.89 and 0.85, Gwet's AC1). Reliability coefficients for each hazard category were >0.8 for all except obstructed path (0.76) for paired raters. Among individual raters, reliability coefficients were >0.8, except for slip/trip/fall/injury risk (0.68) and impaired access to equipment (0.77). CONCLUSIONS: Hazard Assessment and Remediation Tool (HART) provides a framework to identify and quantify hazards in the built environment. The tool is highly reliable when applied to direct video review of simulations by either paired raters or trained single clinical raters. Subsequent work will (1) assess the tool's ability to discriminate between rooms with different physical attributes, (2) develop strategies to apply HART to improve facility design, and (3) assess transferability to non-ICU acute care environments.

Influence of intraoperative residual lesions and timing of extracorporeal membrane oxygenation on outcomes following first-stage palliation of single-ventricle heart disease.

BACKGROUND: Data regarding the influence of intraoperative residual lesions on extracorporeal membrane oxygenation (ECMO) following the Norwood procedure are limited. Moreover, the significance of postoperative ECMO timing on in-hospital outcomes remains incompletely characterized. METHODS: This was a single-center, retrospective review of consecutive patients who underwent the Norwood operation from January 1997 to November 2017. Patients with at least minor residual lesions based on the intraoperative postcardiopulmonary bypass echocardiogram were identified. The association between residual lesions and postoperative ECMO was assessed with logistic regression, adjusting for age, weight, prematurity, various preoperative system-specific and procedural risk factors, shunt type, and era. Among patients receiving ECMO, associations between late ECMO (≥3 days post-Norwood) and in-hospital mortality or transplant, postoperative hospital length-of-stay, and cost of hospitalization were evaluated using logistic regression or generalized linear models with a gamma distribution and logarithmic link. RESULTS: Among 500 patients, 78 (15.6%) received ECMO postoperatively. On multivariable analysis, the presence of at least minor residual lesions (odds ratio, 4.4; 95% CI, 2.1-9.3; P < .001) was associated with postoperative ECMO. In the ECMO subpopulation, there were 44 (56.4%) deaths or transplants. Late ECMO was associated with increased risk of in-hospital mortality or transplant (adjusted odds ratio, 6.2; 95% CI, 1.5-26.0), longer postoperative hospital length of stay (regression coefficient, 0.7; 95% CI, 0.3-1.1), and greater cost (regression coefficient, 0.6; 95%, CI 0.4-0.7), versus early ECMO (all P values < .05). CONCLUSIONS: The presence of even minor intraoperative residua significantly increases the risk of ECMO following the Norwood operation. Among patients receiving ECMO postoperatively, early institution of ECMO is associated with lower mortality and resource utilization.

Trends in In-Hospital Cardiac Arrest and Mortality Among Children With Cardiac Disease in the Intensive Care Unit: A Systematic Review and Meta-analysis.

Importance: Data on trends in incidence and mortality for in-hospital cardiac arrest (IHCA) in children with cardiac disease in the intensive care unit (ICU) are lacking. Additionally, there is limited information on factors associated with IHCA and mortality in this population. Objective: To investigate incidence, trends, and factors associated with IHCA and mortality in children with cardiac disease in the ICU. Data Sources: A systematic review was conducted using PubMed, Web of Science, EMBASE, and CINAHL, from inception to September 2021. Study Selection: Observational studies on IHCA in pediatric ICU patients with cardiac disease were selected (age cutoffs in studies varied from age ≤18 y to age ≤21 y). Data Extraction and Synthesis: Quality of studies was assessed using the National Institutes of Health Quality Assessment Tools. Data on incidence, mortality, and factors associated with IHCA or mortality were extracted by 2 independent observers. Random-effects meta-analysis was used to compute pooled proportions and pooled ORs. Metaregression, adjusted for type of study and diagnostic category, was used to evaluate trends in incidence and mortality. Main Outcomes and Measures: Primary outcomes were incidence of IHCA and in-hospital mortality. Secondary outcomes were proportions of patients who underwent extracorporeal membrane oxygenation (ECMO) cardiopulmonary resuscitation (ECPR) and those who did not achieve return of spontaneous circulation (ROSC). Results: Of the 2574 studies identified, 25 were included in the systematic review (131 724 patients) and 18 in the meta-analysis. Five percent (95% CI, 4%-6%) of children with cardiac disease in the ICU experienced IHCA. The pooled in-hospital mortality among children who experienced IHCA was 51% (95% CI, 42%-59%). Thirty-nine percent (95% CI, 29%-51%) did not achieve ROSC; in centers with ECMO, 22% (95% CI, 14%-33%) underwent ECPR, whereas 22% (95% CI, 12%-38%) were unable to be resuscitated. Both incidence of IHCA and associated in-hospital mortality decreased significantly in the last 20 years (both P for trend < .001), whereas the proportion of patients not achieving ROSC did not significantly change (P for trend = .90). Neonatal age, prematurity, comorbidities, univentricular physiology, arrhythmias, prearrest mechanical ventilation or ECMO, and higher surgical complexity were associated with increased incidence of IHCA and mortality odds. Conclusions and Relevance: This systematic review and meta-analysis found that 5% of children with cardiac disease in the ICU experienced IHCA. Decreasing trends in IHCA incidence and mortality suggest that education on preventive interventions, use of ECMO, and post-arrest care may have been effective; however, there remains a crucial need for developing resuscitation strategies specific to children with cardiac disease.

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.