Validation of Automated Data Extraction From the Electronic Medical Record to Provide a Pediatric Risk Assessment Score.

BACKGROUND: Although the rate of pediatric postoperative mortality is low, the development and validation of perioperative risk assessment models have allowed for the stratification of those at highest risk, including the Pediatric Risk Assessment (PRAm) score. The clinical application of such tools requires manual data entry, which may be inaccurate or incomplete, compromise efficiency, and increase physicians' clerical obligations. We aimed to create an electronically derived, automated PRAm score and to evaluate its agreement with the original American College of Surgery National Surgical Quality Improvement Program (ACS NSQIP)-derived and validated score. METHODS: We performed a retrospective observational study of children <18 years who underwent noncardiac surgery from 2017 through 2021 at Boston Children's Hospital (BCH). An automated PRAm score was developed via electronic derivation of International Classification of Disease (ICD) -9 and -10 codes. The primary outcome was agreement and correlation among PRAm scores obtained via automation, NSQIP data, and manual physician entry from the same BCH cohort. The secondary outcome was discriminatory ability of the 3 PRAm versions. Fleiss Kappa, Spearman correlation (rho), and intraclass correlation coefficient (ICC) and receiver operating characteristic (ROC) curve analyses with area under the curve (AUC) were applied accordingly. RESULTS: Of the 6014 patients with NSQIP and automated PRAm scores (manual scores: n = 5267), the rate of 30-day mortality was 0.18% (n = 11). Agreement and correlation were greater between the NSQIP and automated scores (rho = 0.78; 95% confidence interval [CI], 0.76-0.79; P <.001; ICC = 0.80; 95% CI, 0.79-0.81; Fleiss kappa = 0.66; 95% CI, 0.65-0.67) versus the NSQIP and manual scores (rho = 0.73; 95% CI, 0.71-0.74; P < .001; ICC = 0.78; 95% CI, 0.77-0.79; Fleiss kappa = 0.56; 95% CI, 0.54-0.57). ROC analysis with AUC showed the manual score to have the greatest discrimination (AUC = 0.976; 95% CI, 0.959,0.993) compared to the NSQIP (AUC = 0.904; 95% CI, 0.792-0.999) and automated (AUC = 0.880; 95% CI, 0.769-0.999) scores. CONCLUSIONS: Development of an electronically derived, automated PRAm score that maintains good discrimination for 30-day mortality in neonates, infants, and children after noncardiac surgery is feasible. The automated PRAm score may reduce the preoperative clerical workload and provide an efficient and accurate means by which to risk stratify neonatal and pediatric surgical patients with the goal of improving clinical outcomes and resource utilization.

Administration of Milrinone Following Tetralogy of Fallot Repair Increases Postoperative Volume Administration Without Improving Cardiac Output.

BACKGROUND: Phosphodiesterase inhibitors are known to relieve symptoms in the setting of heart failure, although their effects in restrictive ventricular physiology have been poorly characterized. We explored the association between the use of milrinone and volume administration during the first 72 hours following surgical repair of tetralogy of Fallot (TOF). METHODS: We reviewed all cases of primary surgical repair of TOF with pulmonary stenosis or atresia at Boston Children's Hospital between 2011 and 2020. To adjust for baseline differences between patients who did and did not receive milrinone, we matched patients with similar propensity scores in a 1:1 ratio (use of milrinone versus not). We then compared the need for volume administration during the first 72 hours postoperatively, vital signs, and measures of cardiac output between the matched cohorts. Additionally, in the group of patients receiving milrinone, linear regression modeling was used to explore the relationship between total dose of milrinone and total volume administration. RESULTS: Among 351 included patients, 134 received perioperative milrinone. A total of 212 patients (106 per group) were matched based on anatomic and surgical risks using a propensity score. After propensity matching, compared with nonmilrinone-treated patients, milrinone-treated patients were given postoperative volume more frequently (66% vs 52%; difference 14% [95% confidence interval, CI, 1%-27%]; P = .036). Milrinone-treated patients had a slower recovery of tachycardia during the first 12 hours (difference in slope 0.30 [95% CI, 0.14-0.47] beats per minute [BPM]/h; P < .001), and the intergroup difference peaked at 12 hours postoperatively (8 [95% CI, 5-12] BPM). Milrinone administration was not associated with improved cardiac output, including arteriovenous oxyhemoglobin saturation difference. In propensity-matched patients receiving milrinone, the total volume administered during the first 72 postoperative hours was significantly associated with the cumulative dose of postoperative milrinone ( r = 0.20; 95% CI, 0.01-0.38; P = .036). Based on the slope of the regression line, for every 1000 µg/kg of milrinone (equivalent to ~0.25 µg/kg/min for 72 hours) administered in the first 72 postoperative hours, an estimated 11.0 (95% CI, 0.6-21.4) mL/kg additional volume was administered. CONCLUSIONS: The use of milrinone within the first 72 hours following TOF repair is associated with more frequent administration of volume, a positive association between a higher total dose of postoperative milrinone and the amount of postoperative volume administered, a higher heart rate, and a lower blood pressure, but is not associated with improved cardiac output.

Sudden cardiac death in congenital heart disease.

Sudden cardiac death (SCD) accounts for up to 25% of deaths in patients with congenital heart disease (CHD). To date, research has largely been driven by observational studies and real-world experience. Drawbacks include varying definitions, incomplete taxonomy that considers SCD as a unitary diagnosis as opposed to a terminal event with diverse causes, inconsistent outcome ascertainment, and limited data granularity. Notwithstanding these constraints, identified higher-risk substrates include tetralogy of Fallot, transposition of the great arteries, cyanotic heart disease, Ebstein anomaly, and Fontan circulation. Without autopsies, it is often impossible to distinguish SCD from non-cardiac sudden deaths. Asystole and pulseless electrical activity account for a high proportion of SCDs, particularly in patients with heart failure. High-quality cardiopulmonary resuscitation is essential to improve outcomes. Pulmonary hypertension and CHD complexity are associated with lower likelihood of successful resuscitation. Risk stratification for primary prevention implantable cardioverter-defibrillators (ICDs) should consider the probability of SCD due to a shockable rhythm, competing causes of mortality, complications of ICD therapy, and associated costs. Risk scores to better estimate probabilities of SCD and CHD-specific guidelines and consensus-based recommendations have been proposed. The subcutaneous ICD has emerged as an attractive alternative to transvenous systems in those with vascular access limitations, prior device infections, intra-cardiac shunts, or a Fontan circulation. Further improving SCD-related outcomes will require a multidimensional approach to research that addresses disease processes and triggers, taxonomy to better reflect underlying pathophysiology, high-risk features, early warning signs, access to high-quality cardiopulmonary resuscitation and specialized care, and preventive therapies tailored to underlying mechanisms.

Anaesthesia for non-cardiac surgery in children and young adults with Fontan physiology.

INTRODUCTION: Patients with Fontan physiology require non-cardiac surgery. Our objectives were to characterise perioperative outcomes of patients with Fontan physiology undergoing non-cardiac surgery and to identify characteristics which predict discharge on the same day. MATERIALS AND METHOD: Children and young adults with Fontan physiology who underwent a non-cardiac surgery or an imaging study under anaesthesia between 2013 and 2019 at a single-centre academic children's hospital were reviewed in a retrospective observational study. Continuous variables were compared using the Wilcoxon rank sum test, and categorical variables were analysed using the Chi-square test or Fisher's exact test. Multivariable logistic regression analysis results are presented by adjusted odds ratios with 95% confidence intervals and p values. RESULTS: 182 patients underwent 344 non-cardiac procedures with anaesthesia. The median age was 11 years (IQR 5.2-18), 56.4% were male. General anaesthesia was administered in 289 (84%). 125 patients (36.3%) were discharged on the same day. On multivariable analysis, independent predictors that reduced the odds of same-day discharge included the chronic condition index (OR 0.91 per additional chronic condition, 95% CI 0.76-0.98, p = 0.022), undergoing a major surgical procedure (OR 0.17, 95% CI 0.05-0.64, p = 0.009), the use of intraoperative inotropes (OR 0.48, 95% CI 0.25-0.94, p = 0.031), and preoperative admission (OR = 0.24, 95% CI: 0.1-0.57, p = 0.001). DISCUSSION: In a contemporary cohort of paediatric and young adults with Fontan physiology, 36.3% were able to be discharged on the same day of their non-cardiac procedure. Well selected patients with Fontan physiology can undergo anaesthesia without complications and be discharged same day.

The Role of Chronic Conditions in Outcomes following Noncardiac Surgery in Children with Congenital Heart Disease.

OBJECTIVES: To compare outcomes in children with congenital heart disease (CHD) undergoing noncardiac surgery by presence of chronic conditions and identify associated risk factors. STUDY DESIGN: Retrospective analysis of 14 031 children with CHD who underwent noncardiac surgery in the 2016 Healthcare Cost and Utilization Project Kid's Inpatient Database. Multivariable regression was used to assess patient and hospital factors associated with in-hospital mortality and length of stay (LOS). RESULTS: Overall, 94% had at least 1 chronic condition. The in-hospital mortality rate was 5.6%. Neonates with CHD only had the highest mortality (15.6%); otherwise, children with CHD and at least 1 chronic condition had higher mortality than patients with CHD only (infant 3.93%, child 1.22%, adolescent 1.04% vs 2.34%, 0%, and 0%). Neonates (OR, 15.5; 95% CI, 7.1-34.1 vs adolescent), number of chronic conditions (OR, 1.34; 95% CI, 1.27-1.42), chronic conditions type (circulatory system; OR 2.46; 95% CI, 2.04-2.98), and low socioeconomic status (OR, 1.36; 95% CI, 1.05-1.77) were associated with increased mortality. The median LOS was 20 days (IQR, 5-66). Those with CHD and at least 1 chronic condition had a greater LOS (21 days; IQR, 5-68) than those with CHD only (9 days; IQR, 3-46). Neonates (adjusted coefficient, 44.3; 95% CI, 40.3-48.3 vs adolescent), Black race (adjusted coefficient, 4.78; 95% CI, 2.27-7.3), chronic condition indicator number (adjusted coefficient, 5.17; 95% CI, 4.56-5.78), and subtype (adjusted coefficient, 23.6; 95% CI, 20.4-26.7) were associated with a prolonged LOS. CONCLUSIONS: Most children with CHD who undergo noncardiac surgery have at least 1 chronic condition. Age, chronic conditions type and number, low socioeconomic status, and Black race impart increased risks of in-hospital mortality and prolonged LOS. Further research is needed to evaluate the impact of specific chronic conditions and determine barriers to equitable care.

The Association Between Race and Adverse Postoperative Outcomes in Children With Congenital Heart Disease Undergoing Noncardiac Surgery.

BACKGROUND: The association between race and perioperative outcomes has been evaluated in adult cardiac surgical and in healthy pediatric patients but has not been evaluated in children with congenital heart disease (CHD) presenting for noncardiac procedures. This study compares the incidence of the primary outcome of 30-day mortality and adverse postoperative outcomes following noncardiac surgery between Black and White children with CHD, stratified by severity. METHODS: This is a retrospective study. Comparison of outcomes between Black and White children was performed using the 2012-2018 American College of Surgeons National Surgical Quality Improvement Program Pediatric database and after stratification for severity of CHD and propensity score matching. RESULTS: A total of 55,859 patients were included, and divided into 28,601 minor, 23,839 major, and 3419 severe CHD. Black and White children in each category were matched and compared. Following matching in the overall CHD cohort, there were significantly higher rates of the following adverse postoperative outcomes among Black patients as compared to White patients: 30-day mortality (1.84% vs 1.49%; odds ratio [OR], 1.25; 95% confidence interval [CI], 1.05-1.48; P = .014), composite secondary outcomes (19.90% vs 17.88%; OR, 1.14; 95% CI, 1.08-1.21; P < .001), cardiac arrest (1.42% vs 0.98%; OR, 1.46; 95% CI, 1.19-1.79; P < .001), 30-day reoperation (7.59% vs 6.67%; OR, 1.15; 95% CI, 1.05-1.25; P = .002), and reintubation (3.9% vs 2.95%; OR, 1.34; 95% CI, 1.19-1.52; P < .001). No significant statistical interaction between race and CHD severity was found. Following matching and within the minor CHD cohort, Black children had significantly higher rates of composite secondary outcome (17.44% vs 15.60%; OR, 1.15; 95% CI, 1.05-1.25; P = .002), cardiac arrest (1.02% vs 0.53%; OR, 1.94; 95% CI, 1.37-2.76; P < .001), 30-day reoperation (7.19% vs 5.77%; OR, 1.26; 95% CI, 1.11-1.43; P < .001), and thromboembolic complications (0.49% vs 0.23%; OR, 2.17; 95% CI, 1.29-3.63; P = .003) compared to White children. In the major CHD cohort, Black children had significantly higher rates of 30-day mortality (2.75% vs 2.05%; OR, 1.35; 95% CI, 1.08-1.69; P = .008) and reintubation (4.82% vs 3.72%; OR, 1.32; 95% CI, 1.11-1.56; P = .002). There were no statistically significant differences in outcomes in the severe CHD category for 30-day mortality (3.36% vs 3.3%; OR, 1.02; 95% CI, 0.60-1.73; P = .946), composite secondary outcome (22.65% vs 21.36%; OR, 1.08; 95% CI, 0.86-1.36; P = .517) nor the components of the composite secondary outcomes. CONCLUSIONS: Race is associated with postoperative mortality and complications in children with minor and major CHD undergoing noncardiac surgery. No significant association was observed between race and postoperative outcomes in patients with severe CHD. This is consistent with previous findings wherein in patients with severe CHD, residual lesion burden and functional status is the leading predictor of outcomes following noncardiac surgery. Nevertheless, there is no evidence that the relationship between race and outcomes differs across the CHD severity categories. Future studies to understand the mechanisms leading to the racial difference, including institutional, clinical, and individual factors are needed.

The Practice of Pediatric Cardiac Anesthesiology in the United States.

BACKGROUND: With advances in surgical and catheter-based interventions and technologies in patients with congenital heart disease (CHD), the practice of pediatric cardiac anesthesiology has evolved in parallel with pediatric cardiac surgery and pediatric cardiology as a distinct subspecialty over the past 80 years. To date, there has not been an analysis of the distribution of pediatric cardiac anesthesiologists relative to cardiac and noncardiac procedures in the pediatric population. The primary aim is to report the results of a survey and its subsequent analysis to describe the distribution of pediatric cardiac anesthesiologists relative to pediatric cardiac procedures that include surgical interventions, cardiac catheterization procedures, imaging studies (echocardiography, magnetic resonance, computed tomography, positron emission tomography), and noncardiac procedures. METHODS: A survey developed in Research Electronic Data Capture (REDcap) was sent to the identifiable division chiefs/cardiac directors of 113 pediatric cardiac anesthesia programs in the United States. Data regarding cardiac surgical patients and procedures were collected from the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHD). RESULTS: This analysis reveals that only 38% (117 of 307) of pediatric cardiac anesthesiologists caring for patients with CHD pursued additional training in pediatric cardiac anesthesiology, while 44% (136 of 307) have gained experience during their clinical practice. Other providers have pursued different training pathways such as adult cardiac anesthesiology or pediatric critical care. Based on this survey, pediatric cardiac anesthesiologists devote 35% (interquartile range [IQR], 20%-50%) of clinical time to the care of patients in the cardiac operating room, 25% (20%-35%) of time to the care of patients in the cardiac catheterization laboratory, 10% (5%-10%) to patient care in imaging locations, and 15% covering general pediatric, adult, or cardiac patients undergoing noncardiac procedures. Attempts to actively recruit pediatric cardiac anesthesiologists were reported by 49.2% (29 of 59) of the institutions surveyed. Impending retirement of staff was anticipated in 17% (10 of 59) of the institutions, while loss of staff to relocation was anticipated in 3.4% (2 of 59) of institutions. Thirty-seven percent of institutions reported that they anticipated no immediate changes in current staffing levels. CONCLUSIONS: The majority of currently practicing pediatric cardiac anesthesiologists have not completed a fellowship training in the subspecialty. There is, and will continue to be, a need for subspecialty training to meet increasing demand for services especially with increase survival of this patient population and to replace retiring members of the workforce.

The Lymphatic System in the Fontan Patient-Pathophysiology, Imaging, and Interventions: What the Anesthesiologist Should Know.

The Fontan surgery was developed as a palliative intervention for congenital heart disease (CHD) patients with single-ventricle physiology who are not candidates for a biventricular repair. Improvements in the surgery and medical management of these patients have increased survival, yet this population remains at risk for complications and end-organ dysfunction due to Fontan failure. Lymphatic vessels maintain a fluid balance within the extracellular space, participate in fat reabsorption from the small intestine, and play an important role in the body's immune response. Altered Starling forces at the capillary level, capillary leak, and lymphatic obstruction contribute to lymphatic dysfunction in patients with Fontan physiology. These lymphatic complications include edema, pleural effusions, plastic bronchitis (PB), and protein-losing enteropathy (PLE). Over the past decade, there have been innovations in lymphatic imaging. These new imaging techniques include noncontrast magnetic resonance (MR) lymphangiography, intranodal lymphangiography (IL), dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL), and liver lymphangiography. These imaging techniques help in delineating anatomy and guiding the appropriate therapeutic approach. Lymphatic interventions then may be performed to decompress the lymphatic system or to identify and occlude abnormal lymphatic vessels and drainage pathways. The anesthesiologist should have an understanding of the effects of lymphatic disorders on the Fontan circulation and apply appropriate management techniques for the associated interventions. The Fontan surgery was developed as a palliative intervention for CHD patients with single-ventricle physiology who are not candidates for a biventricular repair. The surgery creates a series systemic and pulmonary circulation with the energy necessary to provide gradient-driven pulmonary blood flow generated by the ventricle.1 In the past decades, improvements in the surgery and medical management of these patients have increased survival, with 30-year survival rates close to 85%.2 Despite these improvements, this population remains at risk for complications and end-organ dysfunction due to Fontan failure, which is characterized by elevated systemic venous pressures and low cardiac output. These complications include arrhythmias, cardiac dysfunction, ascites, liver fibrosis/cirrhosis, renal dysfunction, pulmonary failure, and lymphatic complications such as edema, pleural effusions, PB, and PLE. Complications ultimately contribute to increased risk for hospitalization, death, and need for heart transplantation.3,4 For this reason, there has been increasing interest in the role of abnormal lymphatic circulation in the genesis of Fontan failure. The authors characterize the lymphatic pathophysiology associated with Fontan physiology and review the imaging and interventional strategies used to treat these patients.

Biventricular repair as an alternative to single-ventricle palliation in a child with hypoplastic left heart structures: What the anesthesiologist should know.

Enhanced techniques for single-ventricle palliation (SVP), in which the Fontan procedure is the final phase, have improved the survival and quality of life in patients not eligible for biventricular repair (BiVR). However, Fontan physiology also is associated with predictable long-term consequences that ultimately affect quality of life and freedom from adverse events. Given this harsh reality, the advances in ventricular rehabilitation strategies and the conversion from SVP increasingly are being used to achieve biventricular circulation in patients with left-heart hypoplasia who previously were considered to be marginal or unacceptable candidates for BiVR. The techniques for ventricular recruitment include restriction of the atrial septal defect (ASD), atrioventricular valve repair, repair of left ventricular outflow obstruction, endocardial fibroelastosis resection, an augmented pulmonary blood flow, and catheter-based interventions. In addition, reverse double-switch surgery has been used as an option for patients with a left ventricle of insufficient size and function to support the systemic circulation, who would otherwise require a Fontan surgery. Despite improved management and surgical technique, the outcomes after biventricular staging repair and conversion remain variable, and the complications ultimately may exclude patients from future single-ventricle palliation or transplant. The anesthesiologist should have a basic understanding of the anatomy and physiology of this growing patient population in order to develop adequate perioperative management options.

Dosing of Opioid Medications During and After Pediatric Cardiac Surgery for Children With Down Syndrome.

OBJECTIVE: To determine whether children with Down syndrome (DS) receive higher doses of opioid medications compared with children without DS for repair of complete atrioventricular canal (CAVC). DESIGN: A retrospective chart review of children with and without DS who underwent primary repair of CAVC. The exclusion criteria included unbalanced CAVC and patients undergoing biventricular staging procedures. The primary outcome was oral morphine equivalents (OME) received in the first 24 hours after surgery. The secondary outcomes included intraoperative OME, OME at 48 and 72 hours, nonopioid analgesic and sedative medications received, pain scores, time to extubation, and length of stay. SETTING: A pediatric academic medical center in the United States. PARTICIPANTS: One hundred thirty-one patients with DS and 24 without, all

Anesthesia in Children With Pulmonary Hypertension: Clinically Significant Serious Adverse Events Associated With Cardiac Catheterization and Noncardiac Procedures.

OBJECTIVES: To determine the incidence of clinically significant serious adverse events in a contemporary population of pediatric patients with pulmonary hypertension who require anesthesia and identify factors associated with adverse outcomes. DESIGN: A retrospective, cross-sectional study. SETTING: A single-center quaternary-care freestanding children's hospital in the northeastern United States. PARTICIPANTS: Pediatric patients with pulmonary hypertension based on hemodynamic criteria on cardiac catheterization during a 3-year period from 2015 to 2018. INTERVENTIONS: Anesthesia care for cardiac catheterization, noncardiac surgery, and diagnostic imaging. MEASUREMENTS AND MAIN RESULTS: Two hundred forty-nine children underwent 862 procedures, 592 for cardiac catheterization and 278 for noncardiac surgery and diagnostic imaging. The median age was 1.6 years, and the weight was 9.5 lbs. On index catheterization, median pulmonary artery pressure was 36 mmHg, and the pulmonary vascular resistance was 5.1 indexed Wood units. Ten percent of anesthetics were performed with a natural airway, and 80% used volatile anesthetics. Serious adverse events occurred in 26% of procedures (confidence interval [CI], 22%-30%). The rate of periprocedural cardiac arrest was 8 per 1,000 anesthetic administrations. In multivariate analysis, younger age (adjusted odds ratio [aOR], 1.4 per year; CI, 1.1-1.9; p = 0.01), location in the catheterization laboratory (aOR, 5.1; CI, 1.7-16; p = 0.004), and longer procedure duration (aOR, 1.3 per 30 minutes; CI, 1.1-1.4; p = 0.001) were associated with serious adverse events. Patients with a tracheostomy in place were less likely to experience an adverse event (aOR, 0.1; CI, 0.04-0.5; p = 0.001). The primary anesthetic technique was not associated with adverse events. Interventional cardiac catheterization was associated with an increased incidence of adverse events compared with diagnostic catheterization (42% v 21%; OR, 2.23; CI, 1.5-3.3; p < 0.001). CONCLUSIONS: Serious adverse events were common in this cohort. Careful planning to minimize anesthesia time in young children with pulmonary hypertension should be undertaken, and these factors considered in designing risk mitigation strategies.

Viscoelastic hemostatic assays: Update on technology and clinical applications.

Viscoelastic hemostatic assays (VHA) are point of care tests that allow for a global assessment of coagulation using whole blood. The technology to allow this assessment has evolved from the original thromboelastography (TEG, Haemonetic, Boston, MA) to now include thromboelastometry (ROTEM, Instrumentation Laboratory, Bedford, MA), and, most recently, the Quantra Hemostasis Analyzer (Hemosonics, Charlottesville, VA). Diagnosis and treatment algorithms incorporating viscoelastic hemostatic tests for bleeding patients in a variety of clinical situations have now been developed. The original ROTEM and TEG technologies have been updated with emphasis placed on a cartridge-based technologies. Results from the new devices show good correlation with those from the previous versions of the devices, while cartridge-based technology has increased device stability and enhanced portability to the bedside. In this article, we will review recent advances in TEG and ROTEM technology and introduce the Quantra Hemostasis Analyzer device.

Airway Abnormalities in Patients With Congenital Heart Disease: Incidence and Associated Factors.

OBJECTIVES: Airway abnormalities complicate the perioperative course of patients with congenital heart disease (CHD), leading to significant morbidity and mortality. The literature describing airway abnormalities in those patients is scarce. This study aimed to determine the incidence of airway abnormalities in CHD patients and identify associated factors, genetic syndromes, and cardiac diagnoses. DESIGN: Retrospective study conducted after institutional review board approval. SETTING: Tertiary children's hospital. PARTICIPANTS: Patients presenting for cardiac diagnostic, interventional, or surgical procedures from 2012 to 2018. A total of 9,495 encounters were reviewed. EXCLUSION CRITERIA: age >18 years. Methods/Interventions: Age, weight, sex, intubation technique, number of intubation attempts, and difficult intubation (DI) were recorded. Using the International Classification of Diseases, Ninth and Tenth Revisions codes, genetic syndromes, acquired and congenital airway abnormalities, and cardiac diagnoses were identified. Multivariate generalized estimating equations modeling was used to identify independent predictors of airway abnormalities. RESULTS: A total of 4,797 patients, with 8,657 encounters were included. The median age was 1.3 years (interquartile range [IQR]: 0.2-6.0) and weight was 9.2 kg (IQR: 4.3-19.2), and 55% were male. A total of 16.7% had at least 1 genetic syndrome; 8.5% had congenital airway abnormalities and 9.7% acquired. Incidence of DI was 1.1%. The most common syndromes were Down, 22q11.2 microdeletion, and CHARGE. The most frequent congenital airway abnormalities were laryngomalacia and bronchomalacia, and the most frequent acquired were partial and total vocal cord paralysis. CONCLUSION: The likelihood of a coexistent airway abnormality should be considered in premature CHD patients, weight <10 kg, and in those with specific cardiac lesions and a concomitant genetic syndrome. Preoperative identification of patients at high risk of airway abnormalities is useful in planning their perioperative airway management.

Predictors of Increased Lactate in Neonatal Cardiac Surgery: The Impact of Cardiopulmonary Bypass.

OBJECTIVE: Hyperlactatemia develops intraoperatively during cardiac surgery and is associated with postoperative mortality. This study aimed to determine the factors that lead to an increase in lactate during cardiopulmonary bypass (CPB) in neonates undergoing cardiac surgery. DESIGN: Retrospective study from July 2015 to December 2018. SETTING: Academic tertiary children's hospital. PARTICIPANTS: The study comprised 376 neonates. INTERVENTIONS: No interventions were performed. MEASUREMENTS AND MAIN RESULTS: Lactate measurements at prebypass, upon initiation of CPB and before coming off CPB, last in the operating room, and first in the cardiac intensive care unit were collected. The changes in lactate levels were compared using the nonparametric Wilcoxon signed rank test for paired data. Univariate and multivariate median regression models of the change during CPB were determined. The cohort characteristics were male (60%), median age 5 days (range 1-30), and weight 3.2 kg (range 1.5-4.7). Most patients had a STAT score of 4 (45%) or 5 (23%). Significant increases in lactate were observed from pre-CPB to start of CPB (p < 0.001) and from start to end of CPB (p < 0.001). In the multivariate regression analysis, duration of circulatory arrest (coefficient = 1.216; 95% confidence interval [CI] 0.754-1.678; p < 0.001), duration of mean arterial pressure < 25 mmHg (coefficient = 0.423; 95% CI 0.196-to- 0.651; p < 0.001), and duration of mean arterial pressure between 35 and 39 mmHg (coefficient = -0.246; 95% CI -0.397 to -0.095; p = 0.001) were identified as significant independent predictors of the lactate change per 30- minutes duration. CONCLUSION: These results emphasized the importance of blood pressure management during CPB and the importance of the duration of circulatory arrest.

Elective Non-Urgent Balloon-Atrial Septostomy in Infants with d-Transposition of the Great Arteries Does Not Eliminate the Need for PGE1 Therapy at the Time of Arterial Switch Operation.

Once a mainstay in the treatment of neonates with d-transposition of the great arteries (d-TGA), the application of balloon atrial septostomy (BAS) in the d-TGA population has become more selective. Currently, there is no clear evidence for or against a selective BAS strategy. The aims of this single-center retrospective study were to determine the incidence of BAS in the neonatal d-TGA population in the current era, to measure the rate of procedural success, and to compare the outcomes and complication rates of patients who underwent BAS to those who underwent neonatal ASO alone. Between 2012 and 2018, 147 patients with d-TGA underwent initial medical management and ASO, 73 of which underwent BAS. The percentage of patients that underwent BAS decreased from 73 to 33% over the study time period. In patients with d-TGA with intact ventricular septum, 33% of patients remained off of PGE1 at the time of surgery regardless of BAS. In d-TGA with ventricular septal defect, 85.7% of those that underwent BAS and 54.1% of those who did not remained off of PGE1 at the time of surgery, however, this difference did not reach statistical significance. In this single institution retrospective cohort of patients with d-TGA, the performance of a technically successful balloon atrial septostomy did not eliminate the need for PGE1 therapy at the time of definitive ASO. This was true regardless of the presence or absence of a ventricular septal defect.

Hyperlactataemia as a predictor of adverse outcomes post-cardiac surgery in neonates with congenital heart disease.

OBJECTIVE: To evaluate the discriminative ability of hyperlactataemia for early morbidity and mortality in neonates with CHD following cardiac surgery. METHODS: Retrospective, observational study of neonates who underwent cardiac surgery on cardiopulmonary bypass at a tertiary care children's hospital from June 2015 to June 2019. The primary predictor was lactate. The primary composite outcome was defined as ≥1 of the following: cardiac arrest or extracorporeal membrane oxygenation within 72 hours or 30-day mortality post-operatively. The secondary outcome was the presence of major residual lesions, according to the Technical Performance Score. RESULTS: Of 432 neonates, 28 (6.5%) sustained the composite outcome. On univariate analysis, peak lactate within 48 hours, increase in lactate from ICU admission through 12 hours, and single ventricle physiology were significantly associated with the composite outcome. The peak lactate occurred at a median of 2.9 hours (interquartile range: 1, 35) before the event. Through multi-variable analysis, a multi-variable risk algorithm was created. Predicted probabilities demonstrated an increasing risk based on single ventricle status and delta lactate, ranging from 1.8% (95% CI: 0.9, 3.9) to 52.4% (95% CI: 32.4, 71.7). The model had good discriminative ability for the composite outcome on receiver operating characteristic analysis (area under the curve = 0.79; 95% CI: 0.75, 0.89). Moreover, a peak lactate of 7.3 mmol/l or greater was significantly associated with the presence of a major residual lesion (odds ratios: 5.16, 95% CI: 3.01, 8.87). CONCLUSIONS: We present a simple, two-variable model, including delta lactate in the immediate post-operative period and single ventricle status, to prognosticate the risk of early morbidity and mortality in neonates undergoing cardiac surgery for potential intervention.

Integration of the Intrinsic Surgical Risk With Patient Comorbidities and Severity of Congenital Cardiac Disease Does Not Improve Risk Stratification in Children Undergoing Noncardiac Surgery.

BACKGROUND: The objective of this study is to estimate the surgical risk of noncardiac procedures on the incidence of 30-day mortality in children with congenital heart disease. METHODS: Children with congenital heart disease undergoing noncardiac surgery from 2012 to 2016 and included in the American College of Surgeons National Surgical Quality Improvement Program (ACS NSQIP) Pediatric database were included in the derivation cohort, while the 2017 database was used as a validation cohort. Intrinsic surgical risk quartiles were built utilizing 30-day mortality rates for each Current Procedural Terminology code and relative value units to create 2 groups defined as low surgical risk (quartiles 1-3) and high surgical risk procedures (quartile 4). We used multivariable logistic regression to determine the predictors for 30-day mortality including patient comorbidities and intrinsic surgical risk. A partially external validation of the model was performed using the 2017 version of the database. RESULTS: We included 37,658 children with congenital heart disease undergoing noncardiac surgery with an incidence of overall 30-day mortality of 1.7% in the derivation cohort and 1.5% in the validation cohort (n = 13,129). Intrinsic surgical risk of procedures represented by Current Procedural Terminology procedural codes and relative value units risk quartiles was significantly associated with 30-day mortality (unadjusted P < .001). Predicted probability of 30-day mortality ranges from 0.2% (95% confidence interval [CI], 0.2-0.2) with no comorbidities to 39.6% (95% CI, 23.2-56.0) when all comorbidities were present among high surgical risk procedures and from 0.3% (95% CI, 0.3-0.3) to 54.8% (95% CI, 39.4-70.1) among low surgical risk procedures. An excellent discrimination was reported for the multivariable model with area under the curve (AUC) of 0.86 (95% CI, 0.85-0.88). High surgical risk was not associated with increased odds of 30-day mortality after adjustment for all other predictors (adjusted odds ratio [OR]: 0.75, 95% CI, 0.62-0.91). We also estimated the discriminative ability of a model that does not include the surgical risk (0.86 [95% CI, 0.84-0.88], with P value for the direct comparison of the AUC of the 2 models = 0.831). The multivariable model obtained from an external validation cohort reported an optimism corrected AUC of 0.88 (95% CI, 0.85-0.91). CONCLUSIONS: Our study demonstrates that integration of intrinsic surgical risk to comorbidities and severity of cardiac disease does not improve prediction of 30-day mortality in children undergoing noncardiac surgery. In children with congenital heart disease, patient comorbidities, and severity of the cardiac lesion are the predominant predictors of 30-day mortality.

Sensitivity of a Next-Generation NIRS Device to Detect Low Mixed Venous Oxyhemoglobin Saturations in the Single Ventricle Population.

Regional cerebral oxygenation index (rSO2) based on near-infrared spectroscopy (NIRS) is frequently used to detect low venous oxyhemoglobin saturation (ScvO2). We compared the performance of 2 generations of NIRS devices. Clinically obtained, time-matched cerebral rSO2 and ScvO2 values were compared in infants monitored with the FORE-SIGHT (n = 73) or FORE-SIGHT ELITE (n = 47) by linear regression and Bland-Altman analyses. In both devices, cerebral rSO2 correlated poorly with measured ScvO2 (FORE-SIGHT partial correlation 0.50 [95% confidence interval {CI}, 0.40-0.58]; FORE-SIGHT ELITE partial correlation 0.47 [0.39-0.55]) and mean bias was +8 (standard deviation [SD] 13.2) for FORE-SIGHT and +14 (SD 12.5) for FORE-SIGHT ELITE. When ScvO2 was <30%, rSO2 was <40 in 8% of FORE-SIGHT ELITE readings. Future NIRS should be validated in more hypoxic cohorts.

Comprehensive Risk Assessment of Morbidity in Pediatric Patients Undergoing Noncardiac Surgery: An Institutional Experience.

BACKGROUND: Utilizing the intrinsic surgical risk (ISR) and the patient's chronic and acute conditions, this study aims to develop and validate a comprehensive predictive model of perioperative morbidity in children undergoing noncardiac surgery. METHODS: Following institutional review board (IRB) approval at a tertiary care children's hospital, data for all noncardiac surgical encounters for a derivation dataset from July 2017 to December 2018 including 16,724 cases and for a validation dataset from January 2019 to December 2019 including 9043 cases were collected retrospectively. The primary outcome was a composite morbidity score defined by unplanned transfer to an intensive care unit (ICU), acute respiratory failure requiring intubation, postoperative need for noninvasive or invasive positive pressure ventilation, or cardiac arrest. Internal model validation was performed using 1000 bootstrap resamples, and external validation was performed using the 2019 validation cohort. RESULTS: A total of 1519 surgical cases (9.1%) experienced the defined composite morbidity. Using multivariable logistic regression, the Risk Assessment of Morbidity in Pediatric Surgery (RAMPS) score was developed with very good predictive ability in the derivation cohort (area under the curve [AUC] = 0.805; 95% confidence interval [CI], 0.795-0.816), very good internal validity using 1000 bootstrap resamples (bias-corrected Nagelkerke R = 0.21 and Brier score = 0.07), and good external validity (AUC = 0.783; 95% CI, 0.770-0.797). The included variables are age <5 years, critically ill, chronic condition indicator (CCI) ≥3, significant CCI ≥2, and ISR quartile ≥3. The RAMPS score ranges from 0 to 10, with the risk of composite morbidity ranging from 1.8% to 42.7%. CONCLUSIONS: The RAMPS score provides the ability to identify a high-risk cohort of pediatric patients using a 5-component tool, and it demonstrated good internal and external validity and generalizability. It also provides an opportunity to improve perioperative planning with the intent of improving both individual-patient outcomes and the appropriate allocation of health care resources.

Anesthesia in Pediatric Patients With Congenital Heart Disease Undergoing Noncardiac Surgery: Defining the Risk.

The incidence of moderate to severe congenital heart disease (CHD) in the United States is estimated to be 6 per 1,000 live-born, full-term infants. Recent advances in pediatric cardiology, surgery, and critical care have improved significantly the survival rates of patients with CHD leading to an increase in prevalence in both children and adults. Children with CHD significant enough to require cardiac surgery frequently also undergo noncardiac surgical procedures. With this increased demand for procedures that require anesthesia, all anesthesiologists, and more specifically, pediatric anesthesiologists will encounter patients with repaired or unrepaired CHD and other cardiac diseases in their practice. They often are faced with the question, "Is this patient too high risk for anesthesia?" The objective of this literature review is to provide a greater understanding of patients at high risk and to quantify the risk for patients, their families, and clinicians. In addition, specific high-risk lesions (single ventricle, Williams-Beuren syndrome, pulmonary hypertension, cardiomyopathies, and ventricular assist devices) are described.