BACKGROUND:Children with congenital heart disease undergoing noncardiac surgery are at increased risk of perioperative complications. The objectives of our study are (1) to describe the prevalence and characteristics of children undergoing noncardiac surgery in the presence or absence of COVID-19 infection and (2) describe the association between preoperative diagnosis of COVID-19 and outcomes. METHODS:We identified all children who underwent surgery between 2017 and 2022 in the American College of Surgeons National Surgical Quality Improvement Program pediatric database. Starting in 2021, the presence of a preoperative diagnosis of COVID-19 was reported in the database. The outcomes included 30-day mortality and the incidence of the following major postoperative outcomes: cardiac arrest, reintubation, pneumonia, sepsis, and readmission. A composite end point that includes the presence of at least 1 of the abovementioned complications was created. RESULTS:We identified 2919 patients with a preoperative diagnosis of COVID-19 versus 537 622 without. Patients with a preoperative diagnosis of COVID-19 underwent emergent procedures more often (64% versus 29%). Using multivariable logistic regression, the presence of preoperative diagnosis of COVID-19 increased the incidence of the composite endpoint (odds ratio [OR], 1.37 [95% CI, 1.19-1.56]; P<0.001), as did the presence of CHD (OR, 2.06 [95% CI, 2.00-2.13]; P<0.001). CONCLUSIONS:Our study describes the prevalence and characteristics of children with congenital heart disease undergoing noncardiac surgery during the COVID-19 pandemic. We report that the preoperative diagnosis of COVID-19 and the presence of congenital heart disease significantly increased the risk of complications.

DESIGN:Apixaban is a direct oral anticoagulant that inhibits factor Xa. Our objective was to examine the effect of apixaban on perioperative transfusion requirements in pediatric patients undergoing cardiac surgery with cardiopulmonary bypass. SETTING:Quaternary children's hospital. PARTICIPANTS:Children with congenital or acquired heart disease who received apixaban less than 3 days prior to a cardiac surgical procedure on cardiopulmonary bypass from 2021 to 2023. INTERVENTIONS:A retrospective review. MEASUREMENTS AND MAIN RESULTS:Forty-three patients with recent apixaban use (median = 2 doses held) were compared to 172 propensity-matched controls. In the entire cohort, 81% of procedures were urgent or emergent, and 30% were reoperative sternotomies. Patients on apixaban received a greater volume of red cell salvage intraoperatively (20.6 v 12.9 mL/kg, p = 0.041). There was no significant difference in the volume of nonautologous blood products transfused intraoperatively between the 2 groups. In the first 24 hours postoperatively, more apixaban patients received a transfusion of packed red blood cells and fresh-frozen plasma. Between the 2 groups, there was no statistically significant difference in the rate of emergent reexploration after the surgery or death within 7 days of operation. CONCLUSION:Recent apixaban use in children undergoing cardiac surgery was associated with increased intraoperative transfusion of salvaged red cells. The recent use of apixaban does not appreciably increase the perioperative transfusion requirements of pediatric patients undergoing cardiac surgery, including those undergoing emergent procedures.

BACKGROUND:We aimed to evaluate the incidence of preoperative anaemia and transfusion over the last 11 yr and the association between anaemia, transfusion, and postoperative outcomes in children undergoing noncardiac surgery. METHODS:We identified children 1-18 yr of age who had a preoperative haematocrit recorded between 2012 and 2023 in the American College of Surgeons National Surgical Quality Improvement Program paediatric databases. Patients were included in four groups: patients without anaemia who did not receive a transfusion, patients without anaemia who did receive a transfusion, patients with anaemia who did not receive a transfusion, and patients with anaemia who did receive a transfusion. The outcomes were 30-day mortality and postoperative complications. RESULTS:The incidence of preoperative anaemia was 25.7% (n=110 341), and the overall transfusion rate was 10.4% (n=44 808). Between 2012 and 2023, the incidences of preoperative anaemia and transfusion remained stable. The incidence of 30-day mortality increased significantly between children without anaemia who did not receive a transfusion (0.08%), without anaemia who did receive a transfusion (0.30%), with anaemia who did not receive a transfusion (0.27%), or with anaemia who did receive a transfusion (1.11%). The highest incidence of complications was found in children with anaemia exposed to perioperative transfusion. CONCLUSIONS:The incidence of preoperative anaemia and transfusion in children have remained stable in the last 11 yr. Anaemia, transfusion, and the combination of both increased 30-day mortality and the incidence of postoperative complications.

From the *Arthur S. Keats Division of Pediatric Cardiovascular Anesthesia, Department of Anesthesiology, Perioperative and Pain Medicine, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas †Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts. Accepted for publication July 22, 2024. Conflicts of Interest, Funding: Please see DISCLOSURES at the end of this article. Reprints will not be available from the authors. Address correspondence to David Faraoni, MD, PhD, Department of Anesthesiology, Perioperative and Pain Medicine, Texas Children's Hospital, 6651 Main Str, Suite 1940.22, Houston, TX 77030. Address e-mail to [email protected].

OBJECTIVE:To describe cardiac arrest during anesthesia care provided by a pediatric cardiac anesthesiologist in patients with congenital heart disease and identify predictors of outcome. DESIGN:Retrospective chart review. SETTING:Quaternary children's hospital. PARTICIPANTS:Patients with congenital cardiac disease undergoing cardiac procedures or imaging between January 2016 and December 2022 INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS:Seventy-six patients experienced cardiac arrest during anesthesia care. The median patient age was 424 days (interquartile range [IQR], 75-4596 days), and the median weight was 7.6 kg (IQR, 4.7-44.1 kg). Twenty-five patients had single ventricle physiology (34%), and 50 patients had normal systemic ventricular function (66%). Cardiac arrest occurred in the cardiac catheterization laboratory in 43 patients (57%) and in the cardiac operating room in 25 patients (33%). Cardiac arrest occurred most frequently during the procedure (n = 60; 79%). The etiology was arrhythmia in 29 patients (38%) and low cardiac output or ischemia in 21 patients (28%). The median duration of cardiopulmonary resuscitation (CPR) was 4 minutes (IQR, 1-11 minutes). Thirty-one patients (44%) received a code dose of epinephrine (10 µg/kg or 1 mg), and 25 patients (33%) required extracorporeal membrane oxygenation (ECMO). Death or neurologic injury within 7 days occurred in 18 patients (24%). On multivariable analysis, only the use of ECMO (adjusted odds ratio, 16, 95% confidence interval, 2.1-93.2; p = 0.006) was independently associated with the composite outcome of neurologic injury or death. CONCLUSIONS:Patients who experienced cardiac arrest while under the care of a pediatric cardiac anesthesiologist had a median CPR duration of 4 minutes. The sole independent predictor of death or neurologic injury within 7 days was the use of ECMO.

BACKGROUND:Despite advances in medical care, noncardiac surgery in children with congenital heart disease (CHD) remains associated with considerable perioperative morbidity and mortality. This study evaluates trends in postoperative outcomes after noncardiac surgery in children with and without CHD across two time periods. AIMS:We aimed to determine and compare the incidence of adverse outcomes following noncardiac surgery in a large cohort of children without CHD, in children with minor CHD, major CHD, and severe CHD, across two time periods (2012-2016 and 2017-2022, excluding 2020). METHODS:Children undergoing noncardiac surgery from 2012 to 2022 were identified from the ACS-NSQIP Pediatric database, excluding 2020. Patients were stratified by CHD severity (none, minor, major, severe) and by era (2012-2016 vs. 2017-2022). Outcomes included 30-day mortality, cardiac arrest, reintubation, reoperation, and readmission. Multivariable logistic regression was used to compare outcomes across eras, adjusting for demographic, clinical, and procedural variables. RESULTS:Among 1 023 638 children, there were 88.3% patients with no CHD and 11.7% with CHD (5.9% minor, 5.2% major, 0.6% severe). Improvements were seen across all CHD subgroups, particularly in reintubation and readmission rates. The most consistent improvement was in reintubation, including in severe CHD (aOR 0.69; 95% CI: 0.53, 0.89; p = 0.005). CONCLUSION:These findings demonstrate meaningful improvements in postoperative outcomes over time, notably reduced rates of 30-day mortality, reintubation, and readmission in the non-CHD group. Patients with minor and major CHD experienced significant declines in reintubation and readmission, while those with severe CHD showed reduced reintubation. Despite rising CHD prevalence and complexity, improvements likely reflect advances in perioperative care, risk stratification, and multidisciplinary management at specialized centers for children undergoing noncardiac surgery.

Background: A subset of patients undergoing complete atrioventricular septal defect (AVSD) repair underwent prolonged postoperative mechanical ventilation, contributing to extended intensive care unit and hospital stays. This study evaluated perioperative predictors of prolonged ventilation following complete AVSD repair and explored the utility of Brixia scores for early risk stratification. Methods: We performed a retrospective review of complete AVSD repairs in patients who were <12 months of age at a single academic hospital. Patients with heterotaxy syndrome and airway abnormality were excluded. Demographic, perioperative, partial pressure of oxygen (PaO2) and fraction of inspired oxygen (FiO2) ratios (P/F ratio), imaging, and echocardiographic data were collected. Factors associated with prolonged duration of mechanical ventilation (≥24 h) were identified. Results: We included 129 patients for analysis. Patients were divided based on the duration of mechanical ventilation (≥24 and <24 h). The prolonged mechanical ventilation group had a significantly lower median [interquartile range] P/F ratio after cardiopulmonary bypass (102.0 [70.0-180.0] vs 172.5 [79.4-265.0], P = .048). Factors associated with prolonged mechanical ventilation from the multivariable analysis were preoperative Brixia scores and first 24-h postoperative fluid balance. Conclusions: Perioperative factors can be used to identify patients with an increased risk of prolonged ventilation after complete AVSD repairs. The P/F ratios and Brixia scores are useful as early predictors. Postoperative fluid management is a modifiable factor to reduce mechanical ventilation duration.

This document has been developed as an expert consensus document by the Pediatric and Congenital Interventional Cardiovascular Society (PICS), the Association for European Paediatric and Congenital Cardiology (AEPC), the Asia-Pacific Pediatric Cardiac Society (APPCS), the Cardiac Society of Australia and New Zealand (CSANZ), Society for Cardiovascular Angiography & Interventions (SCAI), and the Latin American Society of Interventional Cardiology (SOLACI), with additional endorsement from the Congenital Cardiac Anesthesia Society (CCAS) and the American Association of Physicists in Medicine (AAPM, Sections 7 and 11).

Coarctation of the aorta (CoA) is a ductus arteriosus (DA)-dependent form of congenital heart disease (CHD) characterized by narrowing in the region of the aortic isthmus. CoA is a challenging diagnosis to make prenatally and is the critical cardiac lesion most likely to go undetected on the pulse oximetry-based newborn critical CHD screen. When undetected CoA causes obstruction to blood flow, life-threatening cardiovascular collapse may result, with a high burden of morbidity and mortality. Hemodynamic monitoring practices during DA closure (known as an “arch watch”) vary across institutions and existing tools are often insensitive to developing arch obstruction. Novel measures of tissue oxygenation and oxygen deprivation may improve sensitivity and specificity for identifying evolving hemodynamic compromise in the newborn with CoA. We explore the benefits and limitations of existing and new tools to monitor the physiological changes of the aorta as the DA closes in infants at risk of CoA.

Intensive care of the neonate involves a delicate balance of monitoring, intervention, and adaptation, particularly in the face of hemodynamic instability. Neonatal hemodynamic management has become increasingly sophisticated and comprehensive in recent years, driven by a deeper understanding of cardiovascular physiology and the development of innovative diagnostic and therapeutic modalities. Traditionally, hemodynamic status and end-organ perfusion are assessed utilizing measurements of heart rate, blood pressure, serum lactate, capillary refill time, and urine output. These traditional parameters, however, have been shown to have reduced utility in the comprehensive hemodynamic management of premature infants and critically ill neonates.1, 2 Consequently, targeted neonatal echocardiography (TNE) has emerged as an essential tool, offering invaluable real-time insights into neonatal hemodynamics and cardiovascular physiology which can be used to guide personalized therapeutic interventions in the neonatal intensive care unit (NICU).3 In this issue of the Journal of the American Society of Echocardiography, McNamara et al. update the guidelines for TNE, first published in 2011.3 Since that time, the performance of TNE has advanced dramatically in both breadth and sophistication, with formal training programs in TNE now established. TNE is now widely utilized to provide a more comprehensive dynamic hemodynamic assessment, providing functional echocardiographic findings in neonates with low concern for major structural defects. TNE is used to evaluate neonates with patent ductus arteriosus (PDA), acute and chronic pulmonary hypertension (PH), shock, low systemic blood flow states during transition in premature neonates, perinatal asphyxia, congenital diaphragmatic hernia, and pericardial or pleural effusion.3, 4 In this expanded editorial, we discuss neonatal hemodynamic management, with a specific focus on the role of echocardiography in guiding clinical decision-making. Hemodynamically significant PDA (hsPDA) has been associated with the development of intraventricular hemorrhage (IVH), necrotizing enterocolitis (NEC), retinopathy of prematurity (ROP), and bronchopulmonary dysplasia (BPD).5-8 The incidence of hsPDA is inversely proportional to gestational age and can be as high as 90% in neonates < 24 weeks gestation.1, 5 While there is no widely agreed-upon definition of hsPDA, the principles of echocardiographic assessment of hsPDA focus on the characterization of:1)The PDA. Necessary measurements include ductal length and diameter, shunt direction, and trans-ductal pressure gradient, with the caveat that the simplified Bernoulli's Equation may underestimate the true gradient across a long PDA.2)Excessive pulmonary blood flow (pulmonary overcirculation) and resultant left-sided volume overload. This requires an assessment of the LV/RV output (LVO/RVO) ratio, the presence of LA/LV dilation, and the LA/aortic root diameter (LA/Ao) ratio as determined by M-mode, with a ratio > 1.4 indicative of LV volume overload.3)Biventricular systolic function. Assessment of LVEF, RV fractional area change (FAC), tricuspid annular plane systolic excursion (TAPSE), and global longitudinal strain (GLS) is required. GLS is less impacted by heart size, tethering, and angle errors than TAPSE.4)LV diastolic function. Assessment of E/A and E/e' is required. In neonates, high heart rates can merge E and A peaks, and sometimes normally functioning neonatal hearts have a higher peak A than peak E. Although not specific to a PDA, distal descending aorta diastolic flow reversal may suggest substantial L-R shunt and systemic hypoperfusion. Additional metrics include the absence or reversal of flow in the celiac or middle cerebral artery. In neonates < 28 weeks gestation or with clinical suspicion of PDA, the initial TNE exam should exclude ductal-dependent systemic or pulmonary blood flow lesions. Follow-up TNE can evaluate treatment effectiveness or spontaneous closure of the PDA. Treatment options for hsPDA include medical management, surgical ligation, and transcatheter PDA closure. Since FDA approval of the Amplatzer Piccolo device in 2019, there has been an increased trend in transcatheter PDA closure in extreme low birth weight infants down to 700 grams.5, 8 Transcatheter PDA closure in neonates and infants has demonstrated lower resource utilization, mechanical ventilation, LOS, and mortality than surgical ligation.8 Post PDA ligation cardiac syndrome (PLCS) is a maladaptive response seen after surgical or transcatheter closure of hsPDA, defined as a vasopressor requirement with systolic blood pressure less than the 3rd percentile and ventilation and oxygenation failure.7, 9 PLCS poses unique challenges in the management of critically ill neonates, particularly premature infants. It generally occurs 6-12 hours post-closure. It is driven by an abrupt reduction in L-R shunting, causing increased afterload and a mismatch between ventricular contractile function (Ees) and the lumped sum measure of afterload (Ea). As a result, there is a decrement in LV function and a reduction in stroke volume. LV diastolic dysfunction can occur as well and often leads to worsening oxygenation and ventilation due to the presence of LA hypertension. Early use of TNE may identify neonates at risk of PLCS, evaluate residual PDA shunting, and detect immediate procedural complications such as pericardial/pleural effusion and tricuspid regurgitation (TR).9 LVO < 200 ml/kg/min in the first hour after PDA closure predicts cardiopulmonary instability and inotropic support requirement better than conventional measures such as ejection fraction (EF) or fractional shortening (FS).10 Other measures of LV performance, such as tissue Doppler imaging with measurement of myocardial velocity at the mitral annulus, and myocardial deformation analysis assessing GLS, may also provide helpful information to guide management after PDA closure.11 GLS has been proven to detect subtle regional and global LV dysfunction even before LVEF begins to decline. Interestingly, in adult patients with aortic stenosis and normal LVEF, decreased GLS, resulting from increased LV afterload, predicts the need for inotropic support after AVR.12 Pulmonary hypertension (PH) represents another complex hemodynamic condition encountered in the neonatal population, characterized by elevated pulmonary vascular resistance (PVR) and RV dysfunction. The management of PH in neonates requires a multimodal approach that addresses underlying etiologies, optimizes oxygenation and ventilation, reduces PVR, and prevents worsening of RV function. TNE plays a crucial role in monitoring response to therapy, identifying treatment failures, and guiding escalation or weaning of care.13 The key principles of echocardiographic assessment include:1)Determination of pulmonary artery pressure (PAP) and PVR. It must be determined whether PH is a consequence of excessive pulmonary blood flow (PBF) in an arterial bed with normal neonatal PVR or whether it is a consequence of more limited PBF into an arterial bed with elevated PVR. This distinction requires determination of the LVO/RVO ratio, the pulmonary vein (PV) flow profile, and the estimation of PAP using TR and PR jet velocities. The dynamic ventricular septal wall morphology, specifically an LV end-systolic eccentricity index (Els) ≥ 1.3, has an excellent inter-observer agreement in identifying PH in neonates.14 An indirect measure of PVR can be obtained with the RV ejection time/PA acceleration time (RVET/PAAT) ratio or alternatively the PAAT/RVET ratio. While LVO can be a useful surrogate measure of PBF, it is only reliable in the absence of intracardiac shunting.2)Biventricular function. Since qualitative estimation of ventricular function can be highly subjective, quantitative measurement with LVEF, RV FAC, TAPSE, tissue Doppler, and deformation imaging is recommended.3)PDA. The presence of a PDA in PH can be pathologic or supportive.13 When PH is due to excessive PBF or LA hypertension and the PAP is sub-systemic, a large PDA is pathologic as L-R shunting further increases PBF. In contrast, in severe PH with supra-systemic PAP, a PDA will offload the failing RV into the descending aorta. In this case, systemic cardiac output can be maintained with R-L shunting at the expense of reduced lower body oxygen saturation. The role of neonatal echocardiography in monitoring neonates during the "arch watch" has recently been reviewed.15 Undetected PDA closure in the presence of coarctation of the aorta (CoA) can lead to near complete or complete obstruction to distal aortic blood flow and cardiovascular collapse. In the presence of suspected CoA, serial TNE exams in conjunction with upper and lower extremity blood pressure differentials and pulse oximetry, can track the evolution of aortic anatomy and blood flow dynamics and identify infants requiring surgical intervention. Cardiac point-of-care ultrasound (cPOCUS) is another topic addressed in the updated guideline.3 This modality can provide clinicians with information to guide clinical decision-making at the bedside. cPOCUS allows for rapid, real-time evaluation of cardiopulmonary function and volume status, detection of pericardial and pleural effusions, and the position of umbilical vein and artery catheters. In addition to traditional echocardiographic parameters, novel techniques such as tissue Doppler imaging, myocardial deformation analysis using speckle tracking echocardiography (STE), and three-dimensional (3D) echocardiography have expanded the diagnostic capabilities of neonatal echocardiography. These advanced imaging modalities enable clinicians to assess myocardial mechanics and visualize cardiac anatomy in greater detail, enhancing our understanding of neonatal cardiovascular physiology and pathology. Despite its immense promise, several challenges associated with TNE limit its widespread adoption and utility in clinical practice. Limited availability of trained operators, variability in image quality, and cost constraints may hinder the routine use of TNE in resource-limited settings. Moreover, interpretation of echocardiographic findings requires expertise and experience, underscoring the need for standardized protocols and training programs to ensure consistency and accuracy. Technological innovations such as hardware and software updates, portable ultrasound devices with wireless connectivity, and artificial intelligence hold promise for improving accessibility, image quality, and diagnostic accuracy. Certainly, interdisciplinary collaboration between neonatologists, pediatric cardiologists, and echocardiographers is essential to maximize the utility of echocardiography and integrate it into routine clinical care pathways. TNE plays an indispensable role in the hemodynamic management of neonates. By leveraging the capabilities of echocardiography, clinicians can optimize hemodynamic management, improve clinical outcomes, and enhance the quality of care for critically ill neonates. Continued investment in training, technology, and research is essential to unlocking the full potential of TNE and refining neonatal hemodynamic management.

OBJECTIVES:Perioperative management strategies and outcomes for low-risk congenital heart disease (CHD) surgery vary between institutions. To date, no consensus exists on standardized management for pediatric patients undergoing cardiac surgery. This study seeks to benchmark the perioperative management of 4 common CHD lesions and explore clinical factors affecting postoperative outcomes. DESIGN:A retrospective review of CHD procedures performed between 2015 and 2020. SETTING:The study was conducted at a single academic tertiary pediatric hospital. PARTICIPANTS:All patients presenting for repair of ventricular septal defects (VSDs), complete atrioventricular canal defects, tetralogy of Fallot (TOF), and transposition of the great arteries (TGA) were reviewed. INTERVENTIONS:Demographic and clinical data were collected; clinical outcomes were defined as postoperative length of ventilation (LOV) and hospital length of stay, divided into reference and prolonged course groups analyzed for variables associated with differences in outcomes. MEASUREMENTS AND MAIN RESULTS:We selected 931 patients for review. Prolonged length of ventilation and length of stay in all cohorts were associated with longer operative, cardiopulmonary bypass, and cross-clamp times; higher intraoperative requirements for inotropic support; more blood transfusions and higher opioid administration; lower pH preoperatively and higher lactic acid postoperatively. Worse outcomes were associated with younger age in VSD, older age in TGA, and lower weight in TOF and TGA. Worse outcomes were also associated with a higher preoperative hematocrit in VSD and TOF and elevated preoperative blood glucose in VSD and TGA. CONCLUSIONS:A better understanding of clinical factors affecting outcomes may facilitate streamlining perioperative management strategies for pediatric patients undergoing low-risk cardiac surgery.

The authors have no conflicts of interest. Data sharing not applicable – no new data generated.

There is significant variability in postoperative neurological injury rates in patients with congenital heart disease, with early injuries impacting long-term neurodevelopmental outcomes; therefore, there is an urgent need for identifying effective strategies to mitigate such injuries.

Providing thorough care for children with congenital heart disease (CHD) undergoing non-cardiac surgery requires a strong understanding of common heart defects and the procedures used to treat them. To care for these patients, multidisciplinary teams must consider the severity of the underlying cardiac disease, comorbid conditions, and preoperative severity of illness. The American Heart Association's Scientific Statement on Perioperative Considerations for Pediatric Patients with Congenital Heart Disease Presenting for Noncardiac Procedures offers valuable guidance.