ALCAPA in Children with Complex Congenital Heart Disease: A Multicenter Study.

We sought to report characteristics and outcomes of children with complex anomalous left coronary artery from the pulmonary artery (ALCAPA) and to compare the outcomes to children with isolated ALCAPA. We performed a retrospective data analysis of children with ALCAPA who underwent cardiac surgery between 1/2009 and 3/2018 at 21 centers. Characteristics and outcomes of patients with complex ALCAPA are provided using descriptive statistics. Outcomes were compared between complex ALCAPA and isolated ALCAPA using Fisher's exact test. We reviewed 258 patients who underwent surgical repair of ALCAPA at 21 centers. We identified 10 patients (3.9%) with complex ALCAPA. Median age at initial cardiac surgery was 49 days (range: 4 days, 12.8 years). Cardiac lesions associated with ALCAPA were HLHS (n = 3); scimitar syndrome (n = 2); VSD with aortic coarctation (n = 2); VSD with right pulmonary artery discontinuity (1); DORV with mitral atresia (n = 1); and ToF (n = 1). ALCAPA was diagnosed prior to surgical intervention in 1 patient; during the initial cardiac surgery in 4 patients; in the early postoperative period via cardiac catheterization in 3 patients; and later in childhood after initial surgical repair in 2 patients. Following ALCAPA repair, patients with complex ALCAPA, as compared to patients with isolated ALCAPA, were more likely to be placed on ECMO (50% vs 12%, p = 0.002), receive CPR (30% vs 6%, p = 0.017), or suffer operative mortality (50% vs 3%, p < 0.001). Complex ALCAPA is uncommon. All but one with complex ALCAPA was not diagnosed preoperatively and postoperative morbidity and mortality were significantly greater in these complex patients compared to patients with isolated ALCAPA.

Clinical Outcomes After Tracheostomy in Children With Single Ventricle Physiology: Collaborative Research From the Pediatric Cardiac Intensive Care Society Multicenter Cohort, 2010-2021.

OBJECTIVES: Multicenter studies reporting outcomes following tracheostomy in children with congenital heart disease are limited, particularly in patients with single ventricle physiology. We aimed to describe clinical characteristics and outcomes in a multicenter cohort of patients with single ventricle physiology who underwent tracheostomy before Fontan operation. DESIGN: Multicenter retrospective cohort study.SETTING: Twenty-one tertiary care pediatric institutions participating in the Collaborative Research from the Pediatric Cardiac Intensive Care Society. PATIENTS: We reviewed 99 children with single ventricle physiology who underwent tracheostomy before the Fontan operation at 21 institutions participating in Collaborative Research from the Pediatric Cardiac Intensive Care Society between January 2010 and December 2020, with follow-up through December 31, 2021. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Death occurred in 51 of 99 patients (52%). Cox proportional hazard analysis was performed to determine factors associated with death after tracheostomy. Results are presented as hazard ratio (HR) with 95% CIs. Nonrespiratory indication(s) for tracheostomy (HR, 2.21; 95% CI, 1.14-4.32) and number of weeks receiving mechanical ventilation before tracheostomy (HR, 1.06; 95% CI, 1.02-1.11) were independently associated with greater hazard of death. In contrast, diagnosis of tricuspid atresia or Ebstein's anomaly was associated with less hazard of death (HR, 0.16; 95% CI, 0.04-0.69). Favorable outcome, defined as survival to Fontan operation or decannulation while awaiting Fontan operation with viable cardiopulmonary physiology, occurred in 29 of 99 patients (29%). Median duration of mechanical ventilation before tracheostomy was shorter in patients who survived to favorable outcome (6.1 vs. 12.1 wk; p < 0.001), and only one of 16 patients with neurologic indications for tracheostomy and 0 of ten patients with cardiac indications for tracheostomy survived to favorable outcome. CONCLUSIONS: For children with single ventricle physiology who undergo tracheostomy, mortality risk is high and should be carefully considered when discussing tracheostomy as an option for these children. Favorable outcomes are possible, although thoughtful attention to patient selection and tracheostomy timing are likely necessary to achieve this goal.

Factors associated with the need for inotropic support following pulmonary artery banding surgery for CHD.

OBJECTIVE: We aimed to identify factors independently associated with the need for inotropic support for low cardiac output or haemodynamic instability after pulmonary artery banding surgery for CHD. METHODS: We performed a retrospective chart review of all neonates and infants who underwent pulmonary banding between January 2016 and June 2019 at our institution. Bivariate and multivariable analyses were performed to identify factors independently associated with the use of post-operative inotropic support, defined as the initiation of inotropic infusion(s) for depressed myocardial function, hypotension, or compromised perfusion within 24 hours of pulmonary artery banding. RESULTS: We reviewed 61 patients. Median age at surgery was 10 days (25%,75%:7,30). Cardiac anatomy was biventricular in 38 patients (62%), hypoplastic right ventricle in 14 patients (23%), and hypoplastic left ventricle in 9 patients (15%). Inotropic support was implemented in 30 patients (49%). Baseline characteristics of patients who received inotropic support, including ventricular anatomy and pre-operative ventricular function, were not statistically different from the rest of the cohort. Patients who received inotropic support, however, were exposed to larger cumulative doses of ketamine intraoperatively - median 4.0 mg/kg (25%,75%:2.8,5.9) versus 1.8 mg/kg (25%,75%:0.9,4.5), p < 0.001. In a multivariable model, cumulative ketamine dose greater than 2.5mg/kg was associated with post-operative inotropic support (odds ratio 5.5; 95% confidence interval: 1.7,17.8), independent of total surgery time. CONCLUSIONS: Inotropic support was administered in approximately half of patients who underwent pulmonary artery banding and more commonly occurred in patients who received higher cumulative doses of ketamine intraoperatively, independent of the duration of surgery.

Staged vs Complete Repair in Tetralogy of Fallot With Pulmonary Atresia.

BACKGROUND: We sought to compare outcomes for infants with tetralogy of Fallot with pulmonary atresia (TOF/PA) and confluent pulmonary arteries who underwent staged or primary complete surgical repair. METHODS: This retrospective study included infants undergoing initial surgical intervention between 0 and 60 days of age with TOF/PA without aortopulmonary collaterals from 2009 to 2018 at 20 centers. The primary outcome was days alive and out of the hospital in the first year of life (DAOH365). Secondary outcomes were mortality at 1 year of age and a composite major complication outcome. Multivariable modeling with generalized estimating equations were used to compare outcomes between groups. RESULTS: Of 221 subjects, 142 underwent staged repair and 79 underwent primary complete repair. There was no significant difference in median DAOH365 between the staged and primary repair groups (317 days [interquartile range, 278-336] vs 338 days [interquartile range, 314-348], respectively; adjusted P = .13). Nine staged repair patients (7%) died in the first year of life vs 5 primary repair patients (6%; adjusted odds ratio, 1.00; 95% CI, 0.25-3.95). At least 1 major complication occurred in 37% of patients who underwent staged repair vs 41% of patients who underwent primary complete repair (P = .75), largely driven by the need for unplanned cardiac reinterventions. CONCLUSIONS: For infants with TOF/PA with confluent pulmonary arteries, a surgical strategy of staged or primary complete repair resulted in statistically similar DAOH365, early mortality, and morbidity.

Operational Definitions Related to Pediatric Ventilator Liberation.

BACKGROUND: Common, operational definitions are crucial to assess interventions and outcomes related to pediatric mechanical ventilation. These definitions can reduce unnecessary variability among research and quality improvement efforts, to ensure findings are generalizable, and can be pooled to establish best practices. RESEARCH QUESTION: Can we establish operational definitions for key elements related to pediatric ventilator liberation using a combination of detailed literature review and consensus-based approaches? STUDY DESIGN AND METHODS: A panel of 26 international experts in pediatric ventilator liberation, two methodologists, and two librarians conducted systematic reviews on eight topic areas related to pediatric ventilator liberation. Through a series of virtual meetings, we established draft definitions that were voted upon using an anonymous web-based process. Definitions were revised by incorporating extracted data gathered during the systematic review and discussed in another consensus meeting. A second round of voting was conducted to confirm the final definitions. RESULTS: In eight topic areas identified by the experts, 16 preliminary definitions were established. Based on initial discussion and the first round of voting, modifications were suggested for 11 of the 16 definitions. There was significant variability in how these items were defined in the literature reviewed. The final round of voting achieved ≥ 80% agreement for all 16 definitions in the following areas: what constitutes respiratory support (invasive mechanical ventilation and noninvasive respiratory support), liberation and failed attempts to liberate from invasive mechanical ventilation, liberation from respiratory support, duration of noninvasive respiratory support, total duration of invasive mechanical ventilation, spontaneous breathing trials, extubation readiness testing, 28 ventilator-free days, and planned vs rescue use of post-extubation noninvasive respiratory support. INTERPRETATION: We propose that these consensus-based definitions for elements of pediatric ventilator liberation, informed by evidence, be used for future quality improvement initiatives and research studies to improve generalizability and facilitate comparison.

Executive Summary: International Clinical Practice Guidelines for Pediatric Ventilator Liberation, A Pediatric Acute Lung Injury and Sepsis Investigators (PALISI) Network Document.

Rationale: Pediatric-specific ventilator liberation guidelines are lacking despite the many studies exploring elements of extubation readiness testing. The lack of clinical practice guidelines has led to significant and unnecessary variation in methods used to assess pediatric patients' readiness for extubation. Methods: Twenty-six international experts comprised a multiprofessional panel to establish pediatrics-specific ventilator liberation clinical practice guidelines, focusing on acutely hospitalized children receiving invasive mechanical ventilation for more than 24 hours. Eleven key questions were identified and first prioritized using the Modified Convergence of Opinion on Recommendations and Evidence. A systematic review was conducted for questions that did not meet an a priori threshold of ⩾80% agreement, with Grading of Recommendations, Assessment, Development, and Evaluation methodologies applied to develop the guidelines. The panel evaluated the evidence and drafted and voted on the recommendations. Measurements and Main Results: Three questions related to systematic screening using an extubation readiness testing bundle and a spontaneous breathing trial as part of the bundle met Modified Convergence of Opinion on Recommendations criteria of ⩾80% agreement. For the remaining eight questions, five systematic reviews yielded 12 recommendations related to the methods and duration of spontaneous breathing trials, measures of respiratory muscle strength, assessment of risk of postextubation upper airway obstruction and its prevention, use of postextubation noninvasive respiratory support, and sedation. Most recommendations were conditional and based on low to very low certainty of evidence. Conclusions: This clinical practice guideline provides a conceptual framework with evidence-based recommendations for best practices related to pediatric ventilator liberation.

High variability in cardiac education and experiences during United States paediatric critical care fellowships.

BACKGROUND: Paediatric cardiac critical care continues to become more sub-specialised, and many institutions have transitioned to dedicated cardiac ICUs. Literature regarding the effects of these changes on paediatric critical care medicine fellowship training is limited. OBJECTIVE: To describe the current landscape of cardiac critical care education during paediatric critical care medicine fellowship in the United States and demonstrate its variability. METHODS: A review of publicly available information in 2021 was completed. A supplemental REDCap survey focusing on cardiac ICU experiences during paediatric critical care medicine fellowships was e-mailed to all United States Accreditation Council of Graduate Medical Education-accredited paediatric critical care medicine fellowship programme coordinators/directors. Results are reported using inferential statistics. RESULTS: Data from 71 paediatric critical care medicine fellowship programme websites and 41 leadership responses were included. Median fellow complement was 8 (interquartile range: 6, 12). The majority (76%, 31/41) of programmes had a designated cardiac ICU. Median percentage of paediatric critical care medicine attending physicians with cardiac training was 25% (interquartile range: 0%, 69%). Mandatory cardiac ICU time was 16 weeks (interquartile range: 13, 20) with variability in night coverage and number of other learners present. A minority of programmes (29%, 12/41) mandated other cardiac experiences. Median CHD surgical cases per year were 215 (interquartile range: 132, 338). When considering the number of annual cases per fellow, programmes with higher case volume were not always associated with the highest case number per fellow. CONCLUSIONS: There is a continued trend toward dedicated cardiac ICUs in the United States, with significant variability in cardiac training during paediatric critical care medicine fellowship. As the trend toward dedicated cardiac ICUs continues and practices become more standardised, so should the education.

Contemporary Multicenter Outcomes for Truncus Arteriosus With Interrupted Aortic Arch.

BACKGROUND: Truncus arteriosus with interrupted aortic arch (TA-IAA) is a rare congenital heart defect with historically poor outcomes. Contemporary multicenter data are limited. METHODS: A retrospective cohort study of children who underwent repair of TA-IAA between 2009 and 2016 at 12 tertiary care referral centers within the United States was performed. Major adverse cardiac events (MACE) were defined as postoperative extracorporeal membrane oxygenation, cardiopulmonary resuscitation, or operative mortality. TA-IAA patients were compared with TA patients who underwent repair during the study period from the same institutions. RESULTS: We reviewed 35 patients with TA-IAA. MACE occurred in 12 patients (34%). Improvement over time was observed during the study period with 11 events (92%) occurring in the first half of the study period (P = .03). Factors associated with MACE included moderate or severe truncal valve insufficiency (P < .01), concomitant truncal valve repair (P = .04), and longer cardiopulmonary bypass duration (P = .02). In comparison with 216 patients who underwent TA repair, patients with TA-IAA had a higher rate of MACE, but this finding was not statistically significant (34% vs 20%, respectively; P = .07). Additionally no differences between TA-IAA and TA groups were observed for unplanned reoperations (14% vs 22%, respectively; P = .3), hospital length of stay (24 vs 23 days, P = .65), or late deaths (7% vs 7%, P = 1.00). CONCLUSIONS: In this contemporary, multicenter cohort the rate of MACE after repair of TA-IAA was high but improved during the study period. Early childhood outcomes of patients with TA-IAA were similar to those with TA.

Procedural Outcomes of Pulmonary Atresia With Intact Ventricular Septum in Neonates: A Multicenter Study.

BACKGROUND: Multicenter contemporary data describing short-term outcomes after initial interventions of neonates with pulmonary atresia with intact ventricular septum (PA-IVS) are limited. This multicenter study describes characteristics and outcomes of PA-IVS neonates after their initial catheter or surgical intervention and identifies factors associated with major adverse cardiac events (MACE). METHODS: Neonates with PA-IVS who underwent surgical or catheter intervention between 2009 and 2019 in 19 centers were reviewed. Risk factors for MACE, defined as cardiopulmonary resuscitation, mechanical circulatory support, stroke, or in-hospital mortality, were analyzed using multivariable logistic regression models. RESULTS: We reviewed 279 neonates: 79 (28%) underwent right ventricular decompression, 151 (54%) underwent systemic-to-pulmonary shunt or ductal stent placement only, 36 (13%) underwent right ventricular decompression with shunt or ductal stent placement, and 11 (4%) underwent transplantation. MACE occurred in 57 patients (20%): 26 (9%) received mechanical circulatory support, 37 (13%) received cardiopulmonary resuscitation, stroke occurred in 16 (6%), and 23 (8%) died. The presence of 2 major coronary artery stenoses (adjusted odds ratio, 4.99; 95% CI, 1.16-21.39) and lower weight at first intervention (adjusted odds ratio, 1.52; 95% CI, 1.01-2.27) were significantly associated with MACE. Coronary ischemia was the most frequent presumed mechanism of death (n = 10). CONCLUSIONS: In a multicenter cohort, 1 in 5 neonates with PA-IVS experienced MACE after their initial intervention. Patients with 2 major coronary artery stenoses or lower weight at the time of the initial procedure were most likely to experience MACE and warrant vigilance during preintervention planning and postintervention management.

Multi-Lobar Atelectasis in Children Receiving Venoarterial Extracorporeal Membrane Oxygenation for Cardiac Indications.

BACKGROUND: Respiratory complications are common in patients who require venoarterial (VA) extracorporeal membrane oxygenation (ECMO) for cardiac indications. We aimed to examine the frequency and characteristics of patients who develop multi-lobar atelectasis early in the course of VA ECMO and to identify factors associated with its occurrence. METHODS: We performed a single-center retrospective review of consecutive pediatric subjects on VA ECMO in the cardiovascular ICU from 2014 to 2019. Chest radiographs before VA ECMO initiation and daily for up to 5 d of VA ECMO support were reviewed. Multi-lobar atelectasis was defined as the collapse of ≥2 lobes of the same lung. Patients with multi-lobar atelectasis before or immediately after VA ECMO cannulation were excluded. Bivariate comparisons and multivariable logistic regression analyses were performed to identify factors independently associated with lung collapse. Results of the multivariable analysis are provided as odds ratio (OR) with 95% CI. RESULTS: We reviewed 119 VA ECMO runs in 101 unique subjects. Multi-lobar atelectasis occurred in 36 runs (30%), with an isolated collapse of the left lung occurring most frequently (no. runs = 20). VA ECMO runs complicated by multi-lobar atelectasis were significantly longer and associated with lower hospital survival (51% vs 77%, survival in subjects without multi-lobar atelectasis; P = .01). Multivariable logistic regression analysis identified a subsequent ECMO run during the same admission to be independently associated with multi-lobar atelectasis (OR 5.4, 95% CI 1.2-21.5). Subanalysis of the subjects with isolated left lung collapse revealed male sex (OR 8.9, 95% CI 1.6-48.2) and subsequent ECMO run during the same admission (OR 4.0, 95% CI 1.2-13.6) to be independently associated with this complication, and mechanical ventilation at least 12 h before ECMO may be protective (OR 0.22, 95% CI 0.07-0.76). CONCLUSIONS: Multi-lobar atelectasis commonly occurred in children who were receiving VA ECMO for cardiac failure and was associated with worse outcomes. Male patients, a subsequent VA ECMO run during the same hospitalization, and patients in whom mechanical ventilation was initiated shortly before ECMO cannulation may be at increased risk for this complication.

Cardiac Arrest in Children Following Cardiac Surgery: A Scoping Review of Contributing Factors.

Nearly half of children experiencing cardiac arrest following cardiac surgery do not survive hospital discharge and patients who survive often experience significant neurological impairment. Additionally, increased resource utilization following cardiac arrest translates into adverse logistical and financial consequences. Although some studies have identified patient characteristics that increase the risk of cardiac arrest after pediatric cardiac surgery, modifiable risk factors, which could provide a foundation for effective prevention strategies, have been elusive. This scoping review explores the current knowledge surrounding risk factors associated with cardiac arrest in children following cardiac surgery and provides recommendations for future research.

Pulmonary Atresia with Intact Ventricular Septum: Midterm Outcomes from a Multicenter Cohort.

Contemporary multicenter data regarding midterm outcomes for neonates with pulmonary atresia with intact ventricular septum are lacking. We sought to describe outcomes in a contemporary multicenter cohort, determine factors associated with end-states, and evaluate the effect of right ventricular coronary dependency and coronary atresia on transplant-free survival. Neonates treated during 2009-2019 in 19 United States centers were reviewed. Competing risks analysis was performed to determine cumulative risk of each end-state, and multivariable regression analyses were performed to identify factors associated with each end-state and transplant-free survival. We reviewed 295 patients. Median tricuspid valve Z-score was - 3.06 (25%, 75%: - 4.00, - 1.52). Final end-state was biventricular repair for 45 patients (15.2%), one-and-a half ventricle for 16 (5.4%), Fontan for 75 (25.4%), cardiac transplantation for 29 (9.8%), and death for 54 (18.3%). Seventy-six patients (25.7%) remained in mixed circulation. Cumulative risk estimate of death was 10.9%, 16.1%, 16.9%, and 18.8% at 1, 6 months, 1 year, and 5 years, respectively. Tricuspid valve Z-score was inversely, and coronary atresia positively associated with death or transplantation [odds ratio (OR) = 0.46, (95% confidence interval (CI) = 0.29-0.75, p < 0.001) and OR = 3.75 (95% CI 1.46-9.61, p = 0.011), respectively]. Right ventricular coronary dependency and left coronary atresia had a significant effect on transplant-free survival (log-rank p < 0.001). In a contemporary multicenter cohort of patients with PAIVS, consisting predominantly of patients with moderate-to-severe right ventricular hypoplasia, we observed favorable survival outcomes. Right ventricular coronary dependency and left, but not right, coronary atresia significantly worsens transplant-free survival.

Seizures in Children with Cardiac Disease on Extracorporeal Membrane Oxygenation.

BACKGROUND: Children supported with extracorporeal membrane oxygenation (ECMO) have been shown to be at risk for developing seizures. However, previous studies have consisted of heterogeneous patient populations. We aimed to describe the rate of seizures in pediatric patients receiving ECMO for cardiac indications and to identify risk factors for the occurrence of this complication. METHODS: This is a retrospective cohort study of consecutive pediatric patients on ECMO for congenital or acquired cardiac disease between 2014 and 2018 at a tertiary care pediatric hospital. RESULTS: We reviewed 110 children, of whom 104 (95%) received continuous electroencephalogram for at least 48 h after ECMO initiation. Seizures were observed in 20 (18%) children. Seizures were subclinical only in 13 (65%) patients, and 8 (40%) developed status epilepticus. The median time from ECMO initiation to first seizure was 34 h (25%, 75%: 19, 44). Children with seizures were more likely to have suffered pre-ECMO cardiac arrest (odds ratio 5.7, 95% confidence interval 2.0-16.1, p < 0.001), require extracorporeal cardiopulmonary resuscitation (odds ratio 5.2, 95% confidence interval 1.9-14.7, p < 0.001), and have been cannulated via the cervical vessels (p = 0.029). Children with seizures also had lower pH nadir prior to ECMO (p = 0.015) and had higher peak lactate prior to ECMO (p = 0.002). Patients with seizures had significantly a longer median intensive care unit length of stay, (43 versus 32 days, p = 0.02), had a significantly worse pediatric cerebral performance score (2 versus 1, p = 0.03), and tended to have worse survival to hospital discharge (50% versus 71%, p = 0.069). CONCLUSIONS: Seizures in pediatric patients on ECMO for cardiac indications are common, occurring in nearly one in five patients. Seizures are frequently subclinical only and often progress to status epilepticus. Continuous electroencephalogram is therefore warranted for this patient population, especially in the setting of cardiac arrest, extracorporeal cardiopulmonary resuscitation, or severe metabolic acidosis.

Rest ventilator management in children on veno-venous extracorporeal membrane oxygenation.

BACKGROUND: We aimed to use the Extracorporeal Life Support Organization registry to describe the current practice of rest mechanical ventilation setting in children receiving veno-venous extracorporeal membrane oxygenation (V-V ECMO) and to determine if relationships exist between ventilator settings and mortality. METHODS: Data for patients 14 days to 18 years old who received V-V ECMO from 2012-2016 were reviewed. Mechanical ventilation data available includes mode and settings at 24 h after ECMO cannulation. Multivariable logistic regression analysis was performed to determine if rest settings were associated with mortality. RESULTS: We reviewed 1161 subjects, of which 1022 (88%) received conventional mechanical ventilation on ECMO. Rest settings, expressed as medians (25th%, 75th%), are as follows: rate 12 breaths/minute (10, 17); peak inspiratory pressure (PIP) 22 cmH2O (20,27); positive end expiratory pressure (PEEP) 10 cmH2O (8, 10); and fraction of inspired oxygen (FiO2) 0.4 (0.37, 0.60). Survival to discharge was 68%. Higher ventilator FiO2 (odds ratio:1.13 per 0.1 increase, 95% confidence interval:1.04, 1.23), independent of arterial oxygen saturation, was associated with mortality. CONCLUSIONS: Current rest ventilator management for children receiving V-V ECMO primarily relies on conventional mechanical ventilation with moderate amounts of PIP, PEEP, and FiO2. Further study on the relationship between FiO2 and mortality should be pursued.

Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery: A Retrospective Multicenter Study.

OBJECTIVES: We aimed to describe characteristics and operative outcomes from a multicenter cohort of infants who underwent repair of anomalous left coronary artery from the pulmonary artery. We also aimed to identify factors associated with major adverse cardiovascular events following anomalous left coronary artery from the pulmonary artery repair. DESIGN: Retrospective chart review. SETTING: Twenty-one tertiary-care referral centers. PATIENTS: Infants less than 365 days old who underwent anomalous left coronary artery from the pulmonary artery repair. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Major adverse cardiovascular events were defined as the occurrence of postoperative extracorporeal membrane oxygenation, cardiopulmonary resuscitation, left ventricular assist device, heart transplantation, or operative mortality. Factors independently associated with major adverse cardiovascular events were identified using multivariable logistic regression analysis. We reviewed 177 infants (< 365 d old) who underwent anomalous left coronary artery from the pulmonary artery repair between January 2009 and March 2018. Major adverse cardiovascular events occurred in 36 patients (20%). Twenty-nine patients (16%) received extracorporeal membrane oxygenation, 14 (8%) received cardiopulmonary resuscitation, four (2%) underwent left ventricular assist device placement, two (1%) underwent heart transplantation, and six (3.4%) suffered operative mortality. In multivariable analysis, preoperative inotropic support (odds ratio, 3.5; 95% CI, 1.4-8.5), cardiopulmonary bypass duration greater than 150 minutes (odds ratio, 6.9 min; 95% CI, 2.9-16.7 min), and preoperative creatinine greater than 0.3 mg/dL (odds ratio, 2.4 mg/dL; 95% CI, 1.1-5.6 mg/dL) were independently associated with major adverse cardiovascular events. In patients with preoperative left ventricular end-diastolic diameter measurements available (n = 116), left ventricular end-diastolic diameter z score greater than 6 was also independently associated with major adverse cardiovascular events (odds ratio, 7.6; 95% CI, 2.0-28.6). CONCLUSIONS: In this contemporary multicenter analysis, one in five children who underwent surgical repair of anomalous left coronary artery from the pulmonary artery experienced major adverse cardiovascular events. Preoperative characteristics such as inotropic support, creatinine, and left ventricular end-diastolic diameter z score should be considered when planning for potential postoperative complications.

Development and Validation of a Seizure Prediction Model in Neonates After Cardiac Surgery.

BACKGROUND: Electroencephalographic seizures (ESs) after neonatal cardiac surgery are often subclinical and have been associated with poor outcomes. An accurate ES prediction model could allow targeted continuous electroencephalographic monitoring (CEEG) for high-risk neonates. METHODS: ES prediction models were developed and validated in a multicenter prospective cohort where all postoperative neonates who underwent cardiopulmonary bypass (CPB) also underwent CEEG. RESULTS: ESs occurred in 7.4% of neonates (78 of 1053). Model predictors included gestational age, head circumference, single-ventricle defect, deep hypothermic circulatory arrest duration, cardiac arrest, nitric oxide, extracorporeal membrane oxygenation, and delayed sternal closure. The model performed well in the derivation cohort (c-statistic, 0.77; Hosmer-Lemeshow, P = .56), with a net benefit (NB) over monitoring all and none over a threshold probability of 2% in decision curve analysis (DCA). The model had good calibration in the validation cohort (Hosmer-Lemeshow, P = .60); however, discrimination was poor (c-statistic, 0.61), and in DCA there was no NB of the prediction model between the threshold probabilities of 8% and 18%. By using a cut point that emphasized negative predictive value in the derivation cohort, 32% (236 of 737) of neonates would not undergo CEEG, including 3.5% (2 of 58) of neonates with ESs (negative predictive value, 99%; sensitivity, 97%). CONCLUSIONS: In this large prospective cohort, a prediction model of ESs in neonates after CPB had good performance in the derivation cohort, with an NB in DCA. However, performance in the validation cohort was weak, with poor discrimination, poor calibration, and no NB in DCA. These findings support CEEG of all neonates after CPB.

Characteristics and Surgical Outcomes of Patients With Late Presentation of Anomalous Left Coronary Artery From the Pulmonary Artery: A Multicenter Study.

We sought to describe the clinical course and outcomes of patients who are diagnosed with anomalous left coronary artery from the pulmonary artery (ALCAPA) after infancy. We conducted a retrospective evaluation of patients who underwent ALCAPA surgery between January 2009 to March 2018 at 21 US centers. Clinical presentation, inpatient management, and postoperative outcomes of patients repaired ≥1 year of age were described. To characterize this cohort, we compared these data to patients repaired before 1 year of age. Of 248 ALCAPA patients, 71 (29%) underwent repair ≥1 year of age. Among this subset, the median age at diagnosis was 8.3 years. Chronic arrhythmia occurred in 7%. Patients had good postoperative recovery of left ventricle (LV) dysfunction (90%) and LV dilation (75%), although a low incidence of recovery of mitral regurgitation (40%). Compared to infants, older patients were more likely to present with cardiac arrest (11% vs 1%) and less likely to have moderate or worse LV dysfunction or mitral regurgitation. Older patients had significantly less postoperative extracorporeal membrane oxygenation use, and shorter ICU and hospital stay. In the older cohort, operative mortality occurred in only 1 patient and no patient died after discharge (median follow-up 2.7 years). Survival of patients who presented with ALCAPA beyond infancy was excellent, although chronic mitral regurgitation and chronic arrhythmia were not uncommon. Patients who underwent ALCAPA repair ≥1 year of age were less likely to present with LV dysfunction but more likely to present with cardiac arrest than younger patients.

Effects of Vasopressin Infusion After Pediatric Cardiac Surgery: A Meta-analysis.

Vasopressin has been used to augment blood pressure; however, cardiovascular effects after cardiac surgery have not been well established. The primary objective of this study was to survey the current literature and quantify the pooled effect of vasopressin on hemodynamic parameters in children after pediatric cardiac surgery. A systematic review was conducted to identify studies characterizing the hemodynamic effects of vasopressin after pediatric cardiac surgery. Studies were assessed and those of satisfactory quality with pre- and post-vasopressin hemodynamics for each patient were included in the final analyses. 6 studies with 160 patients were included for endpoints during the first 2 h of infusions. Patients who received vasopressin infusion had greater mean, systolic, and diastolic blood pressures and lower heart rates at 2 h after initiation. 8 studies with 338 patients were included for the effects at 24 h. Patients who received vasopressin infusion had lower central venous pressures and decreased lactate concentrations 24 h after initiation. A subset analysis for children with functionally univentricular hearts found significant decrease in inotrope score and central venous pressure. A subset analysis for neonates found significant decrease in inotrope score and fluid balance. Vasopressin leads to decrease in heart rate and increase in blood pressure in the first 2 h of initiation. Later effects include decrease in inotrope score, central venous pressure, fluid balance, and in lactate within the first 24 h. Findings vary in neonates and in those with functionally univentricular hearts although beneficial effects are noted in both.

Intermediate Outcomes After Repair of Anomalous Left Coronary Artery From the Pulmonary Artery.

BACKGROUND: Multicenter studies on infants with anomalous left coronary artery from the pulmonary artery (ALCAPA) are lacking. We report the intermediate-term outcomes after ALCAPA repair in a multicenter cohort and identify risk factors for reintervention or death after discharge. METHODS: We retrospectively reviewed infants under 1 year of age who underwent ALCAPA repair from January 2009 to March 2018 at 21 US centers. The primary composite outcome was freedom from reintervention or death after discharge. We used the Kaplan-Meier survival analysis to examine freedom from reintervention or death and the Cox proportional hazard analysis to identify risk factors for this composite outcome. RESULTS: One hundred seventy-seven infants underwent ALCAPA repair; 170 (97%) survived to hospital discharge without transplantation. Twenty-three patients were lost to follow-up. The median duration of follow-up in the remaining 147 patients was 3.8 years (25%, 75%: 1.9 years, 6.0 years). Echocardiographic data were available at ∼3 years after discharge in 98 patients. Left ventricular function was normal in 96 patients (98%), whereas 26 patients (27%) had greater than mild mitral valve regurgitation. Sixteen patients (11%) underwent 20 reinterventions with 1 late death. Patients undergoing the Takeuchi procedure or atypical repairs (hazard ratio, 8.0; 95% confidence interval, 2.1-30.0) or with moderate or greater mitral regurgitation on discharge echocardiogram (hazard ratio, 3.4; 95% confidence interval, 1.2-9.1) were at increased risk for reintervention. CONCLUSIONS: Intermediate-term outcomes after ALCAPA repair in infants are favorable. Persistent left ventricular dysfunction and reinterventions were uncommon, and mortality was rare. Patients who required atypical surgical repair or had moderate or greater mitral regurgitation at discharge warrant closer follow-up.

Analysis of factors associated with prolonged post-operative course after surgical repair of aortic coarctation.

OBJECTIVE: We sought to describe patient characteristics associated with prolonged post-operative length of stay in a contemporary cohort of infants who underwent isolated repair of aortic coarctation. METHODS: We reviewed patients less than 1 year of age who underwent isolated repair of aortic coarctation at our institution from 2009 to 2016. Prolonged post-operative length of stay was defined as length of stay within the upper tertile for the cohort. Bivariate and multi-variable analyses were performed to determine independent risk factors for prolonged length of stay. RESULTS: We reviewed 95 consecutive patients who underwent isolated repair of aortic coarctation, of whom 71 were neonates at the time of diagnosis. The median post-operative length of stay was 6.5 days. The upper tertile for post-operative length of stay was greater than 10 days; 32 patients within this tertile and 1 patient who died at 8.5 days after surgery were analysed as having prolonged post-operative length of stay. In a multi-variable analysis, pre-maturity (odds ratio: 3.5, 95% confidence interval: 1.2, 10.7), genetic anomalies (odds ratio: 4.7, 95% confidence interval: 1.2, 18), absence of pre-operative oral feeding (odds ratio: 7.4, 95% confidence interval: 2.4, 22.3), and 12-hour vasoactive-ventilation-renal score greater than 25 (odds ratio: 7.4, 95% confidence interval: 1.9, 29) were independently associated with prolonged length of stay. CONCLUSIONS: In neonates and infants who underwent isolated repair of aortic coarctation, pre-maturity, genetic anomalies, lack of pre-operative oral feedings, and 12-hour vasoactive-ventilation-renal score more than 25 were independent risk factors for prolonged post-operative length of stay. Further study on the relationship between pre-operative oral feedings and post-operative length of stay should be pursued.