Anticoagulant Medications: The Pediatric Extracorporeal Membrane Oxygenation Anticoagulation CollaborativE Consensus Conference.

OBJECTIVES: To derive systematic-review informed, modified Delphi consensus regarding the medications used for anticoagulation for pediatric extracorporeal membrane oxygenation (ECMO) for the Pediatric ECMO Anticoagulation CollaborativE (PEACE). DATA SOURCES: A structured literature search was performed using PubMed, EMBASE, and Cochrane Library (CENTRAL) databases from January 1988 to May 2021. STUDY SELECTION: Included studies assessed anticoagulation used in pediatric ECMO. DATA EXTRACTION: Two authors reviewed all citations independently, with a third reviewer adjudicating any conflicts. Eighteen references were used for data extraction as well as for creation of recommendations. Evidence tables were constructed using a standardized data extraction form. DATA SYNTHESIS: Risk of bias was assessed using the Quality in Prognosis Studies tool. The evidence was evaluated using the Grading of Recommendations Assessment, Development, and Evaluation system. Forty-eight experts met over 2 years to develop evidence-informed recommendations and, when evidence was lacking, expert-based consensus statements, or good practice statements for anticoagulation during pediatric ECMO. A web-based modified Delphi process was used to build consensus via the Research and Development/University of California Appropriateness Method. Consensus was based on a modified Delphi process with agreement defined as greater than 80%. Two recommendations, two consensus statements, and one good practice statement were developed, and, in all, agreement greater than 80% was reached. CONCLUSIONS: There is insufficient evidence to formulate optimal anticoagulation therapy during pediatric ECMO. Additional high-quality research is needed to inform evidence-based practice for anticoagulation during pediatric ECMO.

Antifibrinolytic and Adjunct Hemostatic Agents: The Pediatric Extracorporeal Membrane Oxygenation Anticoagulation CollaborativE Consensus Conference.

OBJECTIVES: To derive systematic-review informed, modified Delphi consensus regarding antifibrinolytic and adjunct hemostatic agents in neonates and children supported with extracorporeal membrane oxygenation (ECMO) for the Pediatric ECMO Anticoagulation CollaborativE consensus conference. DATA SOURCES: A structured literature search was performed using PubMed, EMBASE, and Cochrane Library (CENTRAL) databases from January 1988 to May 2021. STUDY SELECTION: Use of antifibrinolytics (epsilon-aminocaproic acid [EACA] or tranexamic acid), recombinant factor VII activated (rFVIIa), or topical hemostatic agents (THAs). DATA EXTRACTION: Two authors reviewed all citations independently, with a third independent reviewer resolving conflicts. Eleven references were used for data extraction and informed recommendations. Evidence tables were constructed using a standardized data extraction form. MEASUREMENTS AND MAIN RESULTS: Risk of bias was assessed using the Quality in Prognosis Studies tool. The evidence was evaluated using the Grading of Recommendations Assessment, Development, and Evaluation system. Forty-eight experts met over 2 years to develop evidence-based recommendations and, when evidence was lacking, expert-based consensus statements for the management of bleeding and thrombotic complications in pediatric ECMO patients. A web-based modified Delphi process was used to build consensus via the Research And Development/University of California Appropriateness Method. Consensus was defined as greater than 80% agreement. One weak recommendation and three consensus statements are presented. CONCLUSIONS: Evidence supporting recommendations for administration of antifibrinolytics (EACA or tranexamic acid), rFVIIa, and THAs were sparse and inconclusive. Much work remains to determine effective and safe usage strategies.

Executive Summary: The Pediatric Extracorporeal Membrane Oxygenation Anticoagulation CollaborativE (PEACE) Consensus Conference.

OBJECTIVES: To present recommendations and consensus statements with supporting literature for the clinical management of neonates and children supported with extracorporeal membrane oxygenation (ECMO) from the Pediatric ECMO Anticoagulation CollaborativE (PEACE) consensus conference. DATA SOURCES: Systematic review was performed using PubMed, Embase, and Cochrane Library (CENTRAL) databases from January 1988 to May 2021, followed by serial meetings of international, interprofessional experts in the management ECMO for critically ill children. STUDY SELECTION: The management of ECMO anticoagulation for critically ill children. DATA EXTRACTION: Within each of eight subgroup, two authors reviewed all citations independently, with a third independent reviewer resolving any conflicts. DATA SYNTHESIS: A systematic review was conducted using MEDLINE, Embase, and Cochrane Library databases, from January 1988 to May 2021. Each panel developed evidence-based and, when evidence was insufficient, expert-based statements for the clinical management of anticoagulation for children supported with ECMO. These statements were reviewed and ratified by 48 PEACE experts. Consensus was obtained using the Research and Development/UCLA Appropriateness Method. Results were summarized using the Grading of Recommendations Assessment, Development, and Evaluation method. We developed 23 recommendations, 52 expert consensus statements, and 16 good practice statements covering the management of ECMO anticoagulation in three broad categories: general care and monitoring; perioperative care; and nonprocedural bleeding or thrombosis. Gaps in knowledge and research priorities were identified, along with three research focused good practice statements. CONCLUSIONS: The 91 statements focused on clinical care will form the basis for standardization and future clinical trials.

Use of a Postoperative Care Management Pathway Reduces the Incidence of Chylothorax Post-Fontan Palliation.

Pleural effusions and chylothorax are challenging morbidities post-Fontan palliation. We sought to evaluate the efficacy of our Fontan Care Pathway (FCP) in reducing the incidence of post-operative chylothorax and Time to Chest Tube Removal (TTCTR), and to determine risk factors associated with longer TTCTR. Between 2016 and 2022 our institutional approach to post-Fontan care fell into three categories: Group 1 (n = 36): no standardized approach; Group 2 (n = 30): a prophylactic chylothorax diet (fat content < 5%); Group 3 (n = 57): the FCP (a chylothorax diet, fluid restriction, supplemental O2 and aggressive diuresis). The incidence of chylothorax and TTCTR was compared between groups. Predictors of TTCTR were analyzed using linear regression modelling, adjusting for covariates. Chylothorax rate decreased in Group 3 compared to Groups 1 and 2 (9% vs. 28% and 33% respectively, p = 0.011), without alteration in TTCTR. Univariate factors associated with median TTCTR included chylothorax (+ 13.7 days, p = 0.001), additional procedures at time of Fontan (+ 2.4 days per procedure p = 0.017), Fontan revision or takedown (+ 11.7 days, p = 0.018) and minor/major complications (+ 5.1, p = 0.01 and + 15.8, p < 0.001, respectively). On multivariable analysis, chylothorax (+ 6.5 days, p = 0.005) and major complications (+ 15.8 days, p = 0.001) were associated with increased TTCTR. When chylothorax was excluded from multivariable analysis, the FCP showed a significant decrease in TTCTR (- 3.3 days, p = 0.034). A bundled therapy approach was associated with reduced laboratory confirmed chylothorax post-Fontan, whereas diet change alone was not. Additional studies in this area, with larger sample sizes are warranted.

Bivalirudin in pediatric extracorporeal membrane oxygenation.

PURPOSE OF REVIEW: This review summarizes the current literature surrounding the use of bivalirudin as an alternative anticoagulant for pediatric extracorporeal membrane oxygenation (ECMO) patients. RECENT FINDINGS: Recent single center studies describe that bivalirudin may be associated with decreased blood product transfusion, decreased cost and similar clinical outcomes for pediatric ECMO patients who have failed unfractionated heparin (UFH) anticoagulation. aPTT is the most common test to monitor bivalirudin but has several limitations. Other tests including dilute thrombin time (dTT) and viscoelastic assays are promising but more study is needed. Current evidence suggests that bivalirudin is a well tolerated and effective alternative anticoagulant for pediatric ECMO patients who have failed UFH anticoagulation but prospective studies are needed to confirm these results. SUMMARY: Bivalirudin is a promising alternative anticoagulant for pediatric ECMO patients who have failed UFH. Large prospective, multicenter studies are needed to confirm safety and efficacy.

Prospective Exploratory Experience With Bivalirudin Anticoagulation in Pediatric Extracorporeal Membrane Oxygenation.

OBJECTIVES: Objective of this study was to determine if bivalirudin resulted in less circuit interventions than unfractionated heparin. A secondary objective was to examine associations between bivalirudin dose and partial thromboplastin time, international normalized ratio, and activated clotting time. DESIGN: Prospective observational. SETTING: Medical-surgical and cardiac PICUs. PATIENTS: Neonatal and pediatric extracorporeal membrane oxygenation patients who received bivalirudin anticoagulation. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Twenty extracorporeal membrane oxygenation runs in 18 patients used bivalirudin; 90% were venoarterial. Median (interquartile range) age was 4.5 months (1.6-35 mo). Thirteen patients (72%) had an underlying cardiac diagnosis. Of the 20 runs using bivalirudin, 16 (80%) were initially started on unfractionated heparin and transitioned to bivalirudin due to ongoing circuit thrombosis despite therapeutic anti-Xa levels (n = 13), ongoing circuit thrombosis with unfractionated heparin greater than or equal to 40 U/kg/hr (n = 2), or absence of increase in ACT after bolus of 100 U/kg of unfractionated heparin and escalation of unfractionated heparin infusion (n = 1). Initial bivalirudin dose ranged from 0.2 to 0.5 mg/kg/hr; no bolus doses were used. Median (range) bivalirudin dose was 0.9 mg/kg/hr (0.15-1.6 mg/kg/hr). Median (interquartile range) time on extracorporeal membrane oxygenation was 226.5 hours (150.5-393.0 hr) including 84 hours (47-335 hr) on bivalirudin. Nonparametric results are as follows: the rate of circuit intervention was significantly lower in patients on bivalirudin than on unfractionated heparin (median [interquartile range]: 0 [0-1] and 1 [1-2], respectively; Wilcoxon p = 0.0126). Bivalirudin dose was correlated to PTT (rs = 0.4760; p < 0.0001), INR (rs = 0.6833; p < 0.0001), and ACT (rs = 0.6161; p < 0.0001). Four patients had a significant bleeding complication on bivalirudin. Survival to hospital discharge was 56%. CONCLUSIONS: Bivalirudin appears to be a viable option for systemic anticoagulation in pediatric extracorporeal membrane oxygenation patients who have failed unfractionated heparin, but questions remain namely its optimal monitoring strategy. This pilot study supports the need for larger prospective studies of bivalirudin in pediatric extracorporeal membrane oxygenation, particularly focusing on meaningful monitoring variables.

Cardiac Surgery-Associated Kidney Injury in Children and Renal Oximetry.

OBJECTIVES: Cardiac surgery-associated acute kidney injury is common in children and associates with negative outcomes. Novel interventions to reduce cardiac surgery-associated acute kidney injury require knowledge of its pathophysiology. States of altered perfusion, oxygen delivery, and energy consumption occur during cardiopulmonary bypass and could protect against or contribute to renal cellular injury and recovery. Near-infrared spectroscopy is noninvasive technology for monitoring regional blood flow and tissue oxygenation. This study evaluated the relationship between renal regional oxygen saturation and cardiac surgery-associated acute kidney injury, using near-infrared spectroscopy monitoring before, during, and after cardiopulmonary bypass in children. DESIGN: Prospective cohort study. SETTING: Single-center, tertiary care pediatric hospital (Stollery Children's Hospital, Edmonton, AB, Canada). PATIENTS: Children less than or equal to 10 kg undergoing congenital heart disease repair with cardiopulmonary bypass. Heart transplant, preoperative dialysis, sepsis, extracorporeal life support, congenital renal disease, and preoperative nephrotoxins were exclusions. INTERVENTIONS: Renal regional near-infrared spectroscopy monitoring before, during, and after cardiopulmonary bypass. MEASUREMENTS AND MAIN RESULTS: Outcome measure was cardiac surgery-associated acute kidney injury (defined according to Kidney Disease: Improving Global Outcomes criteria). Regional oxygen saturation was measured continuously using near-infrared spectroscopy (INVOS 5100C Cerebral/Somatic Oximeter; Medronic, Troy, MI) from time of anesthesia to time of transfer to intensive care. Cardiac surgery-associated acute kidney injury occurred in 65%. Lower baseline (precardiopulmonary bypass) regional oxygen saturation was associated with decreased risk of cardiac surgery-associated acute kidney injury (p = 0.01); children with baseline regional oxygen saturation in the highest tertile were 7.14 times more likely to get cardiac surgery- associated acute kidney injury (vs lowest tertile). Area under the curve for ability of baseline regional oxygen saturation to predict cardiac surgery-associated acute kidney injury was 0.73 (95% CI, 0.60-0.85). Children with lower baseline glomerular filtration rate had lower mean renal regional oxygen saturation. CONCLUSIONS: Findings demonstrate that preoperative oxygen supply/demand balance is an important predictor of cardiac surgery-associated acute kidney injury, suggesting lower preoperative (and intraoperative) renal blood flow may be protective. There is not yet a definite link between remote ischemic preconditioning and prevention of cardiac surgery-associated acute kidney injury; however, renal protective effects of sublethal ischemia should continue to be explored.

Pediatric extracorporeal membrane oxygenation (ECMO): a guide for radiologists.

Extracorporeal membrane oxygenation (ECMO) is a life-saving treatment for pediatric patients with respiratory and/or cardiac failure. The ECMO circuit oxygenates and sometimes pumps the blood, effectively replacing lung and/or heart function temporarily. ECMO patients are clinically very complex not only because of their underlying, life-threatening pathology, but also because of the many physiological parameters that must be monitored and adjusted to maintain adequate tissue perfusion and oxygenation. Drainage and reinfusion cannulae connecting the patient to the ECMO circuit are visible on radiograph. These cannulae have different functions, different configurations, different radiographic appearances, and different positions that should be familiar to the interpreting pediatric radiologist. The primary complications of ECMO include hemorrhage, thrombosis and ischemia, as well as equipment failure and cannula malpositioning, all of which may be detected on imaging. In this pictorial essay, we discuss the basics of ECMO function and clinical management, ECMO cannula features and configurations, and the many complications of ECMO from an imaging perspective. Our goal is to educate pediatric radiologists about ECMO imaging, equipping them to properly interpret these studies and to become a useful consultant in ECMO patient care.

Prospective Side by Side Comparison of Outcomes and Complications With a Simple Versus Intensive Anticoagulation Monitoring Strategy in Pediatric Extracorporeal Life Support Patients.

OBJECTIVES: A continuous infusion of unfractionated heparin is the most common anticoagulant used for pediatric patients on extracorporeal life support. The objective of this study was to compare extracorporeal life support complications and outcomes between two large-volume pediatric extracorporeal life support centers that use different anticoagulation strategies. DESIGN: Prospective, observational cohort study. SETTING: The University of Michigan used simple anticoagulation monitoring, whereas the University of Alberta used an intensive anticoagulation monitoring strategy. PATIENTS: Pediatric patients on extracorporeal life support. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The primary outcome measure was major bleeding per extracorporeal life support run defined as bleeding that was retroperitoneal, pulmonary, or involved the CNS; bleeding greater than 20 mL/kg over 24 hours; or bleeding that required surgical intervention. Secondary outcomes measured were patient thrombosis per run, circuit thrombosis per run, and survival to hospital discharge per patient. Eighty-eight patients (95 runs) less than 18 years old were enrolled at the two centers over 2 years. The two centers enrolled different extracorporeal life support populations; University of Alberta enrolled more postcardiac surgical patients (74% vs 47%; p = 0.005). The indication for extracorporeal life support support also varied by center (p = 0.04). The two centers used similar proportions of VA extracorporeal life support (p = 0.3). Median (interquartile range) unfractionated heparin doses were similar between University of Michigan and University of Alberta, 30 (21-34) U/kg/hr and 26 (22-31) U/kg/hr, p value equals to 0.3, respectively. Median (interquartile range) antifactor Xa was lower in the University of Michigan cohort (0.23 [0.19-0.28] vs 0.41 [0.36-0.46] U/mL; p < 0.001). There was no significant difference in major bleeding (15% University of Michigan vs 21% University of Alberta; p = 0.6) or in patient thromboses (18% University of Michigan vs 13% University of Alberta; p = 0.5). There was no significant difference in survival to hospital discharge (University of Michigan 63% vs University of Alberta 73%; p = 0.1). CONCLUSIONS: Although this prospective cohort study compared different pediatric extracorporeal life support populations, the results did not identify a significant difference in outcomes between simple and intensive anticoagulation monitoring strategies.

Antithrombin concentrate in pediatric patients requiring unfractionated heparin anticoagulation: a retrospective cohort study.

OBJECTIVE: To describe antithrombin levels, altered unfractionated heparin effect (anti-factor Xa activity and activated partial thromboplastin time), and adverse effects post administration of a single high dose of antithrombin concentrate. DESIGN: Retrospective review. PATIENTS: Infants and children with antithrombin levels less than 50% and a subtherapeutic unfractionated heparin effect. SETTING: Quaternary care children's hospital with a dedicated anticoagulation program. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A single high dose of antithrombin concentrate was administered. Antithrombin level, anti-factor Xa, and activated partial thromboplastin times were measured post antithrombin concentrate infusion and daily until stable. One hundred twenty-one patients received 246 doses of antithrombin. Patients were described using two cohorts based on the ability to obtain exact heparin doses. Cohort 1 included all patients between January 2004 and May 2008 when complete heparin dosing was unavailable. Cohort 2 included patients from May 2008 to May 2011 when heparin dose was available. Median age and weight were 3.7 months and 4.1 kg. Mean antithrombin concentrate dose was 222 IU/kg. Mean antithrombin level increased from 0.39 to 1.20 U/mL following antithrombin concentrate administration. In cohort 2, unfractionated heparin doses to achieve a target anti-factor Xa activity pre-post antithrombin concentrate were 28 and 19 U/kg/hr, respectively, for children 12 months old or younger and 25 and 19 U/kg/hr, respectively, for children older than 12 months. There were no hemorrhagic, thrombotic, or allergic events within 1 week of antithrombin concentrate administration. CONCLUSIONS: This is the largest study of antithrombin concentrate evaluation in children. Administration of antithrombin concentrate increases anti-factor Xa activity with lower administered unfractionated heparin doses.

Acute kidney injury and renal recovery following Fontan surgery.

Objectives: Acute kidney injury has been described after Fontan surgery, but the duration and outcomes are unknown. We sought to describe the incidence of and risk factors for acute kidney injury and the phenotype of renal recovery, and evaluate the impact of renal recovery phenotype on outcomes. Methods: All children who underwent a Fontan operation at a single center between 2009 and 2022 were included. Data collected included Fontan characteristics, vasopressor use, all measures of creatinine, and postoperative outcomes. Logistic regression models were used to assess predictors of acute kidney injury and the association between acute kidney injury and outcomes. Results: We enrolled 141 children (45% female). Acute kidney injury occurred in 100 patients (71%). Acute kidney injury duration was transient (<48 hours) in 77 patients (55%), persistent (2-7 days) in 15 patients (11%), more than 7 days in 4 patients (3%), and unknown in 4 patients (3%). Risk factors for acute kidney injury included higher preoperative indexed pulmonary vascular resistance (odds ratio, 3.90; P = .004) and higher postoperative inotrope score on day 0 (odds ratio, 1.13, P = .047). Risk factors for acute kidney injury duration more than 48 hours included absence of a fenestration (odds ratio, 3.43, P = .03) and longer duration of cardiopulmonary bypass (odds ratio, 1.22 per 15-minute interval, P = .01). Acute kidney injury duration more than 48 hours was associated with longer length of stay compared with transient acute kidney injury (median 18 days [interquartile range, 9-62] vs 10 days [interquartile range, 8-16], P = .006) and more sternal wound infections (17% vs 4%, P = .049). Conclusions: Acute kidney injury after the Fontan operation is common. The occurrence and duration of acute kidney injury have significant implications for postoperative outcomes.

Predicting High-Risk Fetal Cardiac Disease Anticipated to Need Immediate Postnatal Stabilization and Intervention with Planned Pediatric Cardiac Operating Room Delivery.

BACKGROUND: Distances between delivery and cardiac services can make the care of fetuses with cardiac disease at risk of acute cardiorespiratory instability at birth a challenge. In 2013 we implemented a fetal echocardiography-based algorithm targeting fetuses considered high risk for acute cardiorespiratory instability at ≤2 hours of birth for delivery in our pediatric cardiac operating room of our children's hospital, and, herein, examine our experience. METHODS AND RESULTS: We reviewed maternal and postnatal medical records of all fetuses with cardiac disease encountered January 2013 to March 2022 considered high risk for acute cardiorespiratory instability. Secondary analysis was performed including all fetuses with diagnoses of d-transposition of the great arteries/intact ventricular septum (d-TGA/IVS) and hypoplastic left heart syndrome (HLHS) encountered over the study period. Forty fetuses were considered high risk for acute cardiorespiratory instability: 15 with d-TGA/IVS and 7 with HLHS with restrictive atrial septum, 4 with absent pulmonary valve syndrome, 3 with obstructed anomalous pulmonary veins, 2 with severe Ebstein anomaly, 2 with thoracic/intracardiac tumors, and 7 others. Pediatric cardiac operating room delivery occurred for 33 but not for 7 (5 with d-TGA/IVS, 2 with HLHS with restrictive atrial septum). For high-risk cases, fetal echocardiography had a positive predictive value of 50% for intervention/extracorporeal membrane oxygenation/death at ≤2 hours and 70% at ≤24 hours. Of "low-risk" cases, 6/46 with d-TGA/IVS and 0/45 with HLHS required intervention at ≤2 hours. Fetal echocardiography for predicting intervention/extracorporeal membrane oxygenation/death at ≤2 hours had a sensitivity of 67%, specificity 93%, and positive and negative predictive values of 80% and 87%, respectively, for d-TGA/IVS, and 100%, 95%, 71%, and 100% for HLHS, respectively. CONCLUSIONS: Fetal echocardiography can predict the need for urgent intervention in a majority with d-TGA/IVS and HLHS and in half of the entire spectrum of high-risk cardiac disease.

Rapid normalization of vitamin D deficiency in PICU (VITdALIZE-KIDS): study protocol for a phase III, multicenter randomized controlled trial.

BACKGROUND: The rate of vitamin D deficiency (VDD) in critically ill children worldwide has been estimated at 50%. These children are at risk of multiple organ dysfunction, chronic morbidity, and decreased health related quality of life (HRQL). Pediatric and adult ICU clinical trials suggest that VDD is associated with worse clinical outcomes, although data from supplementation trials are limited and inconclusive. Our group's phase II multicenter dose evaluation pilot study established the efficacy and safety of an enteral weight-based cholecalciferol loading dose to rapidly restore vitamin D levels in critically ill children. METHODS: Our aim is to evaluate the impact of this dosing regimen on clinical outcomes. VITdALIZE-KIDS is a pragmatic, phase III, multicenter, double-blind RCT aiming to randomize 766 critically ill children from Canadian PICUs. Participants are randomized using a 1:1 scheme to receive a single dose at enrollment of enteral cholecalciferol (10,000 IU/kg, max 400,000 IU) or placebo. Eligibility criteria include critically ill children aged newborn (> 37 weeks corrected gestational age) to < 18 years who have blood total 25-hydroxyvitamin D < 50 nmol/L. The primary objective is to determine if rapid normalization of vitamin D status improves HRQL at 28 days following enrollment. The secondary objective is to evaluate the impact of rapid normalization of vitamin D status on multiple organ dysfunction. The study includes additional tertiary outcomes including functional status, HRQL and mortality at hospital discharge and 90 days, PICU and hospital length of stay, and adverse events related to vitamin D toxicity. Additionally, we are performing comprehensive vitamin D speciation and non-targeted metabolite profiling as part of a sub-study for the first 100 participants from whom an enrollment and at least one post-intervention blood and urine sample were obtained. DISCUSSION: The VITdALIZE-KIDS trial is the first phase III, multicenter trial to evaluate whether rapid normalization of vitamin D status could represent a simple, inexpensive, and safe means of improving outcomes following pediatric critical illness. Recruitment was initiated in June 2019 and is expected to continue to March 2026. TRIAL REGISTRATION: Clinicaltrials.gov, NCT03742505. Study first submitted on November 12, 2018 https://clinicaltrials.gov/study/NCT03742505.

Pulmonary interstitial glycogenosis associated with pulmonary hypertension and hypertrophic cardiomyopathy.

A neonate with pulmonary interstitial glycogenosis, pulmonary hypertension, and hypertrophic cardiomyopathy is described. The fatal outcome for this patient contrasts with the reported favorable prognosis associated with isolated pulmonary interstitial glycogenosis. To the authors' knowledge, the association of pulmonary interstitial glycogenosis and hypertrophic cardiomyopathy has not been reported previously. The authors have broadened the phenotype of pulmonary interstitial glycogenosis and demonstrate the diagnostic value of lung biopsy in cases of unexplained neonatal pulmonary hypertension.

Heterotaxy syndrome: is a prophylactic Ladd procedure necessary in asymptomatic patients?

Heterotaxy syndrome (HS) is a complex disorder involving thoracic and abdominal asymmetries. Congenital heart disease is often accompanied by an intestinal rotation abnormality (IRA) that may predispose to bowel ischemia and infarction. There is controversy in the literature whether asymptomatic infants with HS must be screened for IRA and, if present, whether a prophylactic Ladd procedure should be performed. We performed a retrospective chart review of all patients who underwent a Ladd procedure from January 2007 to December 2010 at Stollery Children's Hospital, Edmonton, Canada. Twenty-nine patients underwent a Ladd procedure, 22 without HS but with symptomatic malrotation and 7 with HS and asymptomatic malrotation. Asymptomatic HS patients had a complication rate of 57 % after a prophylactic Ladd procedure compared with a complication rate of 9 % in the symptomatic non-HS population. The management of asymptomatic IRA in patients with HS remains controversial. We suggest that HS patients be screened for IRA and that asymptomatic patients be managed conservatively.

Standard Versus Restrictive Transfusion Strategy for Pediatric Cardiac ECLS Patients: Single Center Retrospective Cohort Study.

This retrospective cohort study aimed to compare blood component transfusion before and after the implementation of a restrictive transfusion strategy (RTS) in pediatric cardiac Extracorporeal Life Support (ECLS) patients. The study included children admitted to the pediatric cardiac intensive care unit (PCICU) at the Stollery Children's Hospital who received ECLS between 2012 and 2020. Children on ECLS between 2012 and 2016 were treated with standard transfusion strategy (STS), while those on ECLS between 2016 and 2020 were treated with RTS. During the study, 203 children received ECLS. Daily median (interquartile range [IQR]) packed red blood cell (PRBC) transfusion volume was significantly lower in the RTS group; 26.0 (14.4-41.5) vs. 41.5 (26.6-64.4) ml/kg/day, p value <0.001. The implementation of a RTS led to a median reduction of PRBC transfusion of 14.5 (95% CI: 6.70-21.0) ml/kg/day. Similarly, the RTS group received less platelets: median (IQR) 8.4 (4.50-15.0) vs. 17.5 (9.40-29.0) ml/kg/day, p value <0.001. The implementation of a RTS resulted in a median reduction of platelet transfusion of 9.2 (95% CI: 5.45-13.1) ml/kg/day. The RTS resulted in less median (IQR) fluid accumulation in the first 48 hours: 56.7 (2.30-121.0) vs. 140.4 (33.8-346.2) ml/kg, p value = 0.001. There were no significant differences in mechanical ventilation days, PCICU/hospital days, or survival. The use of RTS resulted in lower blood transfusion volumes, with similar clinical outcomes.

Infants less than or equal to 2.5 kg have increased mortality and worse motor neurodevelopmental outcomes at 2 years of age after Norwood-Sano palliation.

Objectives: In infants with single-ventricle congenital heart disease, prematurity and low weight at the time of the Norwood operation are risk factors for mortality. Reports assessing outcomes (including neurodevelopment) post Norwood palliation in infants ≤2.5 kg are limited. Methods: All infants who underwent a Norwood-Sano procedure between 2004 and 2019 were identified. Infants ≤2.5 kg at the time of the operation (cases) were matched 3:1 with infants >3.0 kg (comparisons) for year of surgery and cardiac diagnosis. Demographic and perioperative characteristics, survival, and functional and neurodevelopmental outcomes were compared. Results: Twenty-seven cases (mean ± standard deviation: weight 2.2 ± 0.3 kg and age 15.6 ± 14.1 days at surgery) and 81 comparisons (3.5 ± 0.4 kg and age 10.9 ± 7.9 days at surgery) were identified. Post-Norwood, cases had a longer time to lactate ≤2 mmol/L (33.1 ± 27.5 vs 17.9 ± 12.2 hours, P < .001), longer duration of ventilation (30.5 ± 24.5 vs 18.6 ± 17.5 days, P = .005), greater need for dialysis (48.1% vs 19.8%, P = .007), and greater need for extracorporeal membrane oxygenation support (29.6% vs 12.3%, P = .004). Cases had significantly greater postoperative (in-hospital) (25.9% vs 1.2%, P < .001) and 2-year (59.2% vs 11.1%, P < .001) mortality. Neurodevelopmental assessment showed the following for cases versus comparisons, respectively: cognitive delay (18.2% vs 7.9%, P = .272), language delay (18.2% vs 11.1%, P = .505), and motor delay (27.3% vs 14.3%, P = .013). Conclusions: Infants ≤2.5 kg at Norwood-Sano palliation have significantly increased postoperative morbidity and mortality up to 2-year follow-up. Neurodevelopmental motor outcomes were worse in these infants. Additional studies are warranted to assess the outcome of alternative medical and interventional treatment plans in this patient population.

2021 ELSO Adult and Pediatric Anticoagulation Guidelines.

DISCLAIMER: These guidelines for adult and pediatric anticoagulation for extracorporeal membrane oxygenation are intended for educational use to build the knowledge of physicians and other health professionals in assessing the conditions and managing the treatment of patients undergoing ECLS / ECMO and describe what are believed to be useful and safe practice for extracorporeal life support (ECLS, ECMO) but these are not necessarily consensus recommendations. The aim of clinical guidelines are to help clinicians to make informed decisions about their patients. However, adherence to a guideline does not guarantee a successful outcome. Ultimately, healthcare professionals must make their own treatment decisions about care on a case-by-case basis, after consultation with their patients, using their clinical judgment, knowledge and expertise. These guidelines do not take the place of physicians' and other health professionals' judgment in diagnosing and treatment of particular patients. These guidelines are not intended to and should not be interpreted as setting a standard of care or be deemed inclusive of all proper methods of care nor exclusive of other methods of care reasonably directed to obtaining the same results. The ultimate judgment must be made by the physician and other health professionals and the patient in light of all the circumstances presented by the individual patient, and the known variability and biological behavior of the clinical condition. These guidelines reflect the data at the time the guidelines were prepared; the results of subsequent studies or other information may cause revisions to the recommendations in these guidelines to be prudent to reflect new data, but ELSO is under no obligation to provide updates. In no event will ELSO be liable for any decision made or action taken in reliance upon the information provided through these guidelines.