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Background: A standard blood prime for cardiopulmonary bypass (CPB) in congenital cardiac surgery may possess non-physiologic values for electrolytes, glucose, and lactate. Pre-bypass Ultrafiltration (PBUF) can make these values more physiologic and standardized prior to bypass initiation. We aimed to determine if using PBUF on blood primes including packed red blood cells and thawed plasma would make prime values more predictable and physiologic. Additionally, we aimed to evaluate whether the addition of PBUF had an impact on outcome measures. Methods: Retrospective review of consecutive patients ≤ 1 year of age undergoing an index cardiac operation on CPB between 8/2017 and 9/2021. As PBUF was performed at the perfusionists’ discretion, a natural grouping of patients that received PBUF vs. those that did not occur. Differences in electrolytes, glucose, and lactate were compared at specific time points using Fisher’s exact test for categorical variables and the Wilcoxon rank sum test for continuous variables. Clinical outcomes were also assessed. Results: In both cohorts, the median age at surgery was 3 months and 47% of patients were female; 308/704 (44%) of the PBUF group and 163/414 (39%) of the standard prime group had at least one preoperative risk factor. The proportion of PBUF circuits which demonstrated more physiologic values for glucose (318 [45%]), sodium (434, [62%]), potassium (688 [98%]), lactate (612 [87%]) and osmolality (595 [92%]) was significantly higher when compared to standard prime circuit levels for glucose (8 [2%]), sodium (13 [3%], potassium (150 [36%]), lactate (56 [13%]) and osmolality (23 [6%]) prior to CPB initiation. There were no differences in clinical outcomes or rates of major adverse events between the two cohorts. Conclusions: PBUF creates standardized and more physiologic values for electrolytes, glucose, and lactate before the initiation of bypass without significant impacts on in-hospital outcomes.

The use of apixaban in the pediatric cardiac population is expanding. We describe our apixaban dosing and monitoring strategy in children and young adults awaiting heart transplantation and outcomes related to bleeding and thrombosis during wait-list and early post-transplant periods. Retrospective, single center analysis of all patients on apixaban awaiting cardiac transplantation. Weight based dosing was monitored with peak drug-specific anti-Xa chromogenic analysis (apixaban levels). Goal apixaban peak levels were extrapolated from adult studies and center experience (prophylaxis 80-150 ng/mL, VAD or treatment 100-300 ng/ml). Between 2/1/2020 and 10/10/2022, 17 patients, median age 11.1 years (0.8 to 24), weighing 45.4 kg (6.76-101.5) received apixaban (median dose 5 mg po BID (0.625-7.5)). Indication was prophylaxis (n=16) and treatment of thrombus (n=1). Four patients were on aspirin co-therapy. There were no clinically relevant non-major, or major bleeding or thrombotic events while awaiting transplant. Median time from last apixaban dose to arrival in OR was 23.2 hours (15.6-33.8), with median random apixaban level of 45.6 ng/ml (<23-81.5), 6.3 hours (0-19.3) prior to arrival in OR. There were no reports of increase in perioperative bleeding. One patient received recombinant Factor Xa (ANDEXXA) with protamine reversal, despite hemostasis and pre-operative apixaban level of 29 ng/ml. This patient developed acute graft failure requiring ECMO in the first 12 hours. Angiogram POD 4 showed multiple pulmonary emboli, with subsequent death. One Fontan patient with complex venous reconstruction developed intra-operative upper venous thrombosis, requiring catheterization for recanalization immediately post-transplant. This event was not felt attributable to apixaban. One patient managed on HM3 LVAD with Apixaban and ASA prophophylaxis developed a global neurologic injury in the peri-transplant period unrelated to bleeding or thrombosis and likely unrelated to apixaban. Careful use of apixaban can be safe and effective while awaiting heart transplant. Anti-Xa monitoring can assess drug clearance preoperatively. There was no appreciable increase in perioperative bleeding. We advise against empiric use of recombinant FXa in the absence of life threatening bleeding, as potential trigger of thrombosis. [ABSTRACT FROM AUTHOR]

Background: Pulmonary hypertension in children often progresses despite optimal therapy. This document provides an evidence-based clinical practice guideline for the management of children with progressive pulmonary hypertension despite optimal therapy., Methods: A multidisciplinary panel identified pertinent questions regarding the management of children with pulmonary hypertension that has progressed despite optimal therapy, conducted systematic reviews of the relevant literature, and applied the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) approach to develop clinical recommendations., Results: After reviewing the research evidence, the panel considered the balance of desirable (benefits) and undesirable (harms and burdens) effects of the interventions in each proposed question. Valuation of our main outcomes was also considered, along with resources required, equity, acceptability, and feasibility. Recommendations were developed for or against interventional strategies specific to children with pulmonary hypertension that has progressed despite optimal therapy., Conclusions: Although there is a growing population of children with pulmonary hypertension, there is a striking lack of empirical evidence regarding management of those whose disease has progressed despite optimal pharmacotherapy. The panel formulated and provided the rationale for clinical recommendations for or against interventional strategies based on this limited empirical evidence, coupled with expert opinion, to aid clinicians in the management of these complex pediatric patients. In addition, we identified important areas for future research.

Background: Few studies describe outcomes after complete vascular ring surgery in a comprehensive manner., Objectives: This study sought to describe the clinical presentation, diagnostic work-up, operative approach, and outcomes in children undergoing surgery for complete vascular rings., Methods: This single-center retrospective cohort study includes consecutive patients (January 1990 through September 2023) undergoing primary surgery for complete vascular rings, or rerepair after primary surgery elsewhere. The primary outcome of interest was complete (as distinct from partial) symptom resolution at latest clinic follow-up. Our current preference is to pursue a comprehensive initial operation including adjunctive vascular and airway procedures targeting common causes of residual aerodigestive symptoms, such as Kommerell diverticulum resection and tracheobronchopexy. Preoperative work-up routinely involved computed tomographic angiography, dynamic bronchoscopy, and laryngoscopy., Results: Of 515 patients (including 39 rerepairs), the most common diagnoses were right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum (n = 323, 62.7%) and double aortic arch (n = 174, 33.8%). There was no perioperative mortality. Chylothorax occurred in 28 patients (5.4%), vocal cord dysfunction in 22 patients (4.3%), and diaphragm paralysis in 2 patients (0.4%). Follow-up was available on 453 patients (88.0%) with a median duration of 3.0 years (Q1-Q3: 0.6-9.2 years). At latest clinic follow-up, 429 patients (94.7%) reported complete symptom resolution. The risk of reoperation for residual or recurrent aerodigestive symptoms was 9.6% (95% CI: 5.7%-13.5%) at 10 years and 12.4% at 20 years (95% CI: 6.9%-17.8%)., Conclusions: Surgery for complete vascular rings provides good symptomatic relief with low risk of complications, whereas reoperations for aerodigestive symptoms are infrequent., Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024. Published by Elsevier Inc.)

There remains high morbidity and mortality with mechanical circulatory support (MCS) in failing bidirectional Glenn (BDG) physiology. We performed a retrospective analysis of children with BDG physiology supported with MCS before and after 2018. Fourteen patients met inclusion criteria (median age 1.5 years, weight 9 kg). Prior to 2018 (n = 7), with variable anticoagulation and strategies including pulsatile VAD, continuous flow VAD, and extracorporeal membrane oxygenation (ECMO), 3 (43%) of patients were transplanted with a total of 536 patient-days of support (median 59 days). Major hemocompatability-related adverse event (MHRAE) rate was 63 per 100 patient-months. After 2018 (n = 7), using a staged support strategy (ECMO to pulsatile VAD) and bivalirudin anticoagulation, 5 (71%) patients were transplanted with a total of 1260 patient-days of support (median 188 days) and MHRAE rate of 24 per 100 patient-months. Despite challenging physiology, we have observed improved survival and reduced MHRAE despite longer support duration., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: There is no FDA-approved left ventricular assist device (LVAD) for smaller children permitting routine hospital discharge. Smaller children supported with LVADs typically remain hospitalized for months awaiting heart transplant-a major burden for families and a challenge for hospitals. We describe the initial outcomes of the Jarvik 2015, a miniaturized implantable continuous flow LVAD, in the NHLBI-funded Pumps for Kids, Infants, and Neonates (PumpKIN) study, for bridge-to-heart transplant., Methods: Children weighing 8 to 30 kg with severe systolic heart failure and failing optimal medical therapy were recruited at 7 centers in the United States. Patients with severe right heart failure and single-ventricle congenital heart disease were excluded. The primary feasibility endpoint was survival to 30 days without severe stroke or non-operational device failure., Results: Of 7 children implanted, the median age was 2.2 (range 0.7, 7.1) years, median weight 10 (8.2 to 20.7) kilograms; 86% had dilated cardiomyopathy; 29% were INTERMACS profile 1. The median duration of Jarvik 2015 support was 149 (range 5 to 188) days where all 7 children survived including 5 to heart transplant, 1 to recovery, and 1 to conversion to a paracorporeal device. One patient experienced an ischemic stroke on day 53 of device support in the setting of myocardial recovery. One patient required ECMO support for intractable ventricular arrhythmias and was eventually transplanted from paracorporeal biventricular VAD support. The median pump speed was 1600 RPM with power ranging from 1-4 Watts. The median plasma free hemoglobin was 19, 30, 19 and 30 mg/dL at 7, 30, 90 and 180 days or time of explant, respectively. All patients reached the primary feasibility endpoint. Patient-reported outcomes with the device were favorable with respect to participation in a full range of activities. Due to financial issues with the manufacturer, the study was suspended after consent of the eighth patient., Conclusion: The Jarvik 2015 LVAD appears to hold important promise as an implantable continuous flow device for smaller children that may support hospital discharge. The FDA has approved the device to proceed to a 22-subject pivotal trial. Whether this device will survive to commercialization remains unclear because of the financial challenges faced by industry seeking to develop pediatric medical devices. (Supported by NIH/NHLBI HHS Contract N268201200001I, clinicaltrials.gov 02954497)., (Copyright © 2024 International Society for the Heart and Lung Transplantation. All rights reserved.)

Background: As more pediatric patients become candidates for heart transplantation (HT), understanding pathological predictors of outcome and the accuracy of the pretransplantation evaluation are important to optimize utilization of scarce donor organs and improve outcomes. The authors aimed to investigate explanted heart specimens to identify pathologic predictors that may affect cardiac allograft survival after HT., Methods: Explanted pediatric hearts obtained over an 11-year period were analyzed to understand the patient demographics, indications for transplant, and the clinical-pathological factors., Results: In this study, 149 explanted hearts, 46% congenital heart defects (CHD), were studied. CHD patients were younger and mean pulmonary artery pressure and resistance were significantly lower than in cardiomyopathy patients. Twenty-one died or underwent retransplantation (14.1%). Survival was significantly higher in the cardiomyopathy group at all follow-up intervals. There were more deaths and the 1-, 5- and 7-year survival was lower in patients ≤10 years of age at HT. Early rejection was significantly higher in CHD patients exposed to homograft tissue, but not late rejection. Mortality/retransplantation rate was significantly higher and allograft survival lower in CHD hearts with excessive fibrosis of one or both ventricles. Anatomic diagnosis at pathologic examination differed from the clinical diagnosis in eight cases., Conclusions: Survival was better for the cardiomyopathy group and patients >10 years at HT. Prior homograft use was associated with a higher prevalence of early rejection. Ventricular fibrosis (of explant) was a strong predictor of outcome in the CHD group. We presented several pathologic findings in explanted pediatric hearts., (© 2024 The Authors. Pediatric Transplantation published by Wiley Periodicals LLC.)

Despite extracorporeal membrane oxygenation (ECMO) utilization in nearly 20% of cases, there are limited data in children with acute fulminant myocarditis (AFM) requiring ECMO. Herein we identify risk factors for death or heart transplant (HT) in children with AFM supported with ECMO, describe our experience with left atrial (LA) decompression, and depict long-term outcomes of survivors. We performed a retrospective cohort of patients <18 years with AFM (≤14 days of symptoms, rapid cardiogenic shock, and normal left ventricular [LV] size on presentation) supported with ECMO admitted to a single intensive care unit from 1997 to 2021. Among 28 patients (median age 9 years), 21 (75%) survived to discharge without HT. Patients were supported on ECMO for a median of 6 days. Three patients were bridged to HT with durable ventricular assist devices (VAD). Four patients died, two of whom were supported with VAD. At presentation, seven (25%) patients had high grade or complete atrioventricular block and eight (29%) had ventricular tachycardia. Before ECMO cannulation, 21 (75%) patients received CPR. The death/HT group had higher peak troponin levels (12.5 vs . 1.0 ng/ml, p = 0.02) and initial mean LA or pulmonary capillary wedge pressure (27 vs . 18 mm Hg, p = 0.03). Left atrial decompression was performed in 22 patients (79%). Twenty-two (79%) had acute myocarditis on endomyocardial biopsy. Among transplant-free survivors, 18 (86%) had normalization in LV function (median 7 days); the remaining three patients had persistent mild LV dysfunction at last follow-up (median 842 days). Transplant-free survival of pediatric patients with AFM supported on ECMO was 75% and associated with lower initial LA pressure and lower peak troponin. Recovery in ventricular function among survivors was rapid and durable., Competing Interests: Disclosure: The authors have no conflicts of interest to report., (Copyright © ASAIO 2023.)

Objectives: Extracorporeal membrane oxygenation to support cardiopulmonary resuscitation (CPR) is increasingly used in children suffering cardiac arrest after cardiac surgery. However, its efficacy in promoting survival has not been evaluated. We compared survival of pediatric cardiac surgery patients suffering in-hospital cardiac arrest who were resuscitated with extracorporeal CPR (E-CPR) to those resuscitated with conventional CPR (C-CPR) using propensity matching., Design: Retrospective study using multicenter data from the American Heart Association Get With The Guidelines-Resuscitation registry (2008-2020)., Setting: Multicenter cardiac arrest database containing cardiac arrest and CPR data from U.S. hospitals., Patients: Cardiac surgical patients younger than 18 years old who suffered in-hospital cardiac arrest and received greater than or equal to 10 minutes of CPR., Interventions: None., Measurements and Main Results: Among 1223 patients, 741 (60.6%) received C-CPR and 482 (39.4%) received E-CPR. E-CPR utilization increased over the study period ( p < 0.001). Duration of CPR was longer in E-CPR compared with C-CPR recipients (42 vs. 26 min; p < 0.001). In a propensity score matched cohort (382 E-CPR recipients, 382 C-CPR recipients), E-CPR recipients had survival to discharge (odds ratio [OR], 2.22; 95% CI, 1.7-2.9; p < 0.001). E-CPR survival was only higher when CPR duration was greater than 18 minutes. Propensity matched analysis using patients from institutions contributing at least one E-CPR case ( n = 35 centers; 353 E-CPR recipients, 353 C-CPR recipients) similarly demonstrated improved survival in E-CPR recipients compared with those who received C-CPR alone (OR, 2.08; 95% CI, 1.6-2.8; p < 0.001)., Conclusions: E-CPR compared with C-CPR improved survival in children suffering cardiac arrest after cardiac surgery requiring CPR greater than or equal to 10 minutes., Competing Interests: Dr. Alexander has received funding from National Institutes of Health (NIH)/National Institute of Child Health and Human Development (NICHD) (R13HD104432), Food and Drug Administration (FP01029501), U.S. Department of Defense (W81XWH2210301), and unrestricted grants from the Extracorporeal Life Support Organization (ELSO); she received funding from Novartis (consultant on the end-point adjudication committee for Prospective Trial to Assess the Angiotensin Receptor Blocker Neprilysin Inhibitor LCZ696 Versus Angiotensin-Converting Enzyme Inhibitor for the Medical Treatment of Pediatric HF (PANORAMA-HF) clinical trial); and she serves as the ELSO Treasurer of the Board of Directors. Dr. Bembea has received grants from NIH/National Institute of Neurological Disorders and Stroke (R01NS106292), NIH/NICHD, ELSO, and Grifols Investigator Sponsored Research Grant. Dr. Thiagarajan has received grants from the U.S. Department of Defense Clinical Trial Award (W81XWH2210301), ELSO, and Society of Critical Care Medicine; he is supported, in part, by the Callaghan Family Chair in Cardiac Critical Care. The remaining authors have disclosed that they do not have any potential conflicts of interest., (Copyright © 2024 by the Society of Critical Care Medicine and Wolters Kluwer Health, Inc. All Rights Reserved.)

Background: The use of apixaban in the pediatric cardiac population is expanding. We describe our apixaban dosing and monitoring strategy in children and young adults awaiting heart transplantation, along with outcomes related to bleeding and thrombosis during wait-list and early post-transplant periods., Methods: This study is a retrospective, single-center analysis of all patients receiving apixaban while awaiting cardiac transplantation. Weight-based dosing was monitored with peak drug-specific anti-Xa chromogenic analysis. Significant post-operative bleeding defined by chest tube output or need for surgical intervention., Results: From September 2020 to December 2022, 19 patients, median age 13.5 years (6.1, 15.8 years), weighing 48.9 kg (15.4, 67.6) received apixaban while awaiting transplant. Indication for apixaban was prophylaxis (n = 18, 3 with ventricular assist devices) and treatment of thrombus (n = 1). There were no clinically relevant non-major or major bleeding, nor thrombotic events while awaiting transplant. The median time from last apixaban dose to arrival in the operating room was 23.2 h (15.6-33.8), with median random apixaban level of 37 ng/mL (28.3, 59), 6.3 h (4.8, 8.4) prior to arrival in the operating room. In this study, 32% of patients had significant post-operative bleeding based on chest tube output post-transplant or need for intervention. No patients meeting criteria for significant post-operative bleeding were thought to be attributable to apixaban., Conclusions: Careful use of apixaban can be safe and effective while awaiting heart transplant. There was no appreciable increase in peri-operative bleeding. The use of apixaban is promising in providing safe, predictable and efficacious anticoagulation while avoiding additional patient stressors., (© 2023 Wiley Periodicals LLC.)

Background: Short-term outcomes using steroid avoidance immune suppression are encouraging in pediatric heart transplant (HT) recipients at low risk of antibody-mediated rejection. We assessed medium-term outcomes in pediatric HT recipients initiated on a steroid avoidance protocol at our institution using surveillance biopsies., Methods: All primary HT recipients during 2006-2020 who did not have a donor-specific antibody were eligible for immune suppression consisting of 5-d Thymoglobulin/steroid induction followed by a tacrolimus-based, steroid-free regimen. We assessed freedom from graft failure (death or retransplant), acute rejection, posttransplant lymphoproliferative disease, and cardiac allograft vasculopathy., Results: Overall, 150 of 181 primary HT recipients were eligible for steroid avoidance regimen. Their median age was 8.7 y, 41% had congenital heart disease, 23% were sensitized, and 35% were on a mechanical support. The median follow-up was 6.1 y. Eleven patients (8%) were on maintenance steroids at discharge and 13% at 1 y. Graft survival was 94% at 1 y and 87% at 5 y. Freedom from rejection was 73% at 1 y and 64% at 5 y. Freedom from posttransplant lymphoproliferative disease was 96% at 1 y and 95% at 5 y. Freedom from moderate cardiac allograft vasculopathy was 94% at 5 y. Eight patients developed diabetes. Estimated glomerular filtration rate was <60 mL/min/1.73 m 2 in 5% of the cohort at 5 y., Conclusions: Pediatric HT recipients at low risk of antibody-mediated rejection have excellent medium-term survival and relatively low incidence of posttransplant morbidities when managed using a steroid avoidance immune suppression protocol., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)