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Despite recent data suggesting improved outcomes with bivalirudin vs heparin in pediatric Ventricular assist devices (VAD), higher costs remain a barrier. This study quantified trends in bivalirudin use and compared outcomes, resource utilization, and cost-effectiveness associated with bivalirudin vs heparin.Children age 0 to 6 year who received VAD from 2009 to 2021 were identified in Pediatric Health Information System. Bivalirudin use was evaluated using trend analysis and outcomes were compared using Fine-Gray subdistrubtion hazard ratios (SHR). Daily-level hospital costs were compared due to differences in length of stay. Cost-effectiveness was evaluated using incremental cost-effectiveness ratio (ICER).Of 691 pediatric VAD recipients (median age 1 year, IQR 0-2), 304 (44%) received bivalirudin with 90% receiving bivalirudin in 2021 (trend p-value0.01). Bivalirudin had lower hospital mortality (26% vs 32%; adjusted SHR 0.57, 95% CI 0.40-0.83) driven by lower VAD mortality (20% vs 27%; adjusted SHR 0.46, 95% CI 0.32-0.77) after adjusting for year, age, diagnosis, and center VAD volume. Post-VAD length of stay was longer for bivalirudin than heparin (median 91 vs 64 days, respectively, p0.001). Median daily-level costs were lower among bivalirudin (cost ratio 0.87, 95% CI 0.79-0.96) with higher pharmacy costs offset by lower imaging, laboratory, supply, and room/board costs. Estimated ICER for bivalirudin vs heparin was $61,192 per quality-adjusted life year gained with a range of $27,673 to $131,243.Bivalirudin use significantly increased over the past decade and is now used in 90% young pediatric VAD recipients. Bivalirudin was associated with significantly lower hospital mortality and an ICER$65,000, making it a cost-effective therapy for pediatric VAD recipients.

Severe forms of pulmonary embolism (PE) in children are rare but cause significant morbidity and mortality. In this article, we review the pathophysiology of severe (high- and intermediate-risk) PE and suggest novel pediatric-specific risk stratifications and an acute treatment algorithm to expedite emergent decision making. We define pediatric high-risk PE as causing cardiopulmonary arrest, sustained hypotension or normotension with signs or symptoms of shock. Rapid primary reperfusion should be pursued with either surgical embolectomy or systemic thrombolysis in conjunction with a heparin infusion and supportive care as appropriate. We define pediatric intermediate-risk PE as those without systemic hypotension nor compensated shock but with evidence of right ventricular strain by imaging and/or myocardial necrosis by elevated cardiac troponin levels. The decision to pursue primary reperfusion in this group is complex and should be reserved for patients with more severe disease; anticoagulation alone may also be appropriate in some cases. If primary reperfusion is pursued, catheter-based therapies may be beneficial. In summary, acute management of severe PE in children may include systemic thrombolysis, surgical embolectomy, catheter-based therapies or anticoagulation alone, and may depend on patient and institutional factors. Pediatric emergency and intensive care physicians should be familiar with the risks and benefits of each therapy in order to expedite care. PE Response Teams may also have added benefit in streamlining care during these critical events.

Ventricular assist devices (VADs) increase waitlist survival, yet the risk of stroke remains notable. The purpose of this study was to analyze how strokes on VAD support impact post-transplant (post-Tx) outcomes in children.About 520 pediatric (18 years) heart transplant candidates listed from January 2011 to April 2018 with a VAD implant date were matched between the United Network of Organ Sharing and Pediatric Health Information System databases. Patients were divided into pre-Tx Stroke and No Stroke cohorts.About 81% of the 520 patients were transplanted; 28% (n = 146) had a pre-Tx Stroke; and 59% (n = 89) of the Stroke patients were transplanted at a median of 57 (IQR 17-102) days from stroke. Significantly more No Stroke cohort (90%) were transplanted (p 0.001). There was no difference in post-Tx survival between the Stroke and No Stroke cohorts (p = 0.440). Time between stroke and transplant for patients who died within 1 year of transplant was 32.0 days (median) compared to 60.5 days for those alive1 year (p = 0.18). Regarding patients in whom time from stroke to transplant was more than 60 days, one-year survival of Stroke vs. No Stroke patients was 96% vs. 95% (p = 0.811), respectively.Patients with stroke during VAD support, once transplanted, enjoy similar survival compared to No Stroke patients. We hypothesize that allowing Stroke patients more time to recover could improve post-Tx outcomes. Unfortunately, the ideal duration of time between stroke and safe transplantation could not be determined and will require more detailed and larger studies in the future.

Purpose: We sought to establish whether nucleated red blood cells (NRBCs) are predictive of disposition, morbidity, and mortality for pediatric patients presenting to the emergency department (ED).Methods: A single center retrospective cohort study examining all ED encounters from patientsResults: The prevalence of NRBCs was 8.9% (4,195/46,991 patient encounters). Patient with NRBCs were younger (median age 4.58 vs 8.23 years; PConclusions: Presence of NRBCs is an independent predictor for mortality, including in-hospital mortality, ICU admission, CPR, and readmission within 30 days for children presenting to the ED.

Objectives: Six months after withdrawal of the HeartWare HVAD System (HVAD; Medtronic) from sale, approximately 4000 patients continue ongoing support with this device. In light of the diminishing experience, this global consensus document summarizes key management recommendations. Methods: International experts with experience in the management of patients with ongoing HVAD support were invited to summarize key aspects of patient and pump management and highlight differences in the current HeartMate 3 (Abbott Laboratories) ventricular assist device. Clinicians from high-implanting HVAD sites reviewed current literature and reported experience to generate a consensus statement. Results: Specific guidelines to assist in the management of ongoing HVAD patients are developed. Key management protocols and helpful techniques developed from experienced clinicians are combined into a short guideline document. As experience with HeartMate 3 increases, key differences in approach to management are highlighted, where appropriate. Conclusions: With decreasing worldwide experience in the ongoing management of HVAD-supported patients, this consensus guideline provides a summary of best practice techniques from international centers. Differences in HeartMate 3 management are highlighted.

Background: Cardiac manifestations in multisystem inflammatory syndrome in children (MIS-C) occur in ∼80% of patients. Left ventricular (LV) systolic dysfunction is the most frequent cardiac finding. Methods: In this single-centre, retrospective cohort study, we report on detailed assessment of LV function in MIS-C patients using strain and strain rate analysis. We compare those with normal peak systolic strain z-scores (both longitudinal and circumferential strain) to those with abnormal peak systolic strain z-scores (decreased circumferential and/or longitudinal strain). Results: Among 25 patients, 14 (56%) were male, 20 (80%) were Black or Hispanic, 13 (52%) were overweight/obese, and the median age was 11.4 years (interquartile range: 7.5 to 16). Median ejection fraction (EF) was 55.2% (interquartile range: 48.3% to 58%), with the abnormal strain patients having a lower EF (P < 0.01). Demographics were similar between groups. The abnormal strain patients had more organ systems involved and were more likely to require inotropic support. In a comparison of MIS-C patients with normal EF (n = 15) to controls, MIS-C patients had lower peak systolic strain as well as lower early diastolic strain rates. In patients with initially depressed function, EF normalized in 8 of 10 (80%), but 4 of 11 (36%) patients had persistently abnormal systolic strain after discharge. Conclusions: LV systolic dysfunction is common in the acute phase of MIS-C, and detection may be improved with strain imaging. Longitudinal cardiac follow-up is imperative, as some patients may be at risk for persistent LV dysfunction. Résumé: Contexte: Des manifestations cardiaques sont observées chez environ 80 % des patients atteints du syndrome inflammatoire multisystémique de l'enfant (SIM-E). La dysfonction systolique ventriculaire gauche est le problème cardiaque observé le plus fréquemment. Méthodologie: Dans cette étude de cohorte rétrospective et unicentrique, nous rapportons les résultats d'une évaluation détaillée de la fonction ventriculaire gauche chez des patients atteints du SIM-E sous l'angle de l'étude des contraintes et des taux de contrainte. Nous comparons les patients dont les écarts z des pics de contrainte systolique sont normaux (contraintes tant longitudinales que circonférentielles) et ceux dont les écarts z des pics de contrainte systolique sont anormaux (réduction de la contrainte circonférentielle ou longitudinale). Résultats: Sur 25 patients, 14 (56 %) étaient de sexe masculin, 20 (80 %) étaient noirs ou hispaniques, 13 (52 %) étaient en surpoids ou obèses, et l'âge médian était de 11,4 ans (intervalle interquartile : de 7,5 à 16). La fraction d'éjection (FE) médiane était de 55,2 % (intervalle interquartile : de 48,3 % à 58 %), et était moins élevée chez les patients présentant une contrainte anormale (p < 0,01). Les caractéristiques démographiques étaient comparables dans tous les groupes. Les patients chez lesquels la contrainte était anormale présentaient un plus grand nombre d'organes atteints et étaient plus susceptibles de nécessiter un soutien inotrope. Comparativement au groupe témoin, les patients SIM-E ayant une FE normale (n = 15) présentaient un pic de contrainte systolique moins élevé et des taux de contrainte diastolique précoce plus faibles. Chez les patients dont la fonction était déprimée à l'origine, la FE s'est normalisée chez huit patients sur 10 (80 %), mais quatre sur 11 (36 %) présentaient une contrainte systolique persistant après leur sortie de l'hôpital. Conclusions: La dysfonction systolique ventriculaire gauche est fréquente dans la phase aiguë du SIM-E, et son repérage pourrait être amélioré par l'imagerie permettant de visualiser les contraintes. Un suivi cardiaque longitudinal est impératif, car certains patients peuvent être à risque de souffrir d'une dysfonction ventriculaire gauche persistante.

BACKGROUND The size of the Fontan population with end-stage heart failure is growing. In this population, heart transplantation has been the only option. This study sought to investigate the efficacy of ventricular assist device (VAD) support in Fontan patients. METHODS We conducted a retrospective study of Fontan patients in the Advanced Cardiac Therapies Improving Outcomes Network. We evaluated patient characteristics, and the clinical and physiologic outcomes after VAD implantation. RESULTS We identified 45 Fontan patients implanted with VAD. The average age of patients was 10 years (interquartile range: 4.5–18) and 30% were female. The majority had a morphologic right ventricle (69%), moderate or greater ventricular dysfunction (83%), and moderate or greater atrioventricular valve regurgitation (65%). The majority of implants were as a bridge to transplantation (76%), and the majority of patients were Interagency Registry for Mechanically Assisted Circulatory Support Profile 2 (56%). The most commonly employed device was the Medtronic HeartWare HVAD (56%). A total of 13 patients were discharged on device support, and 67% of patients experienced adverse events, the most common of which were neurologic (25%). At 1 year after device implantation, the rate of transplantation was 69.5%, 9.2% of patients continued to be VAD supported, and 21.3% of patients had died. Hemodynamically, VAD was effective in decreasing both Fontan and ventricular end-diastolic pressures in some individuals. CONCLUSIONS VAD is effective in supporting patients with end-stage Fontan failure awaiting heart transplantation. Future research should focus on identifying clinical and physiologic characteristics predictive of a favorable response to VAD support.

BACKGROUND The Berlin Heart EXCOR Pediatric (EXCOR) ventricular assist device (VAD) was introduced in North America nearly 2 decades ago. The EXCOR was approved under Humanitarian Device Exemption status in 2011 and received post-market approval (PMA) in 2017 from Food and Drug Administration. Since the initial approval, the field of pediatric mechanical circulatory support has changed, specifically with regard to available devices, anticoagulation strategies, and the types of patients supported. This report summarizes the outcomes of patients supported with EXCOR from the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry. These data were part of the PMA surveillance study (PSS) required by the Food and Drug Administration. METHODS ACTION is a learning collaborative of over 40 pediatric heart failure programs worldwide, which collects data for all VAD implantations as one of its initiatives. All patients in North America with EXCOR implants reported to ACTION from 2018 to 2020 ( n = 72) who had met an outcome were included in the EXCOR PSS group. This was compared with a historical, previously reported Berlin Heart EXCOR study group (Berlin Heart study [BHS] group, n = 320, 2007‒2014). RESULTS Patients in the PSS group were younger, were smaller in weight/body surface area, were more likely to have congenital heart disease , and were less likely to receive a bi-VAD than those in the BHS group. Patients in the PSS group were less likely to be in Interagency Registry for Mechanically Assisted Circulatory Support Profile 1 and were supported for a longer duration. The primary anticoagulation therapy for 92% of patients in the PSS group was bivalirudin . Success, defined as being transplanted, being weaned for recovery, or being alive on a device at 180 days after implantation, was 86% in the PSS group compared with 76% in the BHS group. Incidence of stroke was reduced by 44% and the frequency of pump exchange by 40% in the PSS group compared with those in the BHS group. Similarly, all other adverse events , including major bleeding, were reduced in the PSS group. CONCLUSIONS The PSS data, collected through ACTION, highlight the improvement in outcomes for patients supported with EXCOR compared with the outcomes in a historical cohort. These findings may be the result of changes in patient care practices over time and collaborative learning.

Pediatric heart failure is a complex, heterogenous syndrome that occurs relatively rarely in children, but carries a high burden of morbidity and mortality. This article reflects on the current state of medical therapy for both acute and chronic pediatric heart failure, based on expert consensus guidelines, and the extrapolation of data from trials performed in adults. For the management of acute heart failure specifically, we rely on an initial assessment of the perfusion and volume status of a patient, to guide medical therapy. This paradigm was adapted from adult studies that demonstrated increased morbidity and mortality in heart failure patients whose hemodynamics or examination findings were consistent with a PCWP >18 mmHg and a CI ≤2.2 L/min/m(2). The cornerstone of treatment in the acute setting therefore relies on achieving a euvolemic state with adequate cardiac output. In the chronic setting, patients are typically maintained on a regimen of an angiotensin converting enzyme inhibitor, a beta-blocker, and spironolactone. For those with refractory heart failure, intravenous milrinone therapy has become a mainstay of bridging children to cardiac transplantation. The pediatric-specific data driving these clinical practices are limited and often times, conflicting. The future of pediatric heart failure depends on collaboration, quality improvement, and a commitment to pediatric-specific indications for new medical and device therapies.

In this manuscript, we review the diagnosis, causes, and management of cardiac thrombosis in children with congenital and acquired heart disease. Indications for invasive management and surgery are discussed. The differential diagnostic considerations and management of cardiac thromboses will be reviewed, including those that occur as a consequence of repair and palliation of congenital heart disease. The definition of venous thromboembolism has recently been expanded to include thromboses within both the right and left heart chambers. Direct thrombin inhibitors (DTIs) and direct oral anticoagulants (DOACs) are emerging as treatment alternatives to the heparins and vitamin K antagonists for the management of thrombosis in pediatric heart disease. Thrombosis within the heart occurs as a consequence of abnormalities and alterations in blood flow resulting in stasis and turbulence. Children with acquired and congenital heart disease carry a number of risk factors in Virchow’s triad. Thrombosis can occur throughout any part of the cardiovascular system in congenital heart disease and after surgery for repair or palliation of defects.

Inborn errors of metabolism (IEM) involving the non-oxidative pentose phosphate pathway (PPP) include the two relatively rare conditions, transketolase deficiency and transaldolase deficiency, both of which can be difficult to diagnosis given their non-specific clinical presentations. Current biochemical testing approaches require an index of suspicion to consider targeted urine polyol testing. To determine whether a broad-spectrum biochemical test could accurately identify a specific metabolic pattern defining IEMs of the non-oxidative PPP, we employed the use of clinical metabolomic profiling as an unbiased novel approach to diagnosis. Subjects with molecularly confirmed IEMs of the PPP were included in this study. Targeted quantitative analysis of polyols in urine and plasma samples was accomplished with chromatography and mass spectrometry. Semi-quantitative unbiased metabolomic analysis of urine and plasma samples was achieved by assessing small molecules via liquid chromatography and high-resolution mass spectrometry. Results from untargeted and targeted analyses were then compared and analyzed for diagnostic acuity. Two siblings with transketolase (TKT) deficiency and three unrelated individuals with transaldolase (TALDO) deficiency were identified for inclusion in the study. For both IEMs, targeted polyol testing and untargeted metabolomic testing on urine and/or plasma samples identified typical perturbations of the respective disorder. Additionally, untargeted metabolomic testing revealed elevations in other PPP metabolites not typically measured with targeted polyol testing, including ribonate, ribose, and erythronate for TKT deficiency and ribonate, erythronate, and sedoheptulose 7-phosphate in TALDO deficiency. Non-PPP alternations were also noted involving tryptophan, purine, and pyrimidine metabolism for both TKT and TALDO deficient patients. Targeted polyol testing and untargeted metabolomic testing methods were both able to identify specific biochemical patterns indicative of TKT and TALDO deficiency in both plasma and urine samples. In addition, untargeted metabolomics was able to identify novel biomarkers, thereby expanding the current knowledge of both conditions and providing further insight into potential underlying pathophysiological mechanisms. Furthermore, untargeted metabolomic testing offers the advantage of having a single effective biochemical screening test for identification of rare IEMs, like TKT and TALDO deficiencies, that may otherwise go undiagnosed due to their generally non-specific clinical presentations.

Most severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections in pediatric patients are mild or asymptomatic. However, infants have emerged at higher risk of hospitalization and severe outcomes in pediatric coronavirus disease 2019 (COVID-19). We report a case series of 4 full-term neonates hospitalized with fever and found to have SARS-CoV-2 infection with a spectrum of illness severities. Two neonates required admission to the intensive care unit for respiratory insufficiency and end organ involvement. Half of the patients were found to have a coinfection. One neonate received antiviral therapy with remdesivir and is, to our knowledge, the youngest patient to receive this drug for COVID-19. All neonates had favorable outcomes.

BACKGROUND The HeartMate 3 ventricular assist device (VAD) is a newer centrifugal continuous-flow VAD used for bridge-to-transplant and destination therapy in adults. However, there is limited experience regarding its use in children and adults with complex congenital heart disease (CHD). METHODS The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) is a multicenter learning network comprised of pediatric hospitals implanting VADs in children and adults with complex CHD. We examined the outcomes of patients undergoing HeartMate 3 implantation at an ACTION center between December 2017 and September 2019. RESULTS The HeartMate 3 was implanted in 35 patients at 9 ACTION centers, with a median age of 15.7 (8.8–47.3) years, median weight of 65.7 (19.1–114.1) kg, and median body surface area (BSA) of 1.74 (0.78–2.36) m2. Of the cohort, 14 patients (40%) weighed CONCLUSIONS Use of the HeartMate 3 in ACTION centers was associated with a low incidence of mortality and adverse events. Patients as small as 19 kg (BSA 0.78 m2) were successfully implanted and supported, indicating that this device may be appropriate for older children and small adults.

To provide guidance on the management of Multisystem Inflammatory Syndrome in Children (MIS-C), a condition characterized by fever, inflammation, and multiorgan dysfunction that manifests late in the course of SARS-CoV-2 infection. Recommendations are also provided for children with hyperinflammation during COVID-19, the acute, infectious phase of SARS-CoV-2 infection.The Task Force is composed of 9 pediatric rheumatologists and 2 adult rheumatologists, 2 pediatric cardiologists, 2 pediatric infectious disease specialists, and 1 pediatric critical care physician. Preliminary statements addressing clinical questions related to MIS-C and hyperinflammation in COVID-19 were developed based on evidence reports. Consensus was built through a modified Delphi process that involved anonymous voting and webinar discussion. A 9-point scale was used to determine the appropriateness of each statement (median scores of 1-3 for inappropriate, 4-6 for uncertain, and 7-9 for appropriate). Consensus was rated as low, moderate, or high based on dispersion of the votes. Approved guidance statements were those that were classified as appropriate with moderate or high levels of consensus, which were prespecified before voting.The guidance was approved in June 2020 and updated in November 2020 and October 2021, and consists of 41 final guidance statements accompanied by flow diagrams depicting the diagnostic pathway for MIS-C and recommendations for initial immunomodulatory treatment of MIS-C.Our understanding of SARS-CoV-2-related syndromes in the pediatric population continues to evolve. This guidance document reflects currently available evidence coupled with expert opinion, and will be revised as further evidence becomes available.

Cardiac disease results in significant morbidity and mortality in patients with muscular dystrophy (MD). Single centers have reported their ventricular assist device (VAD) experience in specific MDs and in limited numbers. This study sought to describe the outcomes associated with VAD therapy in an unselected population across multiple centers.We examined outcomes of patients with MD and dilated cardiomyopathy implanted with a VAD at Advanced Cardiac Therapies Improving Outcomes Network (ACTION) centers from 9/2012 to 9/2020.A total of 19 VADs were implanted in 18 patients across 12 sites. The majority of patients had dystrophinopathy (66%) and the median age at implant was 17.2 years (range 11.7-29.5). Eleven patients were non-ambulatory (61%) and 6 (33%) were on respiratory support pre-VAD. Five (28%) patients were implanted as a bridge to transplant, 4 of whom survived to transplant. Of 13 patients implanted as bridge to decision or destination therapy, 77% were alive at 1 year and 69% at 2 years. The overall frequencies of positive outcome (transplanted or alive on device) at 1 year and 2 years were 84% and 78%, respectively. Two patients suffered a stroke, 2 developed sepsis, 1 required tracheostomy, and 1 experienced severe right heart failure requiring right-sided VAD.This study demonstrates the potential utility of VAD therapies in patients with muscular dystrophy. Further research is needed to further improve outcomes and better determine which patients may benefit most from VAD therapy in terms of survival and quality of life.

We sought to characterize strokes in children with ventricular assist devices. Of 407 patients in the ACTION registry (4/1/18-5/3/2021), 45 (11%) experienced 52 strokes (45 ischemic and 7 hemorrhagic). Median time to stroke was 23.5 days and 19/52 (37%) occurred ≤ 10 days. Stroke rate was 0.09 and 0.63 strokes per patient-year for implantable continuous and paracorporeal devices, respectively. Patients with stroke were younger, more likely to have congenital heart disease and have been on extracorporeal membrane oxygenation at time of VAD. Based on these data, ACTION is now focused on decreasing strokes in these higher-risk patients with particular attention to the peri-implant period.

The purpose of this paper is to describe the design and pilot program of a parent designed clothing option for hospitalized infants and children and to better understand the importance and effects of hospital clothing on families. Little research has been done on how clothing a hospitalized child impacts the child's quality of life and their parent's perception of care. Research has been limited to clothing in adults and its relation to infection.A pediatric outfit (the Georgie) for hospitalized infants and children was designed based on insight from bedside nursing, physicians, parents, and supply chain personnel. The garment was pilot tested on select patients from intensive care units of a large children's hospital. A pre- and post-use questionnaire was disseminated with questions focused on aspects of the child's care, comfort in changing child's clothes/diapers, number of times the Georgie was used and comfort level of using the Georgie. Survey responses were summarized using descriptive statistics.Parents overall response to the Georgie was positive with great value placed on having their child dressed. All parents (n = 5) responded that too many lines were an obstacle to having their child dressed. Compared to the hospital Johnny, the Georgie (80%, n = 4) was the most preferred when placement and securement of monitoring lines was taken into consideration with one parent preferring a blanket and diaper only. Nurses felt the benefit outweighed the added effort in dressing the patient. The majority of the nurses had a positive initial reaction to the Georgie (80%, n = 4) and felt the lines or external devices were "very secure/secure" (80%, n = 4) when the patient was wearing the Georgie.Implementing a new family centered care initiative of dressing critically ill patients in the Georgie may improve patient and family's quality of life while hospitalized. A larger scale study is indicated to assess the importance of dressing hospitalized pediatric patients for their families, to clarify the effect on nursing care, to optimize ability to stabilize lines, and to understand logistical issues.

OBJECTIVE: To characterize viscoelastic testing profiles of children with multisystem inflammatory syndrome in children (MIS-C). METHODS: This single-center retrospective review included 30 patients diagnosed with MIS-C from March 1 to September 1, 2020. Thromboelastography (TEG) with platelet mapping was performed in 19 (63%) patients and compared to age- and sex-matched controls prior to cardiac surgery. Relationships between TEG parameters and inflammatory markers were assessed using correlation. RESULTS: Patients with MIS-C had abnormal TEG results compared to controls, including decreased kinetic (K) time (1.1 vs. 1.7 minutes, p

BACKGROUND: Arrhythmogenic cardiomyopathy (ACM) is a variably penetrant disease increasingly identified in young patients. OBJECTIVES: This study sought to describe the diverse phenotype, genotype, and outcomes in pediatric and adolescent patients. METHODS: Records from 1999 to 2016 were reviewed for individuals age

Since 2012, there has been growing use of the HeartWare (Medtronic, Mounds View, MN) intracorporeal continuous flow (CF) ventricular assist device (VAD) in children, despite it not being labeled for use in pediatric patients. We sought to describe the use and outcomes of children with HeartWare VADs.We identified all patients aged19 years and young adults aged 19 to 30 years supported with HeartWare who were entered into the pediatric portion (Pedimacs) of the Interagency Registry for Mechanically Assisted Circulatory Support (Intermacs) and the Intermacs registries, respectively, between September 2012 and June 2017. Adverse events and outcomes were analyzed and compared.We identified 192 children and 247 young adult HeartWare patients. Baseline characteristics of children differed from young adults, with lower median weight of 51.5 kg (range, 13.1-162) vs 75.8 kg (range, 29.8-191; p ≤ 0.0001) and body surface area of 1.5 mSurvival in children supported with HeartWare is encouraging and comparable to young adults; however, adverse events are not uncommon in children. Ongoing evaluation of the HeartWare use in children is necessary to further decrease the rate of adverse events and understand obstacles to discharge.

Background The influence of ventricular assist device (VAD) use in the care of children with end-stage heart failure is growing rapidly through increasing numbers, new devices, expanding indications, and improving outcomes. Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs), a National Institutes of Health–sponsored United States database, provides a platform to understand this emerging population. Methods Between September 19, 2012, and December 31, 2017, 30 hospitals implanted 508 devices in 423 patients aged younger than 19 years. This past year was one of evolution for the database as its management was transitioned to The Society of Thoracic Surgery; therefore, this report does not include data from institutions not under contract by August 1, 2018. Results Of the 423 patients, the diagnosis was cardiomyopathy in 261 (62%), myocarditis in 48 (11%), other in 28 (7%), and congenital heart disease (CHD) in 86 (20%), with 52 of these patients having single-ventricle physiology. The two most common support strategies included left VAD in 342 (81%) and biventricular assist device in 64 (15%). Positive outcome (alive on device or bridge to transplantation/recovery) was 80% at 6 months (overall mortality of 20%). The patient cohort for implantable continuous-flow (IC) pumps (n = 197; age at implant, 13.4 ± 3.8 years; 19% Interagency Registry for Mechanically Assisted Circulatory Support [Intermacs] profile 1, 21% intubated at implant, and 12% with CHD) was significantly different from the paracorporeal continuous-flow (PC) pump cohort (n = 79; age, 3.9 ± 5.2 years; 49% Intermacs profile 1, 86% intubated at implant, and 38% with CHD) and the paracorporeal pulsatile (PP) pump cohort (n = 121; age, 3.3 ± 3.9 years; 41% Intermacs profile 1, 77% intubated at implant, and 21% with CHD). Consistent with their cohort composition, device type positive outcomes at 6 months were PC, 63%; PP, 77%; and IC, 92%. Parametric hazard modeling showed an early hazard for death was associated with Intermacs profile 1, biventricular assist device, percutaneous devices, PC devices, small-volume institutions, low age, and low weight, whereas a constant hazard was associated with intubation and liver dysfunction at time of implant. Conclusions IC VADs are the most common VAD type placed in children. The positive outcomes for the IC VADs exceed 90% at 6 months, which may represent our field’s maturation in both patient selection and timing of implantation. The PP/PC devices are currently limited to supporting our most challenging patients, those weighing less than 20 kg and those with CHD. The introduction of new devices and our communities’ commitment to shared learning and improvement will lead to more pediatric lives saved by VAD support and will also focus on improving the quality of life of children supported with VADs.

Objective: To characterize viscoelastic testing profiles of children with multisystem inflammatory syndrome in children (MIS-C). Methods: This single-center retrospective review included 30 patients diagnosed with MIS-C from January 1 to September 1, 2020. Thromboelastography (TEG) with platelet mapping was performed in 19 (63%) patients and compared to age- and gender- matched controls via Student’s t-test and Wilcoxon rank sum test. Pearson’s and Spearman correlation were used to assess relationships between TEG parameters and inflammatory markers. Results: Patients with MIS-C had abnormal TEG results compared to controls, including decreased K time (1.1 vs. 1.7 min, P

With growing number of pediatric cases of COVID-19, a unique hyper-inflammatory syndrome, linked to SARS-CoV-2 infection, has emerged in children referred to as multisystem inflammatory syndrome in children (MIS-C). This Kawasaki Disease (KD)-like illness has been described across the world. This syndrome shares features of KD, toxic shock syndrome, and macrophage activation syndrome and is associated with significantly elevated inflammatory markers. Everyday there are new data emerging improving the care of these patients. The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) is a collaborative network designed to improve the outcomes of pediatric patients with end-stage heart failure and involves centers from across North America. The committee gathered information concerning COVID-19 anticoagulation practices at various centers and harmonized the data to formulate a set of recommendations.

Initial reports on COVID-19 described children as largely spared from severe manifestations, with only 2–6% of children requiring intensive care treatment. However, since mid-April 2020, clusters of pediatric cases of severe systemic hyperinflammation and shock epidemiologically linked with COVID-19 have been reported. This condition was named as SARS-Cov-2-associated multisystem inflammatory syndrome in children and showed similarities to Kawasaki disease. Here, we present a narrative review of cases reported in literature and we discuss the clinical acute and follow-up management of these patients. Patients with SARS-Cov-2-associated multisystem inflammatory syndrome frequently presented with persistent fever, gastrointestinal symptoms, polymorphic rash, conjunctivitis, and mucosal changes. Elevated inflammatory markers and evidence of cytokine storm were frequently observed. A subset of these patients also presented with hypotension and shock (20–100%) from either acute myocardial dysfunction or systemic hyperinflammation/vasodilation. Coronary artery dilation or aneurysms have been described in 6–24%, and arrhythmias in 7–60%. Cardiac support, immunomodulation, and anticoagulation are the key aspects for the management of the acute phase. Long-term structured follow-up of these patients is required due to the unclear prognosis and risk of progression of cardiac manifestations.Conclusion: Multisystem inflammatory syndrome is a novel syndrome related to SARS-CoV-2 infection. Evidence is still scarce but rapidly emerging in the literature. Cardiac manifestations are frequent, including myocardial and coronary involvement, and need to be carefully identified and monitored over time. What is Known:• Multisystem inflammatory syndrome in children (MIS-C) has been described associated with SARS-CoV-2.What is New:• Patients with MIS-C often present with fever, gastrointestinal symptoms, and shock.• Cardiac involvement is found in a high proportion of these patients, including ventricular dysfunction, coronary artery dilation or aneurysm, and arrhythmias.• Management is based on expert consensus and includes cardiac support, immunomodulatory agents, and anticoagulation.• Long-term follow-up is required due to the unclear prognosis and risk of progression of cardiac manifestation.

Introduction: Thrombosis is a complication associated with Severe Acute Respiratory Syndrome Corona Virus 2 (SARS-CoV-2), Corona Virus Disease 2019 (COVID-19). Although, abnormalities in coagulation markers have been reported in adults with COVID-19, coagulopathy in pediatric patients infected with coronavirus has not been elucidated. Hypothesis: In this study, we evaluated the coagulation profile in pediatric patients with COVID-19 using Thromboelastography (TEG). Methods: This is a retrospective study of pediatric (≤18yrs) patients with seropositive COVID-19 (N=35) regardless of symptom status. Coagulation markers including TEG were obtained from review of medical records under an IRB approved study. All TEG parameters were compared between patients with COVID-19 and age matched controls without COVID-19 (N=35). Results: Regardless of symptom status, COVID-19 patients had significantly lower R-time (5.3±2.7 min vs. 6.5±1.5 min; p=0.03 ), K-time (1.1±0.3 min vs. 1.8±1.1 min; p ) and fibrinolysis (1.7±1.7% vs. 3.2±3.6%; p=0.04 ); and significantly elevated angle (73.6±4.8 deg vs.66.7±6.2 deg; p ), maximum amplitude (68.9±6.1 mm vs. 59.5±8.7 mm; p ) and overall clot strength G (11.7±3.3 Kdynes/cm 2 vs. 7.8±2.4 Kdynes/cm 2 ; p ). Whereas the standard coagulation tests such as PT/INR, PTT, Thrombin time and Heparin levels were within the normal pediatric reference ranges. D-dimer expression was also higher (0.94 μg/ml) in COVID-19 patients compared to the normal pediatric reference range (p ). Conclusions: Abnormal expression of all TEG parameters and D-dimer is consistent with a hypercoagulable profile. TEG may be useful in reducing thrombosis-risk in patients with COVID-19 by guiding anticoagulation management.

Given the adverse event rates involving bleeding and thrombosis among children on ventricular assist devices (VADs), anticoagulant management has become a focal point for quality improvement and innovation. There may be advantages to using direct thrombin inhibitors, such as bivalirudin, though this has not been fully explored. As the percent time in therapeutic range (%TTR) for anticoagulants is classically associated with improved clinical outcomes, we evaluated the %TTR for bivalirudin among pediatric VAD recipients. Using a modification of the Rosendaal method, %TTR was calculated using activated partial thromboplastin time measurements for 11 VAD recipients in the early postoperative period (postoperative days 0-14) and for the duration of VAD support. In the initial 2 weeks after VAD implant, mean %TTR was 68.7 (±13.0). During the entire support course, the mean %TTR improved to 79.6 (±11.0). There was an era effect with improving %TTR in the latter half of the study period. We report very good %TTR for bivalirudin both in the first 2 weeks post implant and this improved over the duration of support. Because %TTR reflects the degree of safety and efficacy in chronic anticoagulation, this relatively high %TTR among a diverse, often critically ill cohort suggests that bivalirudin may be a promising agent. Although this study was underpowered to comprehensively evaluate adverse events on bivalirudin, this represents an important next step for larger scale study.

Thrombotic and bleeding complications have historically been major causes of morbidity and mortality in pediatric ventricular assist device (VAD) support. Standard anticoagulation with unfractionated heparin is fraught with problems related to its heterogeneous biochemical composition and unpredictable pharmacokinetics. We sought to describe the utilization and outcomes in children with paracorporeal VAD support who are treated with direct thrombin inhibitors (DTIs) antithrombosis therapy. Retrospective multicenter review of all pediatric patients (aged

Introduction Guidelines recommend trans-esophageal echocardiography (TEE) for patients with atrial fibrillation (AF) or atrial flutter (AFL) for > 48 hours, due to risk of intra-cardiac thrombus formation. With growing evidence that AFL in adults with structurally normal hearts has less thrombogenic potential compared to AF, and the need for TEE questioned, we compared prevalence of intra-cardiac thrombus detected by TEE in pediatric and congenital heart disease (CHD) patients presenting in AF and AFL. Methods/results Single center, cross-sectional analysis for unique first-time presentations of patients for either AF, AFL or intra-atrial reentrant tachycardia (IART) between 2000-2019. Patients were categorized by presenting arrhythmia (AF vs. AFL/IART), with exclusion of other forms of atrial tachycardia, hemodynamic instability, chronic anti-coagulation prior to TEE, and presentation for reason other than TEE examination for thrombus. A total of 201 patients had TEE with co-diagnosis of AF or AFL. Of these, 105 patients (29 AF, 76 AFL) met inclusion criteria, with no difference in age between AF (median 24.9 years; IQR 18.6-38.3 years) and AFL/IART (23.3 years; 15.4-38.4 years). The prevalence of thrombus in the entire cohort was 9.5%, with no difference between AF (13.8%) and AFL groups (7.9%), p=0.46. Patients with thrombus demonstrated no difference in age, systemic ventricular function, cardiac complexity, or CHADS2/CHA2DS2VASc score at presentation. Conclusions The risk for intra-cardiac thrombus is high in the pediatric and CHD population, with no apparent distinguishing factors to warrant a change in the recommendations for TEE, with all levels of cardiac complexity being at risk for clot. This article is protected by copyright. All rights reserved.

Improving the outcomes of pediatric patients with congenital heart disease with end-stage heart failure depends on the collaboration of all stakeholders; this includes providers, patients and families, and industry representatives. Because of the rarity of this condition and the heterogeneity of heart failure etiologies that occur at pediatric centers, learnings must be shared between institutions and all disciplines to move the field forward. To foster collaboration, excel discovery, and bring data to the bedside, a new, collaborative quality improvement science network-ACTION (Advanced Cardiac Therapies Improving Outcomes Network)-was developed to meet the needs of the field. Existing gaps in care and the methods of improvement that will be used are described, along with the mission and vision, utility of real-world data for regulatory purposes, and the organizational structure of ACTION is described.

Infants with a single ventricle can develop systemic ventricular dysfunction (SVD) after stage 1 operation, but available information is sparse. We reviewed our patients having Norwood, Sano, or hybrid procedures to better understand this problem. We conducted a retrospective, case-controlled cohort study of 267 patients having stage1 operation, examining outcomes between stages 1 and 2 (survival and subsequent cardiac surgeries), predictor variables, and histology of hearts explanted at transplantation. SVD developed in 32 (12%) patients and resolved in 13 (41%); mean age of onset was 3.0 ± 1.63 months; median = 2.79. SVD was not associated with cardiac anatomy, type of stage 1 procedure, weight, coronary abnormality, or atrioventricular valve regurgitation. The mean age of resolution = 12.1 ± 9.6 months; median = 6.3, and resolution may have been more likely with a systemic LV than RV (p = 0.067). Outcomes for the entire SVD group were less favorable than for those without, but patients with resolution of SVD had outcomes at least as good those without SVD. Myocardial histology (n = 4) suggested chronic ischemia. The risk of SVD after stage 1, while low, may be a fundamental feature of this patient population. SVD occurs with either a systemic RV or LV, although patients with a systemic LV may be more likely to have resolution than those with an RV. We identified no predictor variables, but histologic findings suggest chronic ischemia may be involved. Given the low incidence of SVD, multi-center studies will be required to better define predictors of onset and resolution.

Purpose ACTION (Advanced Cardiac Therapies Improving Outcomes Network) is a multi-faceted quality improvement (QI) and research network with the goal of improving pediatric and congenital heart disease VAD and heart failure (HF) outcomes. This report describes ACTION initiatives. Methods ACTION includes QI, educational activities, and a data registry. Stakeholders include providers, patients and families, and industry partners. The core values of ACTION include collaboration and transparency. ACTION started in 2017, with data collection beginning in April 2018. This report utilizes ACTION data and describes selected completed and ongoing initiatives. Results The ACTION registry report is shared with sites as an interactive dashboard (refreshed monthly). A comprehensive data quality strategy includes data queries and adjudication of critical events. There are 50 US/Canadian sites in the network and 34 of the sites contribute data. There are additional international sites that participate in all activities except patient-level data collection. The registry includes 388 patients implanted with 511 devices with 51,411 device days. DCM (203) and CHD (143) are the major underlying diagnoses. EXCOR (148), HVAD (97), and HM3 (70), are the most common end devices. Survival is 91% (including ongoing support, transplant and wean). AE rates are: stroke 11%, bleeding 21%, and infection 26%. The dashboard allows intuitive examination of subgroups, e.g., stroke/bleeding rate in patients less than 10 kg/on a specific device. Initial QI projects include an initiative (ABC of stroke), that used a bundle of interventions to reduce stroke during VAD support; a discharge planning project; a telehealth support project; and a harmonization initiative to reduce burden of protocol development for sites. Additionally, a patient-facing education website about VAD/HF care is being deployed and a HF project is collecting baseline data for acute decompensated HF. ACTION data have been used to support regulatory (FDA) approval of selected devices. Conclusion ACTION, a collaborative, multi-stakeholder network, has undertaken numerous approaches to improve outcomes for children with HF and VADs. In keeping with the values of transparency and collaboration, the ACTION annual reports will be made available in their entirety in a patient-facing version, via the ACTION website.

Adults with congenital heart disease represent an expanding and unique population of patients with heart failure (HF) in whom the use of mechanical circulatory support (MCS) has not been characterized. We sought to describe overall use, patient characteristics, and outcomes of MCS in adult congenital heart disease (ACHD).All patients entered into the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) between June 23, 2006, and December 31, 2015, were included. Patients with ACHD were identified using pre-operative data and stratified by ventricular morphology. Mortality was compared between ACHD and non-ACHD patients, and multivariate analysis was performed to identify predictors of death after device implantation.Of 16,182 patients, 126 with ACHD stratified as follows: systemic morphologic left ventricle (n = 63), systemic morphologic right ventricle (n = 45), and single ventricle (n = 17). ACHD patients were younger (42 years ± 14 vs 56 years ± 13; p0.0001) and were more likely to undergo device implantation as bridge to transplant (45% vs 29%; p0.0001). A higher proportion of ACHD patients had biventricular assist device (BiVAD)/total artificial heart (TAH) support compared with non-ACHD patients (21% vs 7%; p0.0001). More ACHD patients on BiVAD/TAH support were INTERMACS profile 1 compared with patients on systemic left ventricular assist device (LVAD) support (35% vs 15%; p = 0.002). ACHD and non-ACHD patients with LVADs had similar survival; survival was worse for patients on BIVAD/TAH support. BiVAD/TAH support was the only variable independently associated with mortality (early phase hazard ratio 4.4; 95% confidence interval, 1.8-11.1; p = 0.001). For ACHD patients receiving MCS, ventricular morphology was not associated with mortality.ACHD patients with LVADs have survival similar to non-ACHD patients. Mortality is higher for patients requiring BiVAD/TAH support, potentially owing to higher INTERMACS profile. These outcomes suggest a promising role for LVAD support in ACHD patients as part of the armamentarium of therapies for advanced HF.

Background Expanded use of pediatric ventricular assist devices (VADs) has decreased mortality in children awaiting heart transplantation. Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs), a National Heart, Lung, and Blood Institute–sponsored North American database, provides a platform to understand this emerging population. Methods Between September 2012 and September 2016, patients aged younger than 19 years who underwent VAD implantation were enrolled in Pedimacs. FDA approved durable devices as well as temporary support devices were included. The second annual report updates the current Pedimacs data. Patients implanted with temporary devices are included in Pedimacs and this analysis includes this group of paracorporeal continuous flow VADs. Results Over the 4 years, 42 hospitals implanted 432 devices in 364 patients less than 19 yrs of age. Diagnoses included cardiomyopathy in 223 (61%), myocarditis in 41 (11%), and congenital heart disease in 77 (21%), of which 48 had single-ventricle physiology. At implant, 87% were at Intermacs patient profile 1 or 2. The age distribution of children (59% male) supported on VAD included 69 (19%) aged younger than 1 year, 66 (18%) aged 1 to 6 years, 56 (15%) aged 6 to 10 years, and 173 (48%) aged 11 to 19 years. Median follow-up was 2.2 months (range, 1 day to 41.5 months). Median (interquartile) age at implant was 1.7 (0.3–10.0) years for paracorporeal continuous-flow pumps ( n = 60), 1.7 (0.4–5.3) years for paracorporeal pulsatile pumps ( n = 105), and 15.0 (11.3–16.9) years for implantable continuous-flow pumps ( n = 174). Support strategies included LVAD in 293 (80%), biventricular device in 55 (15%), and total artificial heart in 8 (2%). Nearly 50% of patients underwent transplantation within 6 months, with overall mortality of 19%. Adverse event burden continues to be high. Conclusions Pedimacs constitutes the largest longitudinal pediatric VAD registry. Preimplant data across centers will be helpful at creating shared protocols with which to improve outcomes. Adverse events continue to be the major challenge, especially among the young critically ill children with complex congenital disease.

OBJECTIVE This quality improvement study was implemented to demonstrate consistent and reliable post procedure anticoagulation for patients undergoing left-sided ablations. We evaluated the safety and efficacy of anticoagulation practice during a transition from anticoagulation with overnight infusion of unfractionated heparin to a single subcutaneous injection of low molecular weight heparin. METHODS Outcomes for patients who received unfractionated heparin from January 2014 to October 2014, were compared with outcomes of patients who received low molecular weight heparin from October 2014 to October 2015. Complications prepractice and postpractice change were documented and compared to establish confidence in the practice change and confirm the safety of the anticoagulation therapy management. RESULTS There were no differences in the type or frequency of complications/adverse events demonstrated between the patients who had received unfractionated heparin for anticoagulation prophylaxis and those who received low molecular weight heparin. No thromboembolic events were reported or documented with either anticoagulation strategy. After confidence in the safety and efficacy of the practice change was established, a decision was made to discharge patients home the same day as there procedure, effectively reducing inpatient bed days and overall costs. CONCLUSION Administration of low molecular weight heparin provides predictable anticoagulation and equally safe as unfractionated heparin when administered to patients post left-sided ablation. A secondary gain has been reduction of procedural costs by elimination of the previously required inpatient observation stay.

To describe the safety and efficacy of warfarin for patients with Kawasaki disease and giant coronary artery aneurysms (CAAs, ≥8 mm). Giant aneurysms are managed with combined anticoagulation and antiplatelet therapies, heightening risk of bleeding complications.We reviewed the time in therapeutic range; percentage of international normalization ratios (INRs) in range (%); bleeding events, clotting events; INRs ≥6; INRs ≥5 and6; and INRs1.5.In 9 patients (5 male), median age 14.4 years (range 7.1-22.8 years), INR testing was prescribed weekly to monthly and was done by home monitor (n = 5) or laboratory (n = 3) or combined (1). Median length of warfarin therapy was 7.2 years (2.3-13.3 years). Goal INR was 2.0-3.0 (n = 6) or 2.5-3.5 (n = 3), based on CAA size and history of CAA thrombosis. All patients were treated with aspirin; 1 was on dual antiplatelet therapy and warfarin. The median time in therapeutic range was 59% (37%-85%), and median percentage of INRs in range was 68% (52%-87%). INR6 occurred in 3 patients (4 events); INRs ≥5 6 in 7 patients (12 events); and INR1.5 in 5 patients (28 events). The incidence of major bleeding events and clinically relevant nonmajor bleeding events were each 4.3 per 100 patient-years (95% CI 0.9-12.6). New asymptomatic coronary thrombosis was detected by imaging in 2 patients.Bleeding and clotting complications are common in patients with Kawasaki disease on warfarin and aspirin, with INRs in range only two-thirds of the time. Future studies should evaluate the use of direct oral anticoagulants in children as an alternative to warfarin.

Mechanical circulatory support in the form of ventricular assist devices (VADs) in children has undergone rapid growth in the last decade. With expansion of device options available for larger children and adolescents, the field of outpatient VAD support has flourished, with many programs unprepared for the clinical, programmatic, and administrative responsibilities. From preimplantation VAD evaluation and patient education to postimplant VAD management, the VAD program, staffed with an interdisciplinary team, is essential to providing safe, effective, and sustainable care for a new technology in an exceedingly complex patient population. Herein, this paper describes the Boston Children's Hospital VAD experience over a decade and important lessons learned from developing a pediatric program focusing on a high-risk but low-volume population. We highlight the paramount role of the VAD coordinator, clinical infrastructure requirements, as well as innovation in care spanning inpatient and outpatient VAD supports at Boston Children's Hospital.

Purpose Establishing and maintaining target anticoagulation (AC) levels is perceived as a way to mitigate the risk of bleeding and thrombotic complications in pediatric ventricular assist device (VAD) support. To better understand potential reasons of having monitoring labs outside institutionally-set goal ranges, we surveyed centers to understand perceived barriers. Methods As part of the Advanced Cardiac Therapies Improving Outcomes Network (ACTION), a 37-center collaborative, we surveyed participating VAD centers across North America. The survey was comprised of multiple choice questions addressing reasons for out of range coagulation labs. Specifically, asking centers to rate the frequency of occurrences; low, happens rarely, medium, happens often, high, happens almost daily and not applicable, meaning this barrier does not exists. We then weighted the average responses. Results Of the 49 responses received from the pediatric VAD-ACTION centers that responded to a 4-question multiple choice survey, the most commonly reported barrier was contamination of lab samples by sampling from a line infusing an anticoagulant through it in over 65% of respondents. This was followed by multiple providers involved in managing AC and micro-managing lab results as the second and third chosen as barriers reported in just under 50%. Interestingly, lack of documentation and obscure guidelines were not perceived as frequent causes of out of range values. Conclusion While achieving and maintaining stable anticoagulation is the goal of therapy, understanding perceived obstacles is vital to targeted intervention. Targeted interventions to decrease the most common barriers, such as contaminated labs, will guide future ACTION quality improvement initiatives.

Purpose The use of bivalirudin has increased dramatically for primary thromboprophylaxis in pediatric paracorporeal ventricular assist device (VAD) support with limited data on optimal monitoring of effect. Partial thromboplastin time (aPTT) is commonly used for monitoring of bivalirudin, but can be impacted by line heparin, variability in assays and other patient factors. As such, we sought to examine the correlation of PTT to International Normalized Ratio (INR), as a possible monitoring tool for bivalirudin in in pediatric VAD patients. Methods Retrospective single center study of all children aged Results From 2015-2019, eleven patients (4 female), median age 5 months (range 0.5-40 months), weighing 7.8 kg (3.4-17.0kg) and body surface area of 0.4 m2 (range 0.2-0.7 m2) at time of implant were treated with bivalirudin for paracorporeal VAD thromboprophylaxis, of which 6 were on EXCOR devices, 4 were transitioned between continuous flow and EXCOR devices, and 1 patients was on CardioHelp extracorporeal membrane oxygenator connected to EXCOR cannula. A total of 900 days of paracorporeal VAD support generated 3,143 laboratory values for correlation. Correlations varied between aPTT assays, with highest median correlation coefficient for HA aPTT to INR of 0.72 (min 0.57 to max 0.81) in 4 patients with a total of 662 paired samples, as compared to correlation coefficient for aPTT to INR of 0.56 (min 0.32-0.91) in 10 patients with a total of 1,188 paired samples. Correlations varied between patients, with no association to age, device type or survival outcomes. The most important determinant for lack of correlation between aPTT to INR was concomitant administration of line heparin. Conclusion Patients treated with bivalirudin demonstrate individual correlations of their INR to PTT, with highest correlation to HA PTT by accounting for exogenous line heparin. This preliminary data provides foundation for ongoing study into the utility of INR for bivalirudin dosing and possible future application of point of care INR testing in this population.

Apixaban is well studied in adults; paediatric data are extremely limited. We describe 3 children (age 2–6 years, weight 13–17 kg) with congenital heart disease who developed intracardiac thrombosis, and in whom typical treatments had failed (in 1) or were considered impractical (in 2). All were treated with apixaban 2.5 mg orally twice daily with complete (in 2) or partial (in 1) thrombus resolution and no clinical bleeding events.

Background Enoxaparin is a commonly used anticoagulant for treatment of thrombosis in pediatric patients. Its use in pediatric cardiac patients has not been well described. We sought to examine the utilization trends, dosing, and safety of enoxaparin in pediatric cardiac patients. Methods Retrospective study of pediatric cardiac patients treated with enoxaparin from 2011 to 2018. Starting and final doses of enoxaparin, number of dose adjustments, and time to therapeutic anti-factor Xa level were compared between age groups utilizing the Kruskal Wallis test with the Dunn test for pairwise comparisons. Results Seventy-one patients were treated with 77 courses of enoxaparin. Most enoxaparin courses were started on patients aged >12 months (40%) and for arterial thrombosis (22 patients, 29%). The most common cardiac diagnosis was hypoplastic left heart syndrome (12 patients, 17%). The mean final dose of enoxaparin was significantly different when comparing patients 0–3-month, 3–12 month, and >12 months (2.1 ± 0.5 mg/kg vs. 1.5 ± 0.4 mg/kg vs. 1.2 ± 1.4 mg/kg, respectively; p 12-month age group (0.4 ± 0.4 mg/kg vs. 0.4 ± 0.4 mg/kg vs. 0.1 ± 0.3 mg/kg, respectively; p 12 months (2.2 ± 1.6 vs. 1.4 ± 1.4, p = 0.02). Antiplatelet therapy was used in 74% of courses. Adverse events were rare and had no relationship to antiplatelet use. Conclusions Enoxaparin is an effective and safe anticoagulant in pediatric cardiac patients. Initial enoxaparin dosing should be increased from current recommended doses in patients between 3 and 12 months of age to achieve more timely therapeutic anticoagulation.

Purpose We sought to characterize strokes in children supported with ventricular assist devices (VADs) in the Advanced Cardiac Therapies Improving Outcomes Network (ACTION). Methods All patients entered in the ACTION registry (4/1/2018-6/8/2020) were included. Stroke was defined by pre-specified criteria and categorized as hemorrhagic or ischemic in etiology. All strokes were adjudicated by the data coordinating center and the adjudication committee which is comprised of members from multiple sites. Results Of 270 patients in the ACTION registry, 26 patients (9.6%) experienced 28 strokes (25 ischemic and 3 hemorrhagic). Of these 26 patients, 12 (46%) were ≤1yo, 12 (46%) had congenital heart disease, 8 (26%) had preimplant ECMO use, 23 (88%) were on paracorporeal VADs and 3 (15%) had intracorporeal VADs. The median time to stroke was 17.5 days. There were 11 (39%) and 17 (60%) strokes that occurred within 7 and 30 days, respectively, and all strokes occurring ≤30 days post-implant were ischemic. On paracorporeal VADs, there were 23 ischemic (EXCOR=9, Centrimag=6, Pedimag=6, Rotaflow=2) and 2 hemorrhagic (EXCOR=1, Centrimag=1) strokes. With intracorporeal support there were 2 ischemic (HVAD=1, HM3=1) and 1 hemorrhagic (HVAD=1) strokes. The overall stroke rate was 145.4 per 10,000 patient-days. Mortality rates were significantly higher for patients who did (10/26; 38.5%) vs. those who did not (32/144; 13.1%) experience a stroke (p Conclusion Although the stroke rate in children on VADs has decreased over time, strokes still occur in 1/10 patients and are associated with significantly increased mortality. The large majority of strokes are ischemic in nature, associated with paracorporeal VADs, and occur within the first 30 days after implant. About half of the children experiencing strokes on a VAD are ≤1yo and/or have congenital heart disease. Based on these data, ACTION is focusing ongoing quality improvement projects on decreasing strokes for children on paracorporeal devices with particular attention to the early post-implant period.

Purpose Cardiac disease results in significant morbidity and mortality in patients with muscular dystrophy (MD). Single centers have described their ventricular assist device (VAD) experience in this population, however broader applicability and outcomes remain unknown. Methods We examined outcomes of all patients with MD and dilated cardiomyopathy implanted with a VAD at Advanced Cardiac Therapies Improving Outcomes Network (ACTION) centers 9/2012-9/2020. Results A total of 19 VADs were implanted in 18 patients across 10 ACTION sites. The median duration of support was 252 days (range 11-1681 days). The majority of pts had dystrophinopathy (66%) and the median age at implant was 17.2 years (Table). Device strategy was bridge to transplant in 5 (27.8%), 4 of whom survived to transplant. The frequency of a positive outcome (transplanted or alive on device) at 6 months and 1 year were 89% and 84%, respectively (Figure). A single patient suffered a stroke (hemorrhagic), 2 patients developed sepsis, 1 patient developed respiratory failure requiring tracheostomy and 4 patients patient developed right heart failure (1 of whom required temporary right VAD). Conclusion In selected MD patients, VADs can serve as effective bridges to HT and chronic therapy. Right heart failure and infectious complications were relatively common, while stroke and respiratory failure were rare. Further research is needed to understand the patients who may benefit most from VAD therapy in terms of survival and quality of life and how to mitigate the risks the of the adverse events identified in the current report.

Continuous-flow (CF) ventricular assist devices (VADs) have largely replaced pulsatile-flow VADs in adult patients. However, there are few data on CF VADs among pediatric patients. In this study we aimed to describe the overall use, patients' characteristics and outcomes of CF VADs in this population.The Pediatric Interagency Registry for Mechanical Circulatory Support (PediMACS) is a national registry for U.S. Food and Drug Adminstration (FDA)-approved VADs in patients19 years of age. Patients undergoing placement of durable CF VADs between September 2012 and June 2015 were included and outcomes were compared with those of adults from the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS).CF VADs were implanted in 109 patients at 35 hospitals. The median age at implantation was 15 years (2.8 to 18.9 years) and median weight was 62 kg (range 16 to 141 kg). The underlying disease was cardiomyopathy in 89 (82%) patients. The INTERMACS level at time of implant was Level 1 in 20 (19%), Level 2 in 64 (61%) and Levels 3 to 7 in 21 (20%) patients. Most were implanted as LVADs (n = 102, 94%). Median duration of support was 2.3 months (range1 day to 28 months). Serious adverse event rates were low, including neurologic dysfunction (early event rate 4.1 per 100 patient-months with 2 late events). Competing outcomes analysis at 6 months post-implant indicated 61% transplanted, 31% alive with device in place and 8% death before transplant. These outcomes compared favorably with the 3,894 adults supported with CF VADs as a bridge to transplant.CF VADs are commonly utilized in older children and adolescents, with excellent survival rates. Further study is needed to understand impact of patient and device characteristics on outcomes in pediatric patients.

The last five yr have been monumental for the pediatric heart failure community. In the US, the most notable has been the FDA approval of the first pediatric specific device (Berlin Heart EXCOR(®) ; Berlin Heart, Inc., Berlin, Germany). Subsequently, the field of heart failure has gained a great deal of knowledge regarding the nuances of MCS in children. Despite FDA approval in the US, the Berlin EXCOR(®) is only currently indicated for in-hospital use. Due to the limitations with discharge and the positive in- hospital experiences with the Berlin EXCOR(®) , there has been an increased interest in the implantation of adult durable devices into children. While many institutions have focused their intial efforts on the first phase of care within the hospital, they are now ready to tackle the challenge of how to safely transition children to the community setting.

Purpose Ventricular assist devices (VADs) increase waitlist survival, yet the risk of stroke is not trivial. There is a knowledge gap regarding the impact of these strokes on heart transplant outcomes. We sought to analyze how strokes during VAD support impact post-transplant (post-Tx) outcomes in children. Methods 520 pediatric ( Results Overall, there were 146 (28%) VAD patients with a pre-Tx stroke; 86 (59% of stroke patients) of them were transplanted at a median of 57 [17-102] days from stroke, significantly lower than the no stroke cohort (90%; p Conclusion Patients with stroke on VAD once transplanted, enjoy similar survival compared to no stroke patients. Allowing children who suffer stroke while on VAD support to have more time to recover appears to improve post-Tx outcomes.