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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.

The number of potential pediatric heart transplant recipients continues to exceed the number of donors, and consequently the waitlist mortality remains significant. Despite this, around 40% of all donated organs are not used and are discarded. This document (62 authors from 53 institutions in 17 countries) evaluates factors responsible for discarding donor hearts and makes recommendations regarding donor heart acceptance. The aim of this statement is to ensure that no usable donor heart is discarded, waitlist mortality is reduced, and post-transplant survival is not adversely impacted.

BACKGROUND The influence of Fontan-associated protein-losing enteropathy's (PLE) severity, duration, and treatment on heart transplant (HTx) outcomes is unknown. We hypothesized that long-standing PLE and PLE requiring more intensive therapy are associated with increased post-HTx mortality. METHODS This 12-center, retrospective cohort study of post-Fontan patients with PLE referred for HTx from 2003 to 2015 involved collection of demographic, medical, surgical, and catheterization data, as well as PLE-specific data, including duration of disease, intensity/details of treatment, hospitalizations, and complications. Factors associated with waitlist and post-HTx outcomes and PLE resolution were sought. RESULTS Eighty patients (median of 5 per center) were referred for HTx evaluation. Of 68 patients listed for HTx, 8 were removed due to deterioration, 4 died waiting, and 4 remain listed. In 52 patients undergoing HTx, post-HTx 1-month survival was 92% and 1-year survival was 83%. PLE-specific factors, including duration of PLE pre-HTx, pre-HTx hospitalizations, need for/frequency of albumin replacement, PLE therapies, and growth parameters had no association with post-HTx mortality. Immunosuppressant regimen was associated with mortality; standard mycophenolate mofetil immunotherapy was used in 95% of survivors compared with only 44% of non-survivors (p = 0.03). Rejection (53%) and infection (42%) post-HTx were common, but not associated with PLE-specific factors. PLE resolved completely in all but 1 HTx survivor at a median of 1 month (interquartile range 1 to 3 months); resolution was not affected by PLE-specific factors. CONCLUSIONS PLE severity, duration, and treatment do not influence post-HTx outcome, but immunosuppressive regimen may have an impact on survival. PLE resolves in nearly all survivors.

BACKGROUND Renal dysfunction (RD) is prevalent among pediatric patients with advanced heart failure. Data are limited regarding changes in renal function after left ventricular assist device (LVAD) placement in this population. METHODS Pediatric LVAD recipients enrolled in the Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) between September 19, 2012 and June 30, 2016 were included. Longitudinal changes in renal function were analyzed for the entire cohort as well as subgroups stratified by patient and device characteristics. Logistic regression was used to attempt to identify factors associated with lack of improvement in renal function after LVAD placement. Post-LVAD outcomes were assessed using the Kaplan‒Meier method. RESULTS Data from 247 patients from 39 centers were analyzed. Baseline RD (estimated glomerular filtration rate [eGFR]

Purpose We sought to evaluate the association between early-onset cardiac allograft vasculopathy (CAV) [ Methods Data from the Pediatric Heart Transplant Society (PHTS) for pediatric ( Results Over a median follow-up period of 4.1 (IQR 1.3 - 8.3) years, angiographic evidence of CAV was identified in 17% (885/5,075) of patients, 28% (252/885) of which were early-onset CAV. Compared to those who developed CAV >10 years post HT, patients with early-onset CAV were older at transplant (8.3 +/- 6.2 vs. 3.8 +/- 4.8 years, p Conclusion Following diagnosis, the progression of CAV & graft survival was similar for patients with early-onset CAV compared with CAV at other time points. These findings suggest that early-onset CAV does not necessarily represent a more aggressive phenotype of disease.

Historically, the "temporary" or short-term ventricular assist device (VAD) was used only as a quick bridge to recovery for children with an acute process. In the current era, the devices that were originally used for temporary support are now being used to support children for longer durations and for a variety of indications. In this study we aimed to describe the overall use, patients' characteristics and outcomes of "temporary" VAD use in children.The Pediatric Interagency Registry for Mechanical Circulatory Support (PediMACS) is a National Institutes of Health-supported national registry for United States Food and Drug Administration-approved VADs in patients19 years of age at the time of VAD implantation (either durable or temporary VAD). Patients undergoing placement of a device classified as a temporary VAD between September 19, 2012 and June 30, 2016 were included.Temporary VADs were implanted in 63 patients at 20 centers, accounting for 19% of all pediatric VAD patients entered into PediMACS. The median age at implantation was 3.7 (range1 day to 18) years. The underlying diseases were: congenital heart disease in 26 (41%), 20 of whom were classified as single ventricle; cardiomyopathy in 25 (40%); and myocarditis/rejection in 12 (19%). Patients were predominately Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) Profile 1 (51%), and 10 patients (16%) had previously been supported with extracorporeal membrane oxygenation. Median duration of support was 15 (range1 day to 227) days, with 41 patients (65%) being on support for ≥10 days. The most frequent adverse events were bleeding (29% of patients) and neurologic dysfunction (24% of patients). Overall, 71% (45) achieved a positive outcome (defined as bridge to recovery [30%], transplantation [17%], alive on device [2%] or transition to durable VAD [22%]). Eighty-eight percent (n = 22) of the cardiomyopathy patients and 60% (n = 12) of the single-ventricle patients achieved a favorable outcome.Devices historically classified as "temporary" pumps are being used not only as a short-term mechanical circulatory support strategy but also as a longer term support strategy. In this multi-institutional, high-acuity, complex patient cohort, the use of "temporary" VADs resulted in a positive outcome (bridge to transplant, recovery durable device or alive) in 71% of patients.

Purpose Plastic bronchitis is a rare post Fontan complication with limited treatment options. Heart transplantation has evolved as a potential curative option, but outcomes have not been well-defined. This study aims to assess contemporary waitlist and post-transplant outcomes in patients with plastic bronchitis. Methods All Fontan patients were identified in the Pediatric Heart Transplant Society database (2010-2018). Waitlist and post-transplant outcomes were compared between Fontan patients with and without plastic bronchitis. Competing outcomes and Kaplan Meier analyses were used to assess the impact of plastic bronchitis on waitlist and post-transplant survival. A secondary analysis excluded those with protein losing enteropathy (PLE) from the comparison cohort. Results Of 645 Fontan patients listed for heart transplant, 69 (8%) had plastic bronchitis. At listing, patients with plastic bronchitis were younger (9.2 vs 10.6 years, p=0.02), but had no other differences at baseline including hemodynamic data. Fewer Fontan patients with plastic bronchitis were listed in the more recent era (46 [15.4%] in 2010-2014 vs. 23 [6.6%] in 2015-2018, p Conclusion Contrary to prior reports, in this large series plastic bronchitis did not have a negative impact on survival to or after heart transplantation in Fontan patients. A 50% reduction in listing in the most recent era suggests improvement in medical management of this rare and life-threatening condition.

Endomyocardial biopsy (EMB) with fluoroscopy is used for rejection surveillance in pediatric heart transplantation. Lowering frame rate may reduce radiation, but decreases temporal resolution and image quality. We undertook a quality initiative reducing frame rate from 10 frames per second (FPS) to 5 FPS. To assess whether lowering frame rate can reduce radiation exposure without compromising safety, data on EMBs from 9/2009 to 4/2013 without angiography or intervention were reviewed. Effective dose was calculated from dose area product (DAP) and fluoroscopy time. Complications were reviewed. Independent t test compared pre- and post-data and a general linear model were used to control for confounders. Paired t test of most proximate data was used for pts with EMB before and after our change. Eighty-six patients had 543 EMB. After adjusting for weight, attending, and presence of a fellow, the lower FPS group had a 60.3% reduction in DAP (p 1 grade by ECHO. There were no deaths or perforations. Lowering the frame rate reduces radiation exposure by >50% without compromising safety. Efforts to further minimize radiation exposure of this vulnerable population should be considered.

1) Lipshultz SE, et al. The incidence of pediatric cardiomyopathy in two regions of the United States. N Engl J Med 2003;348:1647-1655. 2) Webber SA, et al. Outcomes of restrictive cardiomyopathy in childhood and the influence of phenotype: a report from the pediatric cardiomyopathy registry. Circulation 2012:126:1237-1244. 3) Denfield SW and Webber SA. Restrictive cardiomyopathy in childhood. Heart Failure Clin 2010;6:445-452. 4) Ackerman MJ, et al. HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies this document was developed as a partnership between the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA). Heart Rhythm 2011;8(8):1309-1338. The authors have no financial disclosures. Disclosures Conclusions