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Heart failure is the leading cause of morbidity and mortality in adults with congenital heart disease. However, for many in this population, heart transplantation is not possible or requires longer wait times, necessitating prolonged circulatory support. The medium to long-term durable ventricular assist device therapy provides a possible solution. We analyzed outcomes of 9 patients with congenital heart disease and at least 3 years of durable mechanical support, all age 18 or older at the time of ventricular assist device implantation at our affiliated pediatric and adult hospitals. Palliated congenital anatomies varied and included biventricular physiologies as well as single ventricles with Fontan circulation. The median duration of support was 4.2 years, including 3 patients successfully bridged to transplant, averaging 2.1 years on the waitlist. Device-related complications were infrequent with HeartMate 3, a feasible and sustainable option for either bridge to transplant or destination strategies in adults with congenital heart disease., Competing Interests: Disclosure statement C.R.B. reports a financial relationship with Abbott; H.T. reports a relationship with Abbott; I.A. serves as a consultant and proctor for Berlin Heart, Inc., Medtronic Inc., Jarvik Inc., BiVACOR Inc., and Sony-Olympus Medical Solutions Inc.; A.C. reports financial relationships with Medtronic Inc. and Abbott. The remaining authors have no conflicts of interest to report., (Copyright © 2024 International Society for the Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Background: The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) began in 2018 as a collaborative learning health system committed to improving outcomes in pediatric heart failure, including children and adults with congenital heart disease, supported with ventricular assist devices (VADs). This report describes patient and device characteristics, and outcomes through 1-year post-implant., Methods: The ACTION VAD registry report was created from data submitted to the ACTION learning network from April 2018 to June 2023. It includes 1,430 devices implanted in 1,220 pediatric patients (≤18) from 57 sites across North America., Results: Males comprised 55% of the registry patients. The median age was 3.7 years with a median implant weight of 13.6 kg; 36% of the cohort was <10 kg. Nearly 40% of patients had a primary diagnosis of congenital heart disease (CHD). Patients with CHD represented 26% of VAD implants in 2018 which increased to 42% in 2023 (p=0.03). At implant, 25% of patients were supported with extracorporeal membrane oxygenation (ECMO), 4.9% with dialysis, and 54% were mechanically ventilated. Paracorporeal pulsatile pumps comprised 40.2% of implants, followed in incidence by paracorporeal continuous flow (28.5%), and implantable continuous flow (24.1%). The number of patients in the VAD Registry patients increased from 102 in 2018 to 256 in 2022, partly reflecting increased center participation in ACTION. Overall survival on support at 1 year was 79.2%, and the incidence of stroke was 13.7%. Infants demonstrated the poorest outcomes, with a 1-year survival of 72.9% and a higher incidence of stroke (20.8%)., Conclusion: The 5-year ACTION VAD experience highlights the growing collaboration in the pediatric VAD community and changes in clinical practice. More work is needed to improve survival and limit adverse outcomes, especially in younger patients., (Copyright © 2025 International Society for the Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

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: Ventricular assist device (VAD) support for failing Glenn circulation represents a unique challenge., Methods: We conducted a retrospective review of clinical outcomes in patients with VAD support for failing Glenn circulation between 2010 and 2020 at a tertiary pediatric institution., Results: Ten patients were included: INTERMACS profiles were 1 in 3 patients and 2 in 7 patients. The median age, weight, and body surface area were 3.2 years, 13.0 kg, and 0.5 m 2 , respectively. Seven patients (70%) were implanted with continuous-flow devices and 3 with para-corporeal devices. Nine patients (90%) received heart transplant, with a median support duration of 77 days. Four (67%) out of 6 patients supported with discharge-capable devices were managed as outpatients. Post-transplant survival was 100%, with a median (range) follow up duration of 3.5 (1.8-11.9) years. There were 3 neurologic complications in 3 patients (0.9 events per patient-year); 2 intraoperative events (fatal hypoxia and symptomatic embolic stroke) and 1 postoperative (asymptomatic subarachnoid hemorrhage). Pump thrombosis occurred in one patient (0.3 events per patient-year), requiring pump exchange at day 65. Five patients (50%) received concomitant Fontan completion (fenestrated in 1). The Fontan-upgraded patients (vs Glenn) tended to be larger (median (range): 15.9 (12.6-22.9) vs 9.1 (7.7-22.8) kg), older (4.7 (3.1-6.5) vs 1.1 (0.9-10.1) years) and had a higher PaO 2 /FiO 2 ratio (192 (52-336) vs 76 (59-78) mm Hg) on postoperative day 1., Conclusion: Our experience suggests the feasibility of durable VAD support for failing Glenn circulation. Concomitant Fontan completion may be considered in select patients to improve oxygen delivery., Competing Interests: Disclosure statement IA: Serves as a consultant/proctor for Berlin Heart, Inc., Abbot Inc., Abiomed, Inc., Medtronic, Inc., Jarvik Inc., BiVACOR Inc., (Copyright © 2022 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Durable ventricular assist devices (VADs) have significantly improved survival to transplantation among children with advanced stages of heart failure. The fundamental goals of VAD therapy include decreasing mortality, minimizing adverse events, and improving quality of life. As the pediatric VAD experience has evolved with reduced device related complications and improved survival, VAD therapy is being considered not only as a bridge to transplantation (BTT) but also as a bridge to decision (BTD) and as destination therapy (DT). Data regarding pediatric DT VAD are limited to anecdotal or case reports of children being supported for long periods with VADs and by default being classified as DT VAD. This article reviews current trends in the use of DT VAD and adverse events in children vs adults on VAD, and provides a framework for patient selection with the use of a multidisciplinary approach including palliative care. The general approach to determining DT VAD candidacy should include: 1) a reasonable success that the patient will survive the peri- and postoperative state; and 2) a high likelihood that the patient will be able to be discharged out of hospital and have adequate caregiver support. Patients with muscular dystrophy and failing Fontan physiology are examples of pediatric populations for whom DT VAD may be considered and which require unique considerations., (Copyright © 2019 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)