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Introduction: Children with dilated cardiomyopathy (DCM) are increasingly bridged to heart transplant (Tx) with intracorporeal continuous flow ventricular assist devices (VADs), but there continues to be variability in hospital discharge. We sought to assess the impact of a quality improvement (QI) project promoting hospital discharge in pediatric VAD patients with DCM. Methods: Patients enrolled at sites participating in the Discharge and De-Escalation QI project of the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry were eligible. Patients with a HeartWare (HVAD) or HeartMate3 (HM3) VAD, implanted between April 2018 and February 2021, were included. Patient characteristics and outcomes were compared before and after the QI initiative, which targeted team and family expectations, was implemented in November 2019. Results: There were 85 patients (75%) with DCM and/or myocarditis who were included in this study (median age 14 years; median weight 57.1 kg; 67% male). Among 57 patients in the baseline group, 56% (n=32) were discharged with a mean post-implant hospital length of stay (LOS) of 36 days and 44% (n=25) underwent Tx during index VAD admission, with a mean of 52 days on device. There were 28 patients in the intervention group; 64% (n=18) were discharged with a mean LOS of 45 days, while 29% (n=8) patients underwent Tx during index VAD admission, with a mean of 44 hospital days on device. There was no significant difference in the frequency of hospital discharge or in hospital LOS between groups. Conclusions: Pediatric centers discharged just over half of DCM patients supported on an HVAD or HM3, with nearly one third of patients undergoing transplant during the index VAD admission. The QI intervention did not significantly alter the discharge rate. Further work is required to understand practice variation between centers with respect to Tx listing and organ acceptance for children on intracorporeal continuous flow VADs.

Introduction: Discharge of pediatric ventricular assist device (VAD) patients is lagging behind adults, especially pediatric patients with concurrent congenital heart disease (CHD). This quality improvement project aimed to increase discharge rates and decrease time to discharge for pediatric patients with CHD on VAD support. Methods: Patients with CHD implanted with HeartWare HVAD or HeartMate 3 at participating Advanced Cardiac Therapies Improving Outcomes Network (ACTION) centers were included. Baseline demographics and outcomes including explant, transplant, or death, and time to outcome was collected from April 2018 until October 2019. QI interventions (VAD Flight Plan and Discharge Journey Map) were initiated in November 2019. Percentage discharged on VAD as well as time to outcome were compared before and after intervention. Results: Twenty-six patients with CHD (18 single ventricle physiology) were identified (14 pre-intervention and 12-post). The mean age was 16 years old with 27% implanted as Intermacs profile 1. Mortality on device was high at 38%. There were 77% (n=20) considered discharge eligible (alive on device > 30 days) while 42% (n=11) achieved discharge (Table 1). Mean length of stay post-implant for discharged patients was 33 days. A higher percentage of biventricular physiology were discharged (63%) compared to univentricular (33%). QI interventions did not change discharge rate (43% vs 42%), mean time to discharge (46.3 vs 43.4 days), transplant before discharge (17% vs 21%), or death before discharge (42% vs 43%) in the post-intervention group. Conclusion: Discharging CHD patients on VAD support from the hospital remains a challenge largely due to the complex and heterogenous nature of this population at the time of implant. Interventions to address optimal timing of VAD implantation and patient selection could impact outcomes.

Introduction and Background: Arrhythmogenic ventricular cardiomyopathy (AVC) is a hereditable disorder characterized by fibro-fatty infiltration of the right ventricular myocardial wall. The purpose of this study was to describe the clinicopathologic phenotype of AVC in pediatric end-stage heart failure patients who underwent heart transplantation (HTx). Hypothesis: We hypothesized that AVC is misclassified in the young population who require heart transplant. Methods: We investigated 371 consecutive cases of primary pediatric (≤21 years) heart transplantation performed at Texas Children’s hospital between 1989 and 2018. Heart re-transplants were excluded from the study. Explanted hearts and tissue blocks were examined by a cardiac pathologist after HTx. Histological Diagnosis of AVC was based on the presence of major and minor diagnostic criteria according to the 2010 Revised Task Force Criteria. Results: Over half of the patients who underwent HTx had cardiomyopathy (212/371=57%) as the underlying primary diagnosis. After comprehensive histological evaluation of explanted hearts, 8 cases (8/212=3.8%) were diagnosed with AVC. Predominantly right ventricular disease was seen in 2/8 (25%), and biventricular involvement in 6/8 (75%) patients on pathological examination. Six out of 8 patients (6/8=75%) were misclassified, 4 as dilated cardiomyopathy, 1 as viral myocarditis, and 1 as restrictive cardiomyopathy. The median age at heart transplant (AVC cohort) was 11 years. Four of 8 (50%) were males. Six of 8 (75%) had a significant ventricular arrhythmia burden manifesting as non-sustained ventricular tachycardia requiring antiarrhythmic therapy. Genetic testing was undertaken in 3 of 8 (37.5%); all three were found to have pathogenic mutations in the PKP2 gene. Conclusion: Arrhythmogenic ventricular cardiomyopathy is often misclassified in the young population who require heart transplant. Pediatric dilated and restrictive cardiomyopathy phenotypes with end-stage heart failure waitlisted for a heart transplant (HT) who have a significant ventricular arrhythmia burden should be investigated for AVC.