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BACKGROUND Challenges exist with heterotaxy due to the complexity of heart disease, abnormal venous connections, and infection risks. This study aims to understand heart transplant outcomes for children with heterotaxy. METHODS All children with congenital heart disease listed for transplant from 1993 to 2018 were included. Those with and without heterotaxy were compared. Waitlist outcomes and survival post-listing and transplant were analyzed. Post-transplant risk factors were identified using multiphase parametric hazard modeling. RESULTS There were 4814 children listed, of whom 196 (4%) had heterotaxy. Heterotaxy candidates were older (5.8 ± 5.7 vs 4.2 ± 5.5 years, p < 0.01), listed at a lower urgency status (29.8% vs 18.4%, p < 0.01), more commonly single ventricle physiology (71.3% vs 59.2%, p < 0.01), and less often supported by mechanical ventilation (22% vs 29.1%, p < 0.05) or extracorporeal membrane oxygenation (3.6% vs 7.5%, p < 0.05). There were no differences in waitlist outcomes of transplant, death, or removal. Overall, post-transplant survival was worse for children with heterotaxy: one-year survival 77.2% vs 85.1%, with and without heterotaxy, respectively. Heterotaxy was an independent predictor for early mortality in the earliest era (1993-2004), HR 2.09, CI 1.16-3.75, p = 0.014. When stratified by era, survival improved with time. Heterotaxy patients had a lower freedom from infection and from severe rejection, but no difference in vasculopathy or malignancy. CONCLUSIONS Mortality risk associated with heterotaxy is mitigated in the recent transplant era. Early referral may improve waitlist outcomes for heterotaxy patients who otherwise have a lower status at listing. Lower freedom from both infection and severe rejection after transplant in heterotaxy highlights the challenges of balancing immune suppression.

Summary Background ABO-incompatible heart transplantation increases donor availability in young children and is evolving into standard of care in children younger than 2 years. Previous smaller studies suggest similar outcomes to ABO-compatible heart transplantation, but persisting alterations of the immune system in ABO-incompatible recipients might increase the risk of some infections or benefit the graft owing to reduced HLA reactivity. We aimed to assess long-term outcomes in young children after they received ABO-incompatible or ABO-compatible heart transplantation. Methods In this multicentre, prospective cohort study, we analysed data from the Pediatric Heart Transplant Society registry to compare children who received ABO-incompatible or ABO-compatible heart transplantation before age 2 years between Jan 1, 1999, and June 30, 2018. Given significantly different clinical demographics between the two groups, we also matched each ABO-incompatible recipient to two ABO-compatible recipients using propensity score matching. We assessed patient and graft survival, coronary allograft vasculopathy, malignancy, acute rejection (any episode resulting in augmentation of immunosuppression), and infections (requiring intravenous antibiotic or antiviral therapy or life-threatening infections treated with oral therapy). Findings We included 2206 children who received a heart transplant before age 2 years, with 11 332·6 patient-years of cumulative observation time. Children who received an ABO-incompatible transplant (n=364) were younger and a larger proportion had congenital heart disease and ventilator and mechanical circulatory support than the ABO-compatible recipients (n=1842). After matching, only differences in blood group (more O in ABO-incompatible and more AB in ABO-compatible groups) and use of polyclonal induction therapy with anti-thymocyte globulins persisted. The two matched groups had similar post-transplantation graft survival (p=0·74), freedom from coronary allograft vasculopathy (p=0·75), and malignancy (p=0·51). ABO-incompatible recipients showed longer freedom from rejection (p=0·0021) in the overall cohort, but not after matching (p=0·48). Severe infections (p=0·0007), bacterial infections (p=0·0005), and infections with polysaccharide encapsulated bacteria (p=0·0005) that share immunological properties with blood group antigens occurred less frequently after ABO-incompatible heart transplantation. Interpretation ABO-incompatible heart transplantation for children younger than 2 years is a clinically safe approach, with similar survival and incidences of rejection, coronary allograft vasculopathy, and malignancy to ABO-compatible recipients, despite higher-risk pre-transplant profiles. ABO-incompatible transplantation was associated with less bacterial infection, particularly encapsulated bacteria, suggesting that the acquired immunological changes accompanying ABO tolerance might benefit rather than jeopardise transplanted children. Funding Pediatric Heart Transplant Society.

To describe the impact of infectious adverse events (IAEs) during ventricular assist device (VAD) support on graft loss, infection, and rejection after pediatric heart transplant (HT). Pedimacs data were linked to Pediatric Heart Transplant Society (PHTS) data for patients receiving a VAD followed by HT between September 2012 and December 2016. Linked patients were categorized into IAE on VAD (group A) and no IAE on VAD (group B). Infectious adverse event locations included nondevice, device (external or internal), and sepsis. Post-HT outcomes for analysis were graft loss, infection, and rejection. Time-dependent analysis included Kaplan-Meier and multiphase parametric hazard function analysis. We linked 207 patients (age 9.4 ± 6.3 years). Post-HT follow-up was 19.4 patient-months (8 days-4.1 years). Group A included 42 patients (20%) with 62 IAEs. Group B included 165 patients without an IAE. Group A patients were younger (7.4 ± 6.1 vs. 9.5 ± 6.3 years; p = 0.03), waited longer for HT (5.3 ± 4.1 vs. 2.9 ± 2.5 months; p = 0.0005), and were hospitalized longer post-HT (42 ± 59 vs. 23 ± 22 days; p = 0.05). VAD-related IAEs were rare (N = 11). Groups A and B had similar freedom from first post-HT infection, rejection, and graft loss (all p0.1). However, patients with VAD-related IAE were somewhat more likely to experience rejection (p = 0.03) and graft loss (p = 0.01). Children with an IAE on VAD who survive to HT are younger, wait longer for HT, and remain hospitalized longer than those without an IAE on VAD. Overall, IAE on VAD did not impact post-HT outcomes, but VAD-related IAE may be associated with graft loss and rejection.

While clinical status at the time of ventricular assist device (VAD) implant can negatively affect outcomes, it is unclear if early improvement after implant can have a positive effect. Therefore, the objectives of this study were to describe the clinical status of pediatric patients supported with a VAD and determine the impact of clinical status on the 1-month follow-up form on survival and ability to discharge. This was a retrospective analysis of data collected prospectively by the Pediatric Interagency Registry for Mechanical Circulatory Support Registry (Pedimacs) Registry. The Pedimacs database was queried for patients implanted between September 19, 2012, and September 30, 2019, who were alive on VAD support at 1-month postimplant on either a paracorporeal pulsatile or intracorporeal continuous device. Four factors on the 1-month follow-up were the focus of this study: mechanical ventilation, supplemental nutritional support, inotropic support, and ambulatory status. These factors were regarded as present if detected between 1-week and 1-month postimplant and were analyzed to determine their impact on survival following 1 month of VAD support and on successful discharge from hospital in patients with implantable continuous-flow devices. The eligible study cohort consisted of 414 patients with a mean age of 9.6 ± 6.2 years, weight of 40.8 ± 32.3 kg with the majority being male (56.7%) and having cardiomyopathy (68%). An isolated left ventricular assist device (LVAD) was the most common implant (85.5%). At implant, 40% were ventilated, 57% required nutritional support, 93% were on inotropes, and 58% were nonambulating. On the 1-month postimplant form, there were significant improvements in all four categories (14% ventilator support, 46% nutritional support, 53% on inotropes, and 25% nonambulating). However, there was no significant early change in the percentage of patients requiring supplemental nutrition in the paracorporeal pulsatile devices (88% vs. 82%; p = 0.2). Presence of these clinical parameters in early follow-up postimplant had a significant negative impact on survival and on the ability of patients with continuous-flow devices to be discharged. Presence of four specific clinical parameters early after VAD placement is associated with worse overall survival and an inability to discharge patients on VAD support. Ongoing work is needed for optimization of patients before implant and aggressive rehabilitation after implant to help improve long-term outcomes.

Introduction: The shortage of available donor organs means we must re-consider our current policies on donor selection. There is a broad variation in practice between centers as to the acceptable limit to ischaemia time (IT) in pediatric cardiac transplantation, with no recommendations in international guidelines. Hypothesis: We hypothesised that a prolonged ischaemia time was not associated with impaired post-transplant outcomes. Methods: Data from the Pediatric Heart Transplantation Society was analysed for all pediatric patients receiving heart transplants (Jan 1993 - June 2019). Transplants were separated into 5 categories depending on the IT (hours): < 2.4, 2.5-3.4, 3.5-4.4, 4.5-6.0, >6. Risk-adjusted outcomes were assessed by multivariable analysis, adjusting for donor, recipient, and peri-operative characteristics. Results: Data for 6,903 transplants were assessed (IT < 2.4 in 980, 2.5-3.4 in 2032, 3.5-4.4 in 2310, 4.5-6.0 in 1162, >6 in 281). In univariate analysis, increasing IT was associated with reduced 1-year survival (90.1%, 87.7% and 84.6%; p6 groups, respectively. Similar findings, of a deleterious association with prolonged IT, were observed for freedom from infection (73.0%, 66.8% and 56.4%; p 6 hours was associated with graft failure in cardiomyopathy (HR: 2.87, 95% CI: 1.34 - 6.15, p=0.007), but not congenital heart disease patients. Conclusions: In this large multi-centre cohort study, we found a deleterious association between prolonged IT and post-transplant outcomes. We recommend that an IT greater than 6 hours should be avoided in pediatric cardiac transplantation.

Background Infants with heart failure remain at significant risk for wait list mortality, despite mechanical circulatory support (MCS). It is unclear if the outcomes are influenced by modality of support or underlying diagnosis. We sought to compare the outcomes of infants Methods and Results Using the Pediatric Heart Transplant Society database, we evaluated survival following first MCS device in children P =0.2; 5.4% versus 6.4%; P =0.4). However, there was a significant difference in the use of VAD both at listing (8% versus 2.4%; P P P P =0.01) for the CHD or the cardiomyopathy group ( P =0.38), but patients with cardiomyopathy demonstrated better survival on both forms of support. Conclusions Survival for patients

Background The Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs), originally a National Institutes of Health–sponsored U.S. database, provides a platform to understand the population of children supported with ventricular assist devices (VADs) during this time of increasing numbers, new devices, expanding indications, and improved outcomes. Methods Between September 19, 2012, and December 31, 2019, 44 hospitals implanted 1031 devices in 856 patients under 19 years of age. Results Overall, diagnosis was cardiomyopathy in 497 (58%) patients, congenital heart disease (CHD) in 216 (25%), myocarditis in 85 (10%), and other in 58 (7%). Positive outcome (alive on device or bridge to transplantation and recovery) occurred in 82% at 6 months. The patient cohort for implantable continuous (IC) pumps (n = 365) (age 13.2 ± 3.9 years, 18% Interagency Registry for Mechanically Assisted Circulatory Support [Intermacs] profile 1, 23% intubated at implantation, 16% with CHD) was significantly different from the paracorporeal continuous (PC) pump cohort (n = 212) (age 3.6 ± 4.9 years, 46% Intermacs profile 1, 81% intubated, 42% CHD) and the paracorporeal pulsatile (PP) pump cohort (n = 230) (age 2.7 ± 3.5 years, 31% Intermacs profile 1, 76% intubated, 26% CHD). Consistent with their cohort composition, positive outcomes at 6 months based on device type were the following: IC, 92%; PC, 68%; and PP, 81%. The incidence of cerebrovascular accidents in the IC, PC, and PP cohorts was 7%, 14%, and 15%, respectively. Conclusions IC VADs, the most common VAD type placed in children, are associated with improved outcomes compared with PP and PC devices, though PP and PC devices are limited to supporting our most challenging patients. Noteworthy, the incidence of cerebrovascular accidents for pediatric VADs has significantly decreased and is now 11% overall. This report demonstrates again that although often attributed to age, size, or device type, much of the burden in mortality and adverse events is correlated to the patient’s overall state at VAD implantation.

Background: Limited data are available regarding the outcomes of patients supported by extracorporeal membrane oxygenation (ECMO) who undergo durable mechanical circulatory support implantation (dMCS). We analyzed the clinical characteristics, outcomes, and risk factors for mortality in patients who were bridged with ECMO to dMCS. Methods: Adult patients who received dMCS between January 2008 and December 2017 (n=19 824), registered in the Society of Thoracic Surgeons-Interagency Registry for Mechanical Assisted Circulatory Support (STS-INTERMACS) database were included. Baseline characteristics, outcomes, risk factors, and adverse events were compared between ECMO-supported patients (n=933) and INTERMACS profile 1 (IP-1) patients not supported by ECMO (n=2362). A propensity match analysis was performed. Results: ECMO patients had inferior survival at 12 months (66.1%) than non-ECMO patients (75.4%; P P =0.49). A multiphase parametric hazard model identified 2 different periods based on risk of death. ECMO patients had a high hazard for death in the first 6 months after implantation (hazard ratio, 2.18 [1.79–2.66]; P P Conclusions: ECMO-supported patients before dMCS have lower survival compared with other IP-1 patients. A multivariable analysis showed that ECMO is an independent risk factor of poor outcome after dMCS. However, a propensity matched analysis suggested that when important clinical variables are controlled the outcome of both groups is similar. These data support the implantation of dMCS in carefully selected ECMO patients.

Background Fontan physiology results in multiorgan dysfunction, most notably affecting the liver and kidney. We evaluated the utility of Model for End-Stage Liver Disease Excluding INR (MELD-XI) score, a score evaluating the function of both liver and kidney to identify Fontan patients at increased risk for morbidity and mortality post–heart transplant. Methods The Pediatric Heart Transplant Society database was queried to identify Fontan patients listed for heart transplant between January 2005 and December 2018. MELD-XI scores were calculated at listing and heart transplant. A multivariable analysis was conducted to identify risk factors for post–heart transplant mortality. Demographic, clinical characteristics, and survival differences were evaluated and compared between the high and low MELD-XI score cohorts. The impact of changing MELD-XI scores during the waitlist period on post–heart transplant outcomes was also evaluated. Results Of 565 Fontan patients who underwent transplantation, 524 (93%) had calculable MELD-XI scores at the time of heart transplant: 421 calculable at listing and 392 calculable at listing and at heart transplant. On multivariable analysis, only MELD-XI score (squared) (hazard ratio, 1.007), history of protein-losing enteropathy (hazard ratio, 2.1), and ventricular assist device use at transplant (hazard ratio, 3.4) were risk factors for early phase post–heart transplant mortality. Patients with high MELD-XI scores at heart transplant had inferior survival post–heart transplant (P = .02); those in the high MELD-XI score cohort at wait listing and heart transplant tend to have the worst post–heart transplant survival; however, this was not significant (P = .42). Conclusions The MELD-XI, an easily calculated score, serves as a valuable aid in identifying pediatric Fontan patients at increased risk for post–heart transplant mortality.

BACKGROUND We sought to better understand the patient population who receive a temporary total artificial heart (TAH) as bridge to transplant or as bridge to decision by evaluating data from the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) database. METHODS We examined data related to survival, adverse events, and competing outcomes from patients who received TAHs between June 2006 and April 2017 and used hazard function analysis to explore risk factors for mortality. RESULTS Data from 450 patients (87% men; mean age, 50 years) were available in the INTERMACS database. The 2 most common diagnoses were dilated cardiomyopathy (50%) and ischemic cardiomyopathy (20%). Risk factors for right heart failure were present in 82% of patients. Most patients were INTERMACS Profile 1 (43%) or 2 (37%) at implantation. There were 266 patients who eventually underwent transplantation, and 162 died. Overall 3-, 6-, and 12-month actuarial survival rates were 73%, 62%, and 53%, respectively. Risk factors for death included older age (p = 0.001), need for pre-implantation dialysis (p = 0.006), higher creatinine (p = 0.008) and lower albumin (p < 0.001) levels, and implantation at a low-volume center (≤10 TAHs; p < 0.001). Competing-outcomes analysis showed 71% of patients in high-volume centers were alive on the device or had undergone transplantation at 12 months after TAH implantation vs 57% in low-volume centers (p = 0.003). CONCLUSIONS Patients receiving TAHs have rapidly declining cardiac function and require prompt intervention. Experienced centers have better outcomes, likely related to patient selection, timing of implantation, patient care, and device management. Organized transfer of knowledge to low-volume centers could improve outcomes.

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 Socioeconomic disparities negatively impacts outcomes in heart transplantation (HTx). However, the impact of insurance status and the social construct of race in pediatric patients has not been well studied. Methods The Pediatric Heart Transplant Society registry was queried between January 1, 2000- December 31, 2019. Patients were stratified by insurance status: US government (USG), US private (USP) and Single Payer (SP, UK & Canada) and race (black, white, other). Patients with a selection of more than one race were classified as other. Outcomes after HTx were assessed by Kaplan Meier log rank test including graft survival, time to first cardiac allograft vasculopathy (CAV), rejection, hemodynamic compromised (HC) rejection, and malignancy. Results 5879 children underwent HTx with insurance status as follows: 2800 USG (48%), 2431 USP (41%) and 648 SP (11%). Race breakdown included: 4086 white, 1052 black and 936 other. At 10 years post-transplant, graft survival (65.8% USG, 72.6% USP, 77% SP, p= .0005), time to first CAV (73.5% USG, 77.7% USP, 94.6% SP, p Conclusion In this multinational analysis of pediatric HTx patients, USG was associated with worse post-HTx outcomes. When stratified by race, graft survival was worse in black patients with USG and USP insurance. Both insurance and the social construct of race contribute to worse outcomes post heart transplant and may represent disparity in access to care and other racial bias. These results highlight the importance of further work in this area.

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.

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.

Purpose The obesity epidemic has afflicted children of all ages with heart transplant (HT) recipients at significant risk compared to their pediatric counterpart. This study is the first large scale study to evaluate the prevalence of obesity in pediatric HT recipients and its effects on cardiac allograft vasculopathy (CAV) and graft survival. Methods We evaluated pediatric HT recipients (age 2 years) and weight/length z score >95%ile (WHO guidelines; age Results 4756 pediatric HT patients (pts) met study criteria (mean age at 1 year post-HT = 6.7 ± 6.2 yrs; 44.6% female; 69.5% White). At HT, 56% had a normal weight, 10% were overweight, 11% were obese and 23% were underweight. Overall, ∼60% had normal BMI that remained consistent up to 15 years post-HT (56-69%). For obese pts at HT, there was an increased risk for CAV development (HR 1.5, p = 0.002) and graft loss (HR 1.3, p = 0.001), see Figure. The risk of CAV development remained significant for obese pts at 5 years post-HT (p Conclusion Only 60% of pediatric HT recipients had ideal BMI at 1 year and 5 year follow up. Obesity at HT predicts an increased risk of CAV and graft loss. Exploring metabolic risk factors that lead to CAV development and graft loss are important. Healthy lifestyle interventions pre-HT and during HT follow up may potentially reduce morbidity and mortality.

Heart transplantation from ABO incompatible (ABOi) donors has evolved into a progressively accepted therapy in young children. We assessed the recent practice of ABOi listing impact on waitlist and post-transplant outcomes.Using the Pediatric Heart Transplant Society registry, we compared clinical presentation, waitlist parameters, and post-transplant survival of children2 years of age listed for ABOi vs ABO compatible (ABOc) heart transplant between January 2010 and June 2018 with sub-analysis of blood group O recipients.Among 2,039 patients, ABOi listing increased significantly with time from 49% (2010) to 72% (2017). ABOi-listed patients had lower age and body surface area, and higher proportion of congenital heart disease, mechanical ventilation, and high urgency status (all p0.01). Use of mechanical circulatory support was similar between groups. Of 1,288 patients reaching transplant, 239 (18.6%) received an ABOi organ (15%-40%/year). Death while waiting, removal from the waitlist, and waitlist survival were similar between groups. Time to transplant was significantly shorter for ABOi listing in blood group O patients (p0.02), approaching significance (p = 0.057) for all blood groups. Post-transplant survival was similar except for lower survival of patients listed ABOc but transplanted ABOi. These patients showed increasing need for mechanical circulatory support and high urgency listing while waiting.In the current era, primary listing for ABOi heart transplant has become routine for the majority of children2 years old, resulting in shorter waitlist time, especially in blood group O. Post-transplant survival is similar despite ABOi-listed children still showing a higher risk profile.

Purpose Ventricular assist device (VAD) use in patients with congenital heart disease continues to increase. However, the outcomes of supporting a Fontan circulation with a VAD are largely unknown. Methods This is a retrospective review of the Pedimacs and Intermacs databases. 55 patients underwent systemic VAD implant to support the Fontan circulation between September 2012 and December 2019. Results The mean age of patients at implantation was 12.2 ± 8.0 yrs, and mean weight, 38.1 ± 26.8 kgs. Patient demographics are presented in Table 1. 28 more VADs were implanted in 2018-2019 than in 2012-2017 (28 vs 27, P = 0.007, Figure 1). The later era had higher GFR (101.1 ± 48.5 vs 71.2 ± 34.9 P = 0.02) and were more likely being followed for heart failure prior to VAD (96% vs 74% P = 0.02), but there was no difference in INTERMACS profile (P = 0.69). Kaplan-Meier survival on device was 76% at 6 months with no difference by era. Competing outcomes demonstrated a positive outcome of 81% (alive on VAD, transplanted or recovered) at 6 months, with the majority of mortality occurring in month one. Mean length of support was 5.5 ± 8 mos. 5 patients were supported for >1 yr with no added mortality; the longest support time was 4 yrs 7mos. Adverse event rates included pump thrombosis incidence of 4% (3.3/100 pt-mo), GI bleeding of 7% (2.6/100 pt-mo), and non-GI bleeding of 9% (2.3/100 pt-mo). Freedom from stroke and infection were both 95% at 6 months. Conclusion This is the largest reported analysis of systemic VAD support of Fontan patients. Mechanical circulatory support of the Fontan circulation is increasing in prevalence and can be performed with promising outcomes.

Purpose Our study aims to identify the utility of pre-implant MELD-XI score in identifying pediatric patients at higher risk for morbidity and mortality post-VAD implant. Methods We reviewed all patients listed in the PediMACS database from 9/19/12 to 3/31/19. Patients missing bilirubin or creatinine that precluded calculation of MELD-XI score (n=89) were excluded. Patients were divided into three cohorts based on MELD-XI score pre-implant - low (minimum score 9.4, 13.6, 75-100%-tile). Baseline clinical and hemodynamic data were compared; outcomes data on morbidity and mortality were compared across groups. Results Of 596 patients included, 42.5% were in the low, 29.2% in the intermediate and 28.3% in the high MELD-XI cohort. Compared to the low and intermediate cohorts, patients in the high MELD-XI cohort were significantly older (p Conclusion Pediatric patients with an elevated MELD-XI score have higher acuity of illness pre-implant. This easily calculated score based on routinely collected lab values serves as a promising risk assessment tool in identifying pediatric VAD recipients at increased risk for mortality post VAD implant. Trending the MELD-XI score in patients with advanced heart failure may aid clinicians in timing of VAD implantation.

Dyslipidemia in the pediatric heart transplant (HT) population carries significant risk for developing cardiac allograft vasculopathy (CAV), one of the primary reasons for heart retransplantation (retx). This large-scale pediatric study sought to evaluate the prevalence of dyslipidemia and its

BACKGROUND The use of ventricular assist devices (VADs) in children with heart failure may be of particular benefit to those with accompanying renal failure, as improved renal function is seen in some, but not all recipients. We hypothesized that persistent renal dysfunction at 7 days and/or 1 month after VAD implantation would predict chronic kidney disease (CKD) 1 year after heart transplantation (HT). METHODS Linkage analysis of all VAD patients enrolled in both the PEDIMACS and PHTS registries between 2012 and 2016. Persistent acute kidney injury (P-AKI), defined as a serum creatinine ≥1.5× baseline, was assessed at post-implant day 7. Estimated glomerular filtration rate (eGFR) was determined at implant, 30 days thereafter, and 12 months post-HT. Pre-implant eGFR, eGFR normalization (to ≥90 mL/min/1.73 m2 ), and P-AKI were used to predict post-HT CKD (eGFR

BACKGROUND The reported ventricular assist device (VAD) experience in the pediatric congenital heart disease (CHD) population is limited. We sought to describe contemporary use and outcomes of VADs in children with CHD and compare these outcomes to those of non-CHD children. METHODS Patients enrolled in the Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) between September 19, 2012 through June 30, 2017 were included. CHD was classified as biventricular vs single ventricle (Stages 1, 2, or 3). Outcomes were compared between groups and multivariable analysis was used to identify factors associated with mortality on the device. RESULTS Among the 471 patients enrolled, 108 (24%) had CHD (45 biventricular and 63 single ventricle). CHD patients were younger (5.7 ± 5.7 years vs 9.8 ± 6.5 years; p < 0.0001) and smaller (0.8 ± 0.5 m2 vs 1.2 ± 0.7 m2; p < 0.0001) compared with non-CHD patients. CHD patients were more likely to receive a paracorporeal continuous-flow VAD (36.1% vs 12.9%; p < 0.0001) and less likely to receive an implantable continuous-flow VAD (27.8% vs 55.0%; p < 0.0001) compared with non-CHD patients. After 6 months on a VAD, CHD patients had higher mortality (36.4% vs 12.1%) and a lower transplantation rate (29.1% vs 59.9%) than non-CHD patients (p < 0.0001). In the multivariable analysis, CHD was the factor most strongly associated with mortality on VAD (hazard ratio [HR] = 2.9; p < 0.0001), whereas the factors implantable continuous-flow device and high-volume center were protective (HR = 0.3, p < 0.0001, and HR = 0.6, respectively; p = 0.02). CONCLUSIONS VAD use in children with CHD is associated with increased mortality and decreased transplant rates compared to children without CHD. For the subgroup of children with CHD who received implantable continuous-flow VADs, survival rates were higher and comparable to those of children without CHD. Increased experience correlated with better survival in pediatric VADs.

Purpose To analyze the clinical characteristics and outcomes of patients who were bridged with ECMO to durable mechanical circulatory support (dMCS). Methods Adult patients who received dMCS between Jan 2008 and Dec 2017, registered in INTERMACS were included. Baseline characteristics, outcomes, risk factors and adverse events were analyzed in patients supported by ECMO (ECMO group) prior to dMCS implantation, and compared with INTERMACS profile 1 patients not supported by ECMO (non-ECMO group). Results Patients bridged to dMCS on ECMO were younger, more likely to have acute heart failure, ischemic etiology, and a bridge to candidacy implantation compared to other INTERMACS profile 1 patients (Table 1). Patients who received dMCS on ECMO had 1, 6, and 12 month survival of 83.8%, 71.2%, and 66.1%, inferior to non-ECMO group patients (91.4% 81.7% and 75.4%, p Conclusion Patients bridged by ECMO to dMCS have lower survival compared with other INTERMACS profile 1 patients. Older age and longer duration of HF are associated with worse outcomes. ECMO implantation was independently associated with poorer outcome.

Purpose Extra-corporeal membrane oxygenation (ECMO) as bridge to ventricular assist device (EB-VAD) therapy is frequently used for initial resuscitation of critically ill pediatric patients (INTERMACS 1 & 2). Data regarding outcomes with this strategy is limited. Methods INTERMACS 1 & 2 patients enrolled in Pediatric Interagency Registry for Mechanical Circulatory Support (PEDIMACS) between September 19th, 2012 & December 31st, 2017 constituted the study group. Of 370 patients, 271 received primary VAD (P-VAD) implantation & EB-VAD was utilized in 99 patients. Patient characteristics, durable VAD outcomes & time to first adverse event with & without preoperative ECMO bridge were compared. Sub-analysis of INTERMACS 1 patients was conducted. Results Lower age (p=0.0001) & critical cardiogenic shock(p tube feeding , mechanical ventilation & paralytics (p Conclusion In INTERMACS 1 & 2 patients EB-VAD strategy is associated with poor early & late post-implant survival. Within INTERMACS 1 early survival is comparable with both strategies. EB-VAD could potentially benefit high risk INTERMACS 1 patients

Background Adult congenital heart disease (ACHD) prevalence is increasing worldwide, with advanced heart failure (HF) as a leading cause of death. Limited data are available on durable mechanical circulatory support (MCS) in ACHD patients. Methods ACHD patients from the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) database were identified and propensity matched with non-ACHD patients using risk factors from the INTERMACS Seventh Annual Report. We compared these groups for the primary outcome of post-MCS mortality. We also investigated adverse event rates, functional status, and health-related quality of life. Results ACHD (n = 128) and non-ACHD (n = 512) patients were appropriately matched by baseline characteristics. ACHD patients had a longer length of stay at MCS implant (24 vs 19 days, p = 0.006) but similar rates of post-MCS adverse events and hospitalization. There were similar improvements in functional status and health related quality of life post-MCS in both groups. ACHD patients had significantly higher mortality post-MCS exclusively during the first 5 months after implant (p = 0.003) and a lower probability of receiving a transplant (p = 0.003). Risk factors for early mortality were biventricular or total artificial heart device implant and age > 50 years. Conclusions ACHD patients experience a higher early mortality after MCS but have similar adverse event rates and similar improvements in functional capacity and quality of life compared with non-ACHD patients. These data support expansion of MCS use in selected ACHD patients.

Purpose Transplantation (Tx) of young children with ABO incompatible (ABOi) hearts has evolved from an experimental approach to a routine procedure in many centers. Prior studies have shown equal survival after ABOi and ABO compatible (ABOc)-Tx, but also revealed altered B-cell response and less HLA sensitization following ABOi-Tx with yet unknown clinical impact. Using a large registry cohort, we investigated outcomes of ABOi recipients in comparison to all and directly matched ABOc recipients. Methods Data from the Pediatric Heart Transplant Society was analyzed comparing children Results ABOi-Tx was performed in 337 (16%) of children Conclusion In this large cohort of young children receiving ABOi-Tx in a clinical routine setting rates of graft survival, CAV and malignancy were equivalent to matched ABOc recipients. ABOi-Tx was associated with longer time to rejection, suggesting better graft acceptance associated with acquired blood group tolerance. ABOi-Tx was also associated with longer time to severe infection, potentially secondary to less rejection treatment. The benefits of ABOi-Tx seen in the matched cohort analysis persisted when analyzed against all ABOc-Tx children.

Purpose As heart transplant (HT) outcomes in Fontan patients have improved, there is interest in identifying markers of liver disease associated with increased mortality. In adults, an elevated Model for End Stage Liver Disease eXcluding INR (MELD-XI) score is associated with worse post-HT outcomes. We evaluated the relationship between MELD-XI scores at transplant and post-HT morbidity and mortality in Fontan patients. Methods The Pediatric Heart Transplant Society Registry was queried to identify pediatric Fontan patients listed for HT from 2005-2017. The MELD-XI score was calculated [MELD-XI= 5.112 x ln(total bilirubin) + 11.76 x ln(creatinine) + 9.44] at time of transplant. Those patients with a MELD-XI score at the time of HT above the 75thpercentile of the entire cohort were classified as the high MELD-XI score cohort; All others formed the low MELD-XI score cohort. Results Of 698 Fontan patients listed, 506 (73%) underwent HT. A score ≥ 11.5 identified the high MELD-XI score cohort. At HT, patients in the high MELD-XI cohort were more likely to be older (12 ±5.0 vs. 10.8 ±4.8 y, p=0.05), listed UNOS Status 1A (82% vs. 72%, p=0.05), on a ventilator (11% vs. 3%, p=0.001) and on ECMO (9.1% vs. 2.6%, p=0.009). There was no difference in time to first infection, treated rejection, CAV or malignancy between groups. However, both allograft and patient survival (Figure) was inferior in the high MELD-XI score cohort (p=0.02 and p=0.01 respectively). Multiphase parametric hazard modeling demonstrated that high MELD-XI score (HR=1.2, p Conclusion Pediatric Fontan patients with high MELD-XI scores and those who require mechanical circulatory support have increased mortality post-HT. Identification of Fontan patients with high MELD-XI scores during patient selection or while awaiting HT allows for active management of this risk factor which may reduce post-HT mortality.