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Fontan palliation has improved survival for single ventricle patients, but long-term complications persist including cardiovascular dysfunction, neurohormonal abnormalities, and protein-losing enteropathy (PLE). Although chronic inflammation contributes to morbidity, an association between inflammation and vascular dysfunction has not been studied. We assessed inflammation and vascular function in 31 Fontan-palliated patients (52% male, median age 14.3 years), including 10 PLE+. Fontan circulation was associated with altered inflammatory cytokines (TNF-α: mean 2.5 ± 1.4 vs. 0.7 ± 0.2 pg/ml, p0.0001; sTNFR2: 371 ± 108 vs. 2694 ± 884 pg/ml, p0.0001) and vascular dysfunction [log-transformed reactive hyperemia index (lnRHI) 0.28 ± 0.19 vs. 0.47 ± 0.26, p0.01; augmentation index (AI) -2.9 ± 13.8 vs. -16.3 ± 12.0, p = 0.001; circulating endothelial progenitor cells (cEPCs) 5.0 ± 8.1 vs. 22.8 ± 15.9, p = 0.0002)]. Furthermore, PLE+ patients showed greater inflammation (IFN-γ 6.3 ± 2.2 vs. 11.5 ± 7.9 pg/ml, p = 0.01; sTNFR1: 1181 ± 420 vs. 771 ± 350 pg/ml, p = 0.01) and decreased arterial compliance (AI: 5.4 ± 17.1 vs. -6.8 ± 10.2, p = 0.02) than PLE- patients. Circulating EPCs, but not inflammatory cytokines, were inversely associated with arterial stiffness in Fontan patients. In conclusion, chronic inflammation and vascular dysfunction are observed after Fontan operation, with greater inflammation and arterial stiffness in Fontan patients with active PLE. However, there is no clear association between inflammatory cytokines and vascular dysfunction, suggesting these pathophysiologic processes are not mechanistically linked.

To determine the prevalence and influence of clinically significant airway and/or respiratory abnormalities in patients with trisomy 13 and 18 undergoing cardiac surgery.We performed a retrospective, case-control cohort study of all patients with known trisomy 13 or 18 who underwent cardiac operations at our institution from 1994 to 2014. Cases were matched 3:1 by age, surgical date, and cardiac lesion with nontrisomy 13/18 patients. Baseline clinical characteristics and patient outcomes, including postoperative course and management were compared. Descriptive statistics and Wilcoxon rank-sum test or Fisher exact test as appropriate were used to determine significant differences.In the 14 trisomy 13/18 patients who underwent cardiac surgery, there was an increased incidence of postoperative complications. Specifically, 93% had airway or pulmonary complications, including prolonged mechanical ventilation (n = 8), prolonged noninvasive positive pressure ventilation (n = 6), re-intubation (n = 7), tracheitis/pneumonia (n = 6), and tracheostomy (n = 2). The duration of intubation was longer (7.5 vs 2 days; P .0001) as was the duration of noninvasive positive pressure ventilation (8 vs 2 days; P .04) with longer hospital length of stay in the trisomy 13/18 cohort. There was 1 in-hospital mortality, with none in the control group.Although most trisomy 13/18 patients survive cardiac surgery, these patients have an increased incidence of airway complications, requiring longer intensive respiratory support postoperatively that contributes to longer length of stay. Parental guidance before cardiac surgery should include a discussion about postoperative airway management.

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.

Background As survival and neuromuscular function in Duchenne Muscular Dystrophy (DMD) improve with glucocorticoid therapy and respiratory advances, the proportion of cardiac deaths is increasing. Little is known about the use and outcomes of advanced heart failure (HF) therapies in this population. Methods A retrospective cohort study of 436 males with DMD was performed, from January 1, 2005-January 1, 2018, with the primary outcome being use of advanced HF therapies including: implantable cardioverter defibrillator (ICD), left ventricular assist device (LVAD), and heart transplantation (HTX). Results Nine subjects had an ICD placed, 2 of whom (22.2%) had appropriate shocks for ventricular tachycardia; 1 and 968 days after implant, and all of whom were alive at last follow-up; median 18 (IQR: 12.5–25.5) months from implant. Four subjects had a LVAD implanted with post-LVAD survival of 75% at 1 year; 2 remaining on support and 1 undergoing HTX. One subject was bridged to HTX with ICD and LVAD and was alive at last follow-up, 53 months after HTX. Conclusion Advanced HF therapies may be used effectively in select subjects with DMD. Further studies are needed to better understand risk stratification for ICD use and optimal candidacy for LVAD implantation and HTX, with hopes of improving cardiac outcomes.

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.

Infections are frequent in pediatric ventricular assist device (VAD) patients. In this study we aimed to describe infections in durable VAD patients reported to Pedimacs.Durable VAD data from the Pedimacs registry (September 19, 2012 to December 31, 2015) were analyzed. Infections were described with standard descriptive statistics, Kaplan-Meier analysis and competing outcomes analysis.There were 248 implants in 222 patients, with a mean age and a median follow-up of 11 ± 6.4 years and 2.4 patient-months (1 day to 2.6 years), respectively. Device types were pulsatile flow (PF) in 91 (41%) patients and continuous flow (CF) in 131 (59%) patients. PF patients were younger (4 ± 4 vs 14 ± 4 years; p0.0001) and were more likely to have congenital heart disease (25% vs 12%; p = 0.03), prior surgery (53% vs 26%; p0.0001) and prior extracorporeal membrane oxygenation (24% vs 7%; p = 0.0003). Infection accounted for 17% (96 of 564) of the reported adverse events (AEs). A non-device infection was most common (51%), followed by sepsis (24%), external pump component infection (20%) and internal pump component infection (5%). Most infections were bacterial (73%) and required intravenous therapy only (77%). The risk of infection in the constant phase was higher in patients with a history of prior infection and in patients with a history of a non-infectious major AEs. Survival was lower after infection only in CF patients (p = 0.008).Infection was the most common AE after pediatric VAD implantation. Non-device infections were most common. The best predictor of a future infection was a past infection. CF patients have higher risk of death after an infection.

Purpose PRES is an uncommon complication of organ transplantation. Little is known about its clinical course and risk factors. We sought to examine the natural history and risk factors for PRES in pediatric heart transplant (HTx) recipients. Methods 14-center international retrospective study of 46 pediatric PRES cases between 2000-2018, compared with 1372 HTx recipients without PRES between 2000-2018. Demographic and clinical data were entered into a central database. PRES diagnosis was based on a combination of seizures, headache, altered mental status or focal neurological deficits, and required confirmation, either by MRI or resolution of neurological symptoms after CNI withdrawal. Factors associated with PRES were identified by Cox Proportional Hazard multivariable analysis. Results Median onset of symptoms was 16 days (R, 2-2468 d) after HTx; 23 (50%) developed PRES in the first 2 weeks, 32 (70%) in the first month, and 40 (87%) within the first year after HTx. Symptoms at presentation included seizures (40/46, 87%), altered mental status (27/46, 59%), headache (18/46, 39%), focal neurological deficits (16/46, 35%), and lasted for a median of 4 days (R 1-1772d), with almost all recipients (44/46, 96%) making a full recovery. MRI was obtained in 43/46, showing a vasogenic pattern of edema characteristic of PRES in 37 recipients (80%). Recipients with PRES tended to be older (9.1 y vs 5.9 y, p=0.06). Hypertension (SBP>95%ile) was present in 40/44 (91%) recipients with PRES at presentation, with severe hypertension (SBP>99%ile plus 5mmHG) in 37 (85%). Though all cases occurred in the setting of CNI use (TAC 40, CSA 6), levels were supratherapeutic in only 13/42 (31%). Cox analysis revealed a 5.3x increased hazard for PRES with Glenn (CI 1.54,18.01; p=0.008) and a 3.8x increased hazard with Fontan (1.08,13.00; p=0.038), while age, underlying cardiac diagnosis, BMI, ischemic time and type of CNI did not appear to contribute significantly. Conclusion 2/3 of pediatric PRES cases occur in the first month after HTx with seizures as the most common presenting symptom. An overwhelming majority of patients were severely hypertensive, whereas less than a third had supratherapeutic CNI levels. Almost all recipients recovered fully within days. Recipients with pre-existing Glenn or Fontan have a higher rate of developing PRES after HTx.

Purpose Individuals born with defects resulting in a functionally univentricular heart Fontan-type palliation (FP) are prone to physiologic deterioration requiring advanced cardiac therapies. Given limitations to organ availability and long transplant waiting-list times, mechanical circulatory support (MCS) has become an important tool to permit individuals with a single ventricle circulation to either survive to transplant or may be used for destination therapy. The physiologic and clinical impact of MCS in FP patients are undefined. Methods Centers participating in the ACTION Learning Network entered data on FP patients that received a VAD at their center, between 1/26/12 and 10/1/2019, in the ACTION Outcomes registry. Additional survey data on physiologic variables will be completed by Nov 2019. Results Thirty-nine FP patients were reported to the ACTION registry. Limited clinical variables can be found in table 1. MCS types included intracorporeal continuous flow support (25 patients), paracorporeal support (10), total artificial heart (2) and biventricular assist devices (1). Among supported patients, 23 % (9) died while on support, 10 % (4) are currently alive on support, and 67% (26) were transplanted and are alive while none died post-transplant. Major adverse events included major bleeding (17 episodes), driveline infection (n=1) and other neurologic dysfunction (n=1). There was no stroke or pump thrombosis (table 1). MCS implant improved NYHA functional class (12.5% versus 75% NYHA class 1/2) and decreased severe atrioventricular valve regurgitation (47% versus 30%). Complete clinical and hemodynamic data will be available and presented at the ISHLT meeting. Conclusion This is the first multicenter report of FP outcomes post MCS. MCS results in improved functional status and decreased atrioventricular valve regurgitation in FP patients awaiting transplant without adverse post-transplant outcomes and with good on-device survival. MCS for Fontan patients as both destination and bridge to transplant is a reasonable option.

Purpose Ventricular assist devices (VADs) have improved wait list survival in children with end-stage heart failure. However, questions remain about the optimal timing of VAD implantation. In this cost-effectiveness analysis, we compared immediate intracorporeal continuous VAD implantation to a watchful waiting approach using continuous inotropic therapy. Methods Using a Markov model, we simulated a cohort of pediatric patients with dilated cardiomyopathy. We used a monthly cycle and 20-year time horizon. We estimated cost and benefits (in terms of quality-adjusted life years, QALYs) from a healthcare perspective discounted at 3% per year. Transition probabilities, costs, and utility weights were taken from published and grey literature, which we supplemented with expert opinion where there were gaps. We calculated the incremental cost-effectiveness ratio (ICER) and performed one-way and probabilistic sensitivity analyses (PSA) using 10,000 Monte Carlo simulations to understand the impact of parameter uncertainty in the main findings. Cost variables were assigned gamma distributions, while transition probabilities and utilities were assigned beta distributions. Results In a base case analysis, the incremental costs and benefits of immediate intracorporeal continuous VAD implantation compared to medical management was $221,269 (2017 US$) and 0.21 QALYs, respectively, resulting in an ICER of $1.04 million per QALY. One-way sensitivity analysis found that the discount rate, the probability of heart transplant among patients on VAD and medical management, and the cost of VAD implantations exerted the most influence on the ICER, though varying any of the parameters to their lowest and highest values did not bring the ICER close a $100,000 per QALY willingness-to-pay (WTP) threshold. The PSA showed a 3% chance of immediate VAD implantation to be cost-effective at a WTP of $500,000 per QALY and a 36% chance at $1 million per QALY. Conclusion Though VADs provide a net health benefit as a bridge to transplant, immediate implantation in children with inotrope-dependent heart failure is not likely to be a cost-effective strategy compared to medical management based on commonly used ICER thresholds. Future analyses should explore the cost-effectiveness of VAD implantation in children with other disease states, diagnoses and device types.

Duchenne muscular dystrophy (DMD) is characterized by myocardial fibrosis and left ventricular (LV) dysfunction. Implantable cardioverter defibrillator (ICD) use has not been characterized in this population but is considered for symptomatic patients with severe LV dysfunction (SLVD) receiving guideline-directed medical therapy (GDMT). We evaluated ICD utilization and efficacy in patients with DMD. Retrospective cohort study of DMD patients from 17 centers across North America between January 2, 2005 and December 31, 2015. ICD use and its effect on survival were evaluated in patients with SLVD defined as ejection fraction (EF)

Objective To characterize the medical history, disease progression, and treatment of current-era patients with the rare diseases Fontan-associated protein-losing enteropathy (PLE) and plastic bronchitis. Study design A novel survey that queried demographics, medical details, and treatment information was piloted and placed online via a Facebook portal, allowing social media to power the study. Participation regardless of PLE or plastic bronchitis diagnosis was allowed. Case control analyses compared patients with PLE and plastic bronchitis with uncomplicated control patients receiving the Fontan procedure. Results The survey was completed by 671 subjects, including 76 with PLE, 46 with plastic bronchitis, and 7 with both. Median PLE diagnosis was 2.5 years post-Fontan. Hospitalization for PLE occurred in 71% with 41% hospitalized ≥3 times. Therapy varied significantly. Patients with PLE more commonly had hypoplastic left ventricle (62% vs 44% control; OR 2.81, 95% CI 1.43-5.53), chylothorax (66% vs 41%; OR 2.96, CI 1.65-5.31), and cardiothoracic surgery in addition to staged palliation (17% vs 5%; OR 4.27, CI 1.63-11.20). Median plastic bronchitis diagnosis was 2 years post-Fontan. Hospitalization for plastic bronchitis occurred in 91% with 61% hospitalized ≥3 times. Therapy was very diverse. Patients with plastic bronchitis more commonly had chylothorax at any surgery (72% vs 51%; OR 2.47, CI 1.20-5.08) and seasonal allergies (52% vs 36%; OR 1.98, CI 1.01-3.89). Conclusions Patient-specific factors are associated with diagnoses of PLE or plastic bronchitis. Treatment strategies are diverse without clear patterns. These results provide a foundation upon which to design future therapeutic studies and identify a clear need for forming consensus approaches to treatment.

ObjectiveIncreased blood lactate levels reflect tissue oxygen debt and might be indicative of low cardiac output. We hypothesized that the rate of increase in serum lactate would be an ideal marker to discriminate between infants at high and low risk of a poor outcome after surgical repair of congenital heart disease using cardiopulmonary bypass.MethodsIn the present prospective, observational study in a pediatric cardiac intensive care unit, infants (aged

Although nutritional status affects survival after heart transplant (HTx) in adults and older children, its effect on outcomes in young children is unknown. This study aimed to assess the effect of pre-HTx nutrition on outcomes in this population.Children aged 0 to 2 years old listed for HTx from 1997 to 2011 were identified from the Organ Procurement and Transplantation Network database. Nutritional status was classified according to percentage of ideal body weight at listing and at HTx. Logistic regression analysis evaluated the risk of waitlist mortality. Cox proportional hazard models assessed the effect of nutrition on post-HTx survival.Of 1,653 children evaluated, 899 (54%) had normal nutrition at listing, 445 (27%) were mildly wasted, 203 (12%) were moderate or severely wasted, and 106 (6%) had an elevated weight-to-height (W:H) ratio. Moderate or severe wasting (adjusted odds ratio, 1.9; 95% confidence interval, 1.3-2.7) and elevated W:H (adjusted odds ratio, 1.7; 95% confidence interval, 1.1-2.6) were independent risk factors for waitlist mortality. HTx was performed in 1,167 patients, and 1,016 (87%) survived to 1-year post-HTx. Nutritional status at listing or at HTx was not associated with increased post-HTx mortality. Nutritional status did not affect the need for early reoperation, dialysis, or the incidences of infection, stroke, or rejection before hospital discharge.Moderate or severe wasting and an elevated W:H are independent risk factors for waitlist mortality in patients aged2 years but do not affect post-HTx mortality. Optimization of pre-HTx nutritional status constitutes a strategy to reduce waitlist mortality in this age range.

At 6 years of age, patients with hypoplastic left heart syndrome had mean age-adjusted z -scores for weight and height below the normative population, and body mass index was similar to the normative population. Males had the greatest increase in z -scores for body mass index. Trial registration ClinicalTrials.gov: NCT00115934.

We report the use of an absorbable pulmonary artery band to limit postoperative cyanosis due to severe pulmonary arteriovenous fistulae in a 5-year-old girl with post-Fontan palliation takedown to a two-ventricle repair. At 1-year postoperatively, her fistulae had nearly resolved with no distortion of her pulmonary artery anatomy. This case demonstrates a novel approach to limiting cyanosis while pulmonary arteriovenous fistulae resolved after redirection of hepatic blood flow to the affected lung.

Growth faltering is common in children with hypoplastic left heart syndrome (HLHS) after the Norwood operation. We previously demonstrated that transplant-free survivors remained small at 3 years of age with mean weight-for-age z-scores (WAZ) and height-for-age z-scores (HAZ) below average. We

In the Single Ventricle Reconstruction trial, infants with hypoplastic left heart syndrome (HLHS) who received a right-ventricle-to-pulmonary-artery shunt (RVPAS) versus a modified Blalock-Taussig shunt (MBTS) had lower early postoperative mortality, but more complications at 14 months. We explored the effect of shunt type and other patient, medical, and surgical factors on postoperative length of stay (LOS) after the Fontan operation.Fontan postoperative course was ascertained from medical record review. Cox proportional hazards modeling was used to identify factors associated with LOS.Of 327 subjects who underwent Fontan, 323 were analyzed (1 death, 1 biventricular repair, 2 with missing data). Median age and weight at Fontan were 2.8 years (interquartile range [IQR]: 2.3, 3.4) and 12.7 kg (IQR: 11.4, 14.1), respectively. Fontan type was extracardiac in 55% and lateral tunnel in 45%; 87% were fenestrated. The RVPAS and MBTS subjects had similar LOS (median 11 days [IQR: 9, 18] vs 10 days [IQR: 9, 13]; P = .23). Independent risk factors for longer LOS were treatment center (P.01), LOS at stage II (hazard ratio [HR] 1.02 for each additional day; P.01), and pre-Fontan complications (HR 1.03 for each additional complication; P = .04). Use of deep hypothermic circulatory arrest at Fontan (HR 0.64; P = .02) was independently associated with shorter LOS. When center was excluded from the model, pre-Fontan complications and use of circulatory arrest were no longer significant; instead, older age at stage II (HR 1.08 for each additional month; P = .01) predicted longer LOS. In 254 subjects who had a pre-Fontan echocardiogram, at least moderate tricuspid regurgitation was independently associated with longer LOS, both with center (HR 1.72; P.01) and without center in the model (HR 1.49; P = .02).In this multicenter prospective cohort of subjects with HLHS, Norwood shunt type was not associated with Fontan LOS. Rather, global measures of earlier medical complexity indicate greater likelihood of longer LOS after the Fontan operation.

Infants undergoing repair of congenital heart disease (CHD) are at risk for post-operative acute kidney injury (AKI), but the incidence and outcome of AKI in this population is unknown. Furthermore, it is unknown whether AKI can be predicted from pre- and post-operative data. This study aimed to

Many patient and procedural characteristics have been investigated for their impact on outcomes after infant heart surgery, but risk-stratification models incorporating early post-operative biophysical markers are lacking. This study aimed to identify independent risk factors for poor outcome and to

Purpose Repeat heart transplantation (re-HTx) is standard practice in many pediatric centers, with acceptable outcomes in appropriate patients (pts). There are little data available on outcomes of 3 r4 HTx following failure of a 2 144 graft. We sought to compare outcomes of 3 r4 HTx in pediatric and young adult pts to outcomes of comparable pts with 2 144 HTx. Methods and Materials All recipients of 3 r4 HTx in whom primary HTx was prior to age 21 were identified in the United Network of Organ Sharing (UNOS) database (1985-2011) and matched 3:1 with a control group of 2 144 HTx pts by age, era, and re-HTx indication. The primary outcome measure was 1 and 5 year (yr) survival; secondary outcomes included inter-group differences in rejection and cardiac allograft vasculopathy (CAV). Results At time of HTx, cases (n=27) and controls (n=79) were alike in sensitization (PRA > 10 in 29.6% vs 29.1%, p= 0.94), serum creatinine (median 1.2 vs 1.1, p=0.30), bilirubin (median 0.8 vs 0.6, p=0.79), hospitalization (33.3% vs 53.2%, p=0.08), inotropic support (25.9% vs 41.8%, p=0.14) and mechanical ventilation (7.4% vs 7.6%, p=1.0). Inter-HTx interval was shorter for cases than controls (median 5.0 yr vs 9.8 yr, p=0.001). There was no difference in survival between groups at 1 and 5 yrs (76% vs 80% at 1 yr and 62% vs 58% at 5 yrs, p=0.75). The prevalence of rejection within the first yr (33.3% vs 44.3%, p=0.32) and post-HTx CAV (14.8% vs 30.4%, p=0.11) were comparable between groups. Risk factors for non-survival in 3 r4 HTx pts were: mechanical ventilation at HTx (Hazard ratio (HR) 8.4, 95% confidence interval (CI) 1.5-46.5); ECMO support at HTx (HR 25.5, 95% CI 1.6-408); and elevated serum bilirubin at HTx (HR 2.3, 95% CI 1.2-4.2). Conclusions Third HTx remains rare in pediatric and young adult pts. Outcomes of 3 r4 HTx are similar to 2 144 HTx in matched pts, with no difference in short or long term survival and comparable rates of early rejection and CAV. Consideration of 3 r4 HTx appears reasonable in appropriately selected pts.

Purpose Nutritional status has been shown to affect survival after heart transplant (HTx) in adults and older children. The impact of nutritional status on survival to HTx and HTx outcomes in children Methods and Materials Children 0-2 years listed for HTx from 1997-2011 were identified from the Organ Procurement and Transplantation Network database. Weight-for-height z-scores and percent ideal body weight (%IBW) were calculated at listing and at transplant. Nutritional status was categorized using the Waterlow criteria based on %IBW. The impact of nutritional status on survival to HTx was evaluated using logistic regression analysis and 1-year post-HTx survival was analyzed using Cox proportional hazard models. Results 1,653 children were included. At listing, 899 (55%) were categorized as normal nutrition status, 445 (27%) mildly wasted, 203 (12%) moderate or severely wasted, and 106 (6%) with elevated weight-to-height (W:H) ratio. Moderate or severe wasting (Adjusted Odds Ratio [AOR] 1.9; 95% Confidence Interval [CI] 1.3, 2.7) and elevated W:H ratio (AOR 1.6; CI 1.1, 2.6) were independent risk factors for wait-list mortality. Among 1,167 patients who underwent HTx, 87% (n=1,016) survived 1-year post-HTx. Pre-transplant nutritional status was not significantly associated with the need for early reoperation, dialysis, incidence of infection, stroke, rejection prior to hospital discharge, or 1-year mortality. Conclusions Moderate or severe wasting and elevated W:H ratio by Waterlow criteria are independent risk factors for wait-list mortality in patients

Predicting 1-Year Survival in Pediatric Heart Transplant Candidates with Congenital Heart Disease: An Analysis of the PHTS Database K.R. Schumacher, D. Naftel, M. Tresler, R. Kirk, C. Almond, T.P. Singh, R. Spicer, T.M. Hoffman, D. Hsu, C. Canter, M. Zamberlan, R.J. Gajarski. Congenital Heart Center, University of Michigan, Ann Arbor, MI; University of Alabama, Birmingham, AL; Freeman Hospital, Newcastle Upon Tyne, United Kingdom; Boston Children’s Hospital, Boston, MA; Cincinnati Children’s Hospital, Cincinnati, OH; Nationwide Children’s Hospital, Columbus, OH; The Children’s Hospital at Montefiore, New York, NY; St. Louis Children’s Hospital, St. Louis, MO.