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Objective Distraction can reduce pain and distress associated with painful procedures but has never been studied in children with solid organ transplants. We aimed to determine whether there is a difference in pain and distress associated with venipuncture in pediatric posttransplant patients who receive distraction compared with those who do not. Methods Randomized controlled trial of children aged 4 to 17 years with solid organ transplants undergoing venipuncture in the outpatient setting. Patients were randomized to receive distraction or no distraction. The primary outcome was the Faces Pain Scale-Revised. Secondary outcomes were the Observational Scale of Behavioral Distress-Revised; Faces, Leg, Activity, Cry, Consolability; and Children's Hospital of Eastern Ontario Pain Scale. Exploratory outcomes included the number of venipuncture attempts, time to successful venipuncture, and satisfaction of phlebotomists and parents. Results Median age of the 40 children enrolled was 11.5 years. Type of transplants included the heart (67.5%), kidney (22.5%), liver (7.5%), and more than 1 organ (2.5%). There was no difference between the Faces Pain Scale-Revised scores in distraction and no distraction groups (1.4; 95% confidence interval, 0.9-1.9; and 1.3, 95% confidence interval, 0.5-2.1, respectively). There was also no difference in the Observational Scale of Behavioral Distress-Revised; Faces, Leg, Activity, Cry, Consolability; and Children's Hospital of Eastern Ontario Pain Scale scores, number of venipuncture attempts, or time to successful venipuncture. Phlebotomists were more satisfied with the venipuncture when distraction was implemented. Conclusions In children with solid organ transplants, there was no difference in pain and distress associated with venipuncture between those who did and did not receive distraction. There was also no difference in other procedure-related outcomes except for greater phlebotomist satisfaction when distraction was implemented.

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.

Management of infants with pulmonary atresia/intact ventricular septum (PA/IVS) is variable. Because of higher mortality in more severe forms, heart transplant (HT) is an acceptable approach, but waitlist and post-transplant outcomes are unclear. This study compared outcomes of infants with PA/IVS vs. other single ventricle (SV) anatomies listed for HT.Data from the Pediatric Heart Transplant Society (1993-2018) were analyzed for survival and risk factors for mortality.Of 1617 SV infants, 300 had PA/IVS (19%) and 1317 had other SV (81%). Overall, 1-, 5-, and 10-year survival was higher among PA/IVS (74%, 65%, 61%) versus other SV infants (62%, 54%, 50%, p = .004). While waitlist mortality was similar between groups (p = .09), PA/IVS was an independent predictor of improved waitlist survival (HR 0.68, p = .03), and PA/IVS infants had higher incidence of waitlist removal (8% vs. 5.5%, p = .03), most commonly for being "too well." Post-transplant survival was superior among PA/IVS versus other SV infants (1- and 5-year survival 93% and 81% vs. 80% and 71%, p .0001). Risk factors for PA/IVS waitlist mortality (2008-2018) included extracorporeal membrane oxygenation and mechanical ventilation. Prior aortopulmonary (AP) shunt among PA/IVS infants was associated with improved waitlist survival.Overall survival among PA/IVS infants listed for HT exceeds that of other SV infants with PA/IVS identified as an independent predictor of improved waitlist and post-transplant survival. Prior AP shunt among listed PA/IVS infants was associated with improved waitlist outcomes, though, which may reflect a listing selection bias.

Utilization of ventricular assist devices (VADs) in adult populations with severe heart failure as a bridge to transplant has become the standard of care over the past two decades. Analogously, the use of VADs in pediatric populations has become more commonplace as pediatric heart transplantation has become more prevalent. We still have much to learn, however, about the complications after VAD placement in pediatric patients, their impact on transplantation and, in particular, how outcomes have changed over time. The objectives of this study were to (a) review the experience of a single pediatric VAD center, (b) identify risk factors that could lead to poor outcomes in patients on the transplant waitlist after VAD implantation and (c) demonstrate changes in outcomes over time. A retrospective cohort analysis was performed comparing death as a primary outcome and stroke and acute kidney injury (AKI) as secondary outcomes, across the study period divided into three timed eras. We analyzed 88 patients supported by a VAD over a 24-year timeframe. The duration, age at implant and indication for VAD support did not change significantly across the eras. We found that the incidence of stroke decreased over the study period and, while the rates of AKI did not change over the study period, those who developed AKI, while supported on VAD, had an increased risk of death.

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.

Surgical repair for patients with congenital heart disease (CHD) often incorporates homograft tissue or other foreign material that can lead to allosensitization. We sought to identify the relationship between pre-sensitization prior to heart transplant and exposure to homograft tissue in CHD patients.Retrospective chart review of all CHD patients who underwent heart transplant at a major pediatric transplant center between 1/1/2011-3/31/18. Operative records determined use of homograft tissue or foreign material. Panel reactive antibody (PRA) and LuminexFifty-six CHD patients underwent transplant during the review period. Thirteen patients (23%) were pre-sensitized by PRA10%. By SAB testing, 33 patients (59%) developed any anti-HLA antibody0 MFI, 30 patients (54%)2000 MFI, and 19 patients (34%)6000 MFI. Patients with homografts were more likely to be pre-sensitized by PRA (OR = 7.31, p = .007), and to have developed any anti-HLA antibody at various levels,0 (OR = 4.52, p = .034),2000 (OR = 8.59, p = .003), and6000 (OR = 8.50, p = .004). Of patients with homografts, those pre-sensitized by PRA had longer exposure times (9.80 vs 4.96 years, p = .025). There was no difference in exposure time with relation to pre-sensitization by SAB testing.Previous exposure to homograft tissue appears to increase the odds of pre-sensitization by either the PRA or SAB testing. Longer exposure time to homograft tissue prior to transplant is associated with increased pre-sensitization at transplant as determined by PRA, though not by SAB testing.

Immunosuppression strategies have changed over time in pediatric heart transplantation. Thus, comorbidity profiles may have evolved. Clinical Trials in Organ Transplantation in Children-04 is a multicenter, prospective, cohort study assessing the impact of pre-transplant sensitization on outcomes after pediatric heart transplantation. This sub-study reports 1-year outcomes among recipients without pre-transplant donor-specific antibodies (DSAs).We recruited consecutive candidates (21 years) at 8 centers. Sensitization status was determined by a core laboratory. Immunosuppression was standardized as follows: Thymoglobulin induction with tacrolimus and/or mycophenolate mofetil maintenance. Steroids were not used beyond 1 week. Rejection surveillance was by serial biopsy.There were 240 transplants. Subjects for this sub-study (n = 186) were non-sensitized (n = 108) or had no DSAs (n = 78). Median age was 6 years, 48.4% were male, and 38.2% had congenital heart disease. Patient survival was 94.5% (95% confidence interval, 90.1-97.0%). Freedom from any type of rejection was 67.5%. Risk factors for rejection were older age at transplant and presence of non-DSAs pre-transplant. Freedom from infection requiring hospitalization/intravenous anti-microbials was 75.4%. Freedom from rehospitalization was 40.3%. New-onset diabetes mellitus and post-transplant lymphoproliferative disorder (PTLD) occurred in 1.6% and 1.1% of subjects, respectively. There was no decline in renal function over the first year. Corticosteroids were used in 14.5% at 1 year.Pediatric heart transplantation recipients without DSAs at transplant and managed with a steroid avoidance regimen have excellent short-term survival and a low risk of first-year diabetes mellitus and PTLD. Rehospitalization remains common. These contemporary observations allow for improved caregiver and/or patient counseling and provide the necessary outcomes data to help design future randomized controlled trials.

Distraction can reduce pain and distress associated with painful procedures but has never been studied in children with solid organ transplants. We aimed to determine whether there is a difference in pain and distress associated with venipuncture in pediatric posttransplant patients who receive distraction compared with those who do not.Randomized controlled trial of children aged 4 to 17 years with solid organ transplants undergoing venipuncture in the outpatient setting. Patients were randomized to receive distraction or no distraction. The primary outcome was the Faces Pain Scale-Revised. Secondary outcomes were the Observational Scale of Behavioral Distress-Revised; Faces, Leg, Activity, Cry, Consolability; and Children's Hospital of Eastern Ontario Pain Scale. Exploratory outcomes included the number of venipuncture attempts, time to successful venipuncture, and satisfaction of phlebotomists and parents.Median age of the 40 children enrolled was 11.5 years. Type of transplants included the heart (67.5%), kidney (22.5%), liver (7.5%), and more than 1 organ (2.5%). There was no difference between the Faces Pain Scale-Revised scores in distraction and no distraction groups (1.4; 95% confidence interval, 0.9-1.9; and 1.3, 95% confidence interval, 0.5-2.1, respectively). There was also no difference in the Observational Scale of Behavioral Distress-Revised; Faces, Leg, Activity, Cry, Consolability; and Children's Hospital of Eastern Ontario Pain Scale scores, number of venipuncture attempts, or time to successful venipuncture. Phlebotomists were more satisfied with the venipuncture when distraction was implemented.In children with solid organ transplants, there was no difference in pain and distress associated with venipuncture between those who did and did not receive distraction. There was also no difference in other procedure-related outcomes except for greater phlebotomist satisfaction when distraction was implemented.

We sought to evaluate the association between timing of cardiac allograft vasculopathy (CAV) occurrence post-heart transplant (HT) with graft survival and progression of CAV severity in pediatric HT recipients.Data from the Pediatric Heart Transplant Society for pediatric (18 years old) HT recipients between 1993-2019 with available angiographic data were obtained (N = 5,075). The timing of CAV diagnosis (3; 3-5; 5-10; and ≥10 years post-HT) and severity of disease at each assessment (CAV 1-3) was determined. Associations between CAV timing, graft survival, and CAV progression were evaluated using Kaplan-Meier survival curves, multivariable COX proportional hazard regression analyses, and competing risk analyses.Over a median follow-up period of 4.1 (IQR 1.3-8.3) years, CAV was identified in 17% (885/5,075), 28% (252/885) of which were early-onset CAV. Compared with late onset CAV ≥10 years post-HT, patients with early CAV were older at the time of transplant (8.3 ± 6.2 vs. 3.8 ± 4.8 years, p.0001). While the five-year graft-survival in the ≥10-year group (79.2%, p = 0.03) was significantly higher than the3, 3-5, and 5-10 years post-HT groups (65.0%-67%) (p = 0.03), overall post-CAV graft survival was not significantly different across the CAV time-points. CAV disease progression did not vary with CAV timing post-HT, with an overall five-year freedom from CAV ≥2 of 75.4% (73.1%-77.6%).Later onset CAV (≥10-years post-HT) was associated with improved five-year graft survival compared with CAV onset at earlier time-points, but similar and poor long-term outcomes. CAV timing post-HT was not associated with progression of CAV disease severity.

Background Freedom from rejection in pediatric heart transplant recipients is highly variable across centers. This study aimed to assess the center variation in methods used to diagnose rejection in the first-year post-transplant and determine the impact of this variation on patient outcomes. Methods The PHTS registry was queried for all rejection episodes in the first-year post-transplant (2010-2019). The primary method for rejection diagnosis was determined for each event as surveillance biopsy, echo diagnosis, or clinical. The percentage of first-year rejection events diagnosed by surveillance biopsy was used to approximate the surveillance strategy across centers. Methods of rejection diagnosis were described and patient outcomes were assessed based on surveillance biopsy utilization among centers. Results A total of 3985 patients from 56 centers were included. Of this group, 873 (22%) developed rejection within the first-year post-transplant. Surveillance biopsy was the most common method of rejection diagnosis (71.7%), but practices were highly variable across centers. The majority (73.6%) of first rejection events occurred within 3-months of transplantation. Diagnosis modality in the first-year was not independently associated with freedom from rejection, freedom from rejection with hemodynamic compromise, or overall graft survival . Conclusions Rejection in the first-year after pediatric heart transplant occurs in 22% of patients and most commonly in the first 3 months post-transplant. Significant variation exists across centers in the methods used to diagnose rejection in pediatric heart transplant recipients, however, these variable strategies are not independently associated with freedom from rejection, rejection with hemodynamic compromise, or overall graft survival.

Hypertension is a known complication of pediatric heart transplantation. We sought to identify factors associated with anti-hypertensive use in pediatric heart transplant recipients immediately post-transplant and oral anti-hypertensive use at discharge and 1-year post-transplant. Retrospective chart review was conducted of patients ≤18 years who underwent heart transplantation at two major heart transplant centers between August 1, 2009 and December 31, 2017 with ≥1-year follow-up. Exclusion criteria included re-transplant, multi-organ recipients, survival

Background Immunosuppression is considered a risk factor for more severe clinical presentation of COVID‐19. Limited data regarding clinical outcome exist in adults, whereas very little is known about the spectrum of the disease in pediatric heart transplant recipients. Methods We retrospectively reviewed the charts of young heart transplant patients from our tertiary care center during the coronavirus pandemic in New York City and identified patients infected with SARS‐CoV‐2. Results We present four cases with COVID‐19 disease and elaborate on their presentation and clinical course. Conclusions Although far from conclusive and limited by the small sample size and selection bias, these cases demonstrate mild and self‐limited disease despite immunosuppressive therapy and various comorbidities that are expected to increase the severity of the clinical picture based on extrapolation from the adult experience with this novel disease.

INTRODUCTION: Relative contraindications for adenosine use include heart transplant history and the anti-platelet agent dipyridamole. In an initial prospective study, the safety and efficacy of adenosine to induce atrioventricular (AV) block was demonstrated in pediatric and young adult heart transplant recipients. Over half of this cohort was on dipyridamole, which was suspended before testing. This prospective follow-up study evaluates the safety and efficacy of an adenosine dosing protocol in healthy young patients maintained on dipyridamole after heart transplant. METHODS: After routine cardiac biopsy (4/2016 – 6/2017), a temporary ventricular pacing catheter was placed and adenosine was dosed in stepwise increments until AV block occurred (maximum dose 200mcg/kg up to 12mg). The primary outcome was clinically significant asystole (≥ 12 seconds). Secondary outcomes included dose of adenosine eliciting AV block, AV block duration, dysrhythmias and patient reported symptoms. Incidence and 95% confidence intervals for categorical outcomes were calculated. Paired outcomes were compared to the parent study. RESULTS: Thirty of 39 eligible patients (5 – 24 years) were tested. No patient (0%, CI 0 – 8%) experienced clinically significant asystole. AV block occurred in 29/30 patients (97%, CI 86 – 100%). The median dose causing AV block was 50mcg/kg (vs. 100mcg/kg off dipyridamole; P=0.011). Seventeen patients (57%, CI 39 – 72%) required less adenosine to achieve AV block on dipyridamole; six (20%) required more. AV block occurred only at doses ≥25mcg/kg. Length of block, frequency of atrial/ventricular ectopy and incidence of subjective symptoms after adenosine were not different on dipyridamole. No atrial fibrillation/flutter occurred. CONCLUSION: AV block occurs often at twofold lower adenosine doses in healthy pediatric and young adult heart transplant recipients taking oral dipyridamole, as compared to previous testing of the same cohort off dipyridamole. Results still suggest that initial dosing of 25mcg/kg (max 0.8mg) with stepwise escalation poses low risk of prolonged asystole.

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.

The current understanding of the genotype-phenotype relationship in arrhythmogenic cardiomyopathy continues to evolve. Initial studies described arrhythmogenic right ventricular cardiomyopathy as a fibrofatty replacement of cardiac myocytes in the right ventricle associated with a high incidence of ventricular arrhythmias. Newer studies suggest a broader phenotypic array of disease driven by distinct genetic mutations. This includes arrhythmogenic left ventricular cardiomyopathy associated with DSP mutations. Herein, we describe an unusual case of an adolescent male with syncope and findings of ALVC secondary to DSP mutation.

Background Previous studies in adults have suggested that donor dopamine treatment may improve recipient outcomes in organ transplantation; in this analysis, we aimed to determine if donor dopamine reduces the incidence of postoperative right heart failure (RHF) in pediatric heart transplant recipients. Methods Data for recipients aged 18 years or younger transplanted at our institution between January 1, 2000, and June 15, 2011, and their respective donors were obtained. The presence of postoperative RHF was assessed for in all subjects. Donor dopamine dose was stratified into 3 groups: none, low-dose (≤5 μg/kg per minute), and high-dose (>5 μg/kg per minute). Logistic regression was used to assess the relationship between donor dopamine dose and recipient RHF. Results Of 192 recipients, 34 (18%) experienced postoperative RHF. There was no difference in baseline demographics between recipients with and without RHF. When controlling for pulmonary vascular resistance index, graft ischemic time, and cardiopulmonary bypass time, donor low-dose dopamine was independently associated with a decreased risk of RHF (odds ratio, 0.16; 95% confidence interval, 0.04-0.70; P = 0.02); however high-dose dopamine was neither associated with, nor protective of, RHF (odds ratio, 0.31; 95% confidence interval, 0.06-1.6; P = 0.16). Conclusions Despite advances in perioperative care of the recipient, RHF persists as a complication of pediatric heart transplantation. In this study, donor pretreatment with low-dose dopamine is associated with a decreased risk of postoperative RHF in pediatric heart recipients. Further studies into this association may be useful in determining the utility of empiric donor pretreatment with low-dose dopamine.

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.

Rejection with severe hemodynamic compromise is a significant source of morbidity and mortality for pediatric heart transplant patients. Traditionally, treatment for these patients includes inotropes and escalation to extracorporeal membrane oxygenation (ECMO) when necessary. There is increasing interest in using percutaneous ventricular assistive devices in the pediatric population as a less invasive alternative to ECMO. We report the largest case series to date of biventricular support using percutaneous Impella devices. Retrospective case series was performed by chart review. Hemodynamics, left ventricular ejection fraction (LVEF), and indices of end organ function were collected before and after Impella placement. A 14-year-old male, 18-year-old male, and 19-year-old female, all status post heart transplant, presented with severely decreased biventricular function due to presumed clinical rejection, requiring maximal inotropic support without improvement. In all the three cases, simultaneous Impella CP and RP devices were placed percutaneously. Prior to implantation, LVEFs were 40%, 23%, and 25%, respectively. Hemodynamics measured invasively prior to device placement showed elevated filling pressures. Adverse events while on support included bleeding, hemolysis, and right femoral arterial dissection during implantation. All patients were successfully weaned from the devices and survived to discharge. The average time of right-sided support and total support was 11 days and 13 days, respectively. After device removal, right-sided pressures and echocardiographic measurements showed improvement in all patients. Bilateral Impella configuration (BiPella) is a viable option for temporary mechanical circulatory support in pediatric patients with significant graft dysfunction.

Endomyocardial biopsy (EMB) is a common procedure used to aid in the diagnosis, prognosis and treatment of suspected pediatric cardiomyopathy. In suspected cardiomyopathy, no multicenter experience has previously reported on the safety and utility of EMBs. Retrospectively, adverse event (AE) and patient and procedural characteristics were obtained at seven institutions participating in the Congenital Cardiac Catheterization Outcomes Project for both a cardiomyopathy (n = 158) and a post-transplant surveillance (n = 2665) cohort. Descriptive information regarding biopsy indication, pathology and clinical management based on EMB findings were retrospectively obtained. High-severity AEs were more common in the cardiomyopathy cohort when compared to the post-transplant surveillance cohort. The cardiomyopathy cohort was younger, more hemodynamically vulnerable and required more cardiorespiratory support during the procedure. The eight high-severity AEs in the cardiomyopathy group included one myocardial perforation, two ECMO cannulations and three deaths following the EMB. Factors associated with high-severity AEs included performing another catheter-based intervention during the EMB and longer fluoroscopy time. Notably, an increased number of biopsy attempts did not increase the risk of an AE. Suspected myocarditis was the most common indication. Diagnostic EMB pathology and thus alteration to clinical management based on pathology occurred more frequently in patients with suspected myocarditis. In conclusion, there is an increased incidence of high-severity AEs in patients undergoing EMB for suspected cardiomyopathy. EMB may be more clinically useful in the management of suspected myocarditis. The increased risk of high-severity AEs when additional interventions are performed highlights the hemodynamic vulnerability in patients with suspected cardiomyopathy.

OBJECTIVES To describe our experience, at a large pediatric heart transplant center, with percutaneous coronary interventions (PCI) for cardiac allograft vasculopathy (CAV). BACKGROUND CAV is a leading cause of late graft failure, mortality, and re-transplantation in pediatric heart transplant (HTx) recipients. Studies of PCI in adult patients have shown some short-term improvements, but no significant change in long-term outcomes. There are limited data on PCI for CAV in pediatric patients. We describe the largest single-center experience to date. METHODS We performed a retrospective chart review of all pediatric HTx recipients who underwent PCI for a diagnosis of CAV from 2005 to 2014. RESULTS Twenty-three procedures were performed in 13 patients, at a median age of 16.4 years (range 5.6-21.2) and median time from HTx to first PCI of 8.3 years (range 2.9-20.3). Three cases consisted of angioplasty alone, two cases had bare metal stents implanted, and the remaining 18 had drug-eluting stents implanted. There was acute procedural success in all but one case, and there was only one procedure-related complication (rebleeding from access site). During the follow-up interval (median 10.4 months, range 0.2-111.8), 7/13 patients had repeat PCI performed, two patients died (at 1.8 and 5.8 months post-PCI), and five were re-transplanted (range 0.2-18 months post-PCI). Freedom from death or retransplant by Kaplan-Meier analysis was 54% at 1 year. CONCLUSIONS PCI can be performed safely and effectively in pediatric HTx recipients with CAV. Similar to the adult experience, there remains a high rate of disease progression and graft failure. © 2016 Wiley Periodicals, Inc.

Right atrial pressure (RAP) reflects right-sided cardiac hemodynamics and is useful in management of patients with cardiac and systemic disease. Studies in older adults demonstrated that inferior vena cava (IVC) diameter, IVC collapsibility index, hepatic vein systolic filling fraction (SFF), and right atrial volume (RAV) correlated with mean RAP at catheterization. This study aimed to assess the utility of echocardiographic parameters for assessment of RAP in children and young adults. Patients with pulmonary hypertension or heart transplantation undergoing right heart catheterization were recruited for this prospective observational pilot study. Transthoracic echocardiographic assessment of RAP was performed simultaneously with catheterization. For each parameter, three consecutive cardiac cycles were recorded. Long- and short-axis images of the IVC were obtained. RAV was assessed by area-length and biplane methods. IVC diameters and RAV were indexed to body surface area (BSA)(0.5) and (BSA)(1.4), respectively. Relationships between echocardiographic parameters and mean RAP were correlated using "Pearson's r." Fifty subjects aged 0.3-23 years (median 13, mean 12.3 ± 7 years) were enrolled. Mean RAP correlated modestly with RAV (r = 0.51, p < 0.001). Long-axis IVCmax (r = 0.30, p < 0.05) and tricuspid E wave velocity (r = 0.36, p < 0.01) also correlated with mean RAP. RV free wall tissue Doppler velocities, IVC collapsibility index, and hepatic vein SFF had no relation to mean RAP. In a pediatric and young adult population with pulmonary hypertension or heart transplantation, echocardiographic assessment of RAV and long-axis IVCmax provided a reasonable estimate of mean RAP. IVC collapsibility index and hepatic vein SFF demonstrated no association with mean RAP.

Influenza is a cause of significant morbidity and mortality worldwide. Myocarditis is a rare complication of the virus and can vary widely in severity. The published cases of influenza B myocarditis in children tend to be severe with a high mortality rate. Current standard treatment of viral myocarditis is supportive care, although immunomodulatory therapies, such as steroids and intravenous immunoglobulin, are often used. T cells have been implicated in causing significant myocyte damage in myocarditis by leading to the downstream production of antibodies against viral and myocyte antigens; this has created a theoretical basis for the use of antithymocyte globulin to target T cells in these patients. We present a case of acute fulminant influenza B myocarditis in a pediatric patient that required mechanical circulatory support and improved only after treatment with antithymocyte globulin.

Anti-HLA donor-specific antibodies are associated with worse outcomes after organ transplantation. Among sensitized pediatric heart candidates, requirement for negative donor-specific cytotoxicity crossmatch increases wait times and mortality. However, transplantation with positive crossmatch may increase posttransplantation morbidity and mortality. We address this clinical challenge in a prospective, multicenter, observational cohort study of children listed for heart transplantation (Clinical Trials in Organ Transplantation in Children-04 [CTOTC-04]). Outcomes were compared among sensitized recipients who underwent transplantation with positive crossmatch, nonsensitized recipients, and sensitized recipients without positive crossmatch. Positive crossmatch recipients received antibody removal and augmented immunosuppression, while other recipients received standard immunosuppression with corticosteroid avoidance. This first CTOTC-04 report summarizes study rationale and design and relates pretransplantation sensitization status using solid-phase technology. Risk factors for sensitization were explored. Of 317 screened patients, 290 were enrolled and 240 underwent transplantation. Core laboratory evaluation demonstrated that more than half of patients were anti-HLA sensitized. Greater than 80% of sensitized patients had class I (with or without class II) HLA antibodies, and one-third of sensitized patients had at least 1 HLA antibody with median fluorescence intensity of ≥8000. Logistic regression models demonstrated male sex, weight, congenital heart disease history, prior allograft, and ventricular assist device are independent risk factors for sensitization.

Transcatheter stent implantation for SVC obstruction following OHT has been well described, particularly in pediatric patients. This study describes a large single-center pediatric heart transplant experience that investigates the risk factors for SVC stenosis requiring stent implantation and its associated outcomes. All pediatric OHTs between January 1, 2000, and December 12, 2016, were examined for risk factors. Twelve of 349 (3.4%) OHTs required SVC stent implantation. Patients who required stents were younger (2.5 years vs 10.0 years, P = 0.0097), weighed less (8.6 kg vs 26.0 kg, P = 0.0024), and were more likely to have had CHD (83% vs 32%, P = 0.001) or previous SCPA (58% vs 18%, P = 0.002). Bicaval anastomosis was not associated with subsequent SVC stent implantation. Symptoms included SVC syndrome and chylous effusions. All 12 patients had evidence of significant SVC obstruction by both echocardiographic Doppler interrogation and transcatheter angiography. There were no acute procedural complications; however, reinterventions were common (four of 12 patients) and occurred at a mean of 5.7 ± 3.6 months poststent implantation. In conclusion, transcatheter SVC stent implantation is safe and effective after OHT. There were significant associations between SVC stent implantation and younger age, smaller weight, CHD, and history of SCPA, but not with bicaval anastomosis or donor-recipient weight ratio.

If coronary artery sequelae are the only suggestive signs of previous Kawasaki disease, the diagnosis may easily be missed. We describe a rare case of a child with severe occlusive coronary disease likely owing to missed Kawasaki disease. This diagnosis was not initially considered given the age and absence of suggestive history. Careful echocardiographic assessment and low-radiation coronary CT angiogram resulted in successful diagnosis and treatment.

Background Pulmonary arterial hypertension (PAH) is a progressive disease that, without early identification and treatment, may lead to right heart failure, multi-organ dysfunction and early death. In severe PAH, in addition to maximal medical therapy, balloon atrial septostomy (BAS) may be used for palliation and as a bridge to lung transplantation. We present our contemporary institutional experience utilizing BAS in adult and pediatric patients with severe PAH. Methods We performed a retrospective analysis of 46 BASs performed in 32 patients with PAH from 2002 to 2013. Data obtained included vital status, functional class, medications, hemodynamic measurements from right heart catheterizations and biomarkers. Lung transplantation–free and repeat-BAS–free survival was analyzed. Results Median age at BAS was 23 (range 1 to 56) years. The most common indications were symptomatic right heart failure (21 of 46 patients) and pre-syncope/syncope (19 of 46 patients); 69% of patients were WHO Functional Class III or IV pre-BAS. There were no procedural complications or deaths. There were no significant differences in biomarkers or hemodynamic findings between pre-BAS and 1 year or latest follow-up. Seven patients were successfully bridged to lung transplantation. Lung transplantation–free and repeat-BAS–free survival at 30 days, 1 year and 5 years was 87%, 61% and 32%, respectively. Conclusions In our experienced center, BAS was shown to be safe in patients with severe PAH on maximal medical management, with no procedural deaths or complications. BAS was safely used as a bridge to lung transplantation or to alleviate right heart failure symptoms and/or syncope. Other potential benefits for end-organ function and overall survival remain to be determined.

Background: Supraventricular tachycardia is common after heart transplantation. Adenosine, the standard therapy for treating supraventricular tachycardia in children and adults without transplantation, is relatively contraindicated after transplantation because of a presumed risk of prolonged atrioventricular block in denervated hearts. This study tested whether adenosine caused prolonged asystole after transplantation and if it was effective in blocking atrioventricular nodal conduction in these patients. Methods: This was a single-center prospective clinical study including healthy heart transplant recipients 6 months to 25 years of age presenting for routine cardiac catheterization during 2015 to 2016. After catheterization, a transvenous pacing catheter was placed and adenosine was given following a dose-escalation protocol until atrioventricular block was achieved. The incidence of clinically significant asystole (≥12 seconds after adenosine) was quantified. The effects of patient characteristics on adenosine dose required to produce atrioventricular block and duration of effect were also measured. Results: Eighty patients completed adenosine testing. No patient (0%; 95% confidence interval, 0–3) required rescue ventricular pacing. Atrioventricular block was observed in 77 patients (96%; 95% confidence interval, 89–99). The median longest atrioventricular block was 1.9 seconds (interquartile range, 1.4–3.2 seconds), with a mean duration of adenosine effect of 4.3±2.0 seconds. No patient characteristic significantly predicted the adenosine dose to produce atrioventricular block or duration of effect. Results were similar across patient weight categories. Conclusions: Adenosine induces atrioventricular block in healthy pediatric and young adult heart transplant recipients with minimal risk when low initial doses are used (25 μg/kg; 1.5 mg if ≥60 kg) and therapy is gradually escalated. Clinical Trial Registration: URL: http://www.clinicaltrials.gov . Unique identifier: NCT02462941.

BU and CY (BU/CY; 200 mg/kg) before HLA-matched sibling allo-SCT in children with sickle cell disease (SCD) is associated with ~85% EFS but is limited by the acute and late effects of BU/CY myeloablative conditioning. Alternatives include reduced toxicity but more immunosuppressive conditioning. We investigated in a prospective single institutional study, the safety and efficacy of a reduced-toxicity conditioning (RTC) regimen of BU 12.8-16 mg/kg, fludarabine 180 mg/m(2), alemtuzumab 54 mg/m(2) (BFA) before HLA-matched sibling donor transplantation in pediatric recipients with symptomatic SCD. Eighteen patients, median age 8.9 years (2.3-20.2), M/F 15/3, 15 sibling BM and 3 sibling cord blood (CB) were transplanted. Mean whole blood and erythroid donor chimerism was 91% and 88%, at days +100 and +365, respectively. Probability of grade II-IV acute GVHD was 17%. Two-year EFS and OS were both 100%. Neurological, pulmonary and cardiovascular function were stable or improved at 2 years. BFA RTC and HLA-matched sibling BM and CB allo-SCT in pediatric recipients result in excellent EFS, long-term donor chimerism, low incidence of GVHD and stable/improved organ function.

High pulmonary vascular resistance index (PVRI) can lead to right ventricular dysfunction and failure of the donor heart early after pediatric heart transplantation. Oral pulmonary vasodilators such as sildenafil have been shown to be effective modifiers of pulmonary vascular tone. We performed a retrospective, observational study comparing patients treated with sildenafil ("sildenafil group") to those not treated with sildenafil ("nonsildenafil group") after heart transplantation from 2007 to 2012. Pre- and posttransplant data were obtained, including hemodynamic data from right heart catheterizations. Twenty-four of 97 (25%) transplant recipients were transitioned to sildenafil from other systemic vasodilators. Pretransplant PVRI was higher in the sildenafil group (6.8 ± 3.9 indexed Woods units [WU]) as compared to the nonsildenafil group (2.5 ± 1.7 WU, p=0.002). In the sildenafil group posttransplant, there were significant decreases in systolic pulmonary artery pressure, mean pulmonary artery pressure, transpulmonary gradient and PVRI (4.7 ± 2.9 WU before sildenafil initiation to 2.7 ± 1 WU on sildenafil, p=0.0007). While intubation time, length of inotrope use and time to hospital discharge were longer in the sildenafil group, survival was similar between both groups. Oral sildenafil was associated with a significant improvement in right ventricular dysfunction and invasive hemodynamic measurements in pediatric heart transplant recipients with high PVRI early after transplant.

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 Hypertension (HTN) is a known complication of pediatric heart transplant (HTx) both immediately post-Tx and long-term. We sought to identify factors associated with antihypertensive medication (anti-HTN) use in pediatric HTx recipients in the ICU post-Tx, and need for oral anti-HTNs at post-Tx discharge (d/c) and 1 year (yr) post-Tx. Methods Retrospective chart review of patients (pts) ≤18 years who underwent initial HTx at UPMC Children's Hospital of Pittsburgh and Morgan Stanley Children's Hospital of NY between 8/1/09-12/31/17 with ≥1 yr follow-up. Exclusion criteria included re-Tx, multi-organ recipients, pts who survived Results There were 188 pts who met inclusion criteria. Anti-HTN drips were required in the ICU post-Tx in 46 pts (24.5%) for a median of 3 days (1-21 days). Drips were nitroprusside (28/46), calcium channel blocker (8/46), beta-blocker (1/46) and multiple (9/46). Oral anti-HTNs were required in 58 pts (30.9%) at d/c and 1 yr post-Tx. Figure 1 demonstrates significant associations by univariate and multivariate analyses. Anti-HTN drip in the ICU post-Tx was associated with donor-to-recipient weight ratio (wt ratio). Oral anti-HTN use at d/c was associated with wt ratio and pre-Tx VAD, and at 1 yr post-Tx was associated with age at Tx, steroid use at d/c and oral anti-HTN use at d/c. Conclusion HTN is common immediately and at 1 yr post-Tx, with over a quarter of pts requiring anti-HTNs. Wt ratio was the only independent predictor of anti-HTN use in the early post-Tx period, whereas VAD use was also associated with anti-HTN use at d/c. Interestingly, anti-HTN use at 1 yr post-Tx was not associated with those factors, but rather with age at Tx and steroid use.

Purpose As survival in pediatric heart transplant (HTx) improves, an increasing proportion of children receiving HTx are entering adulthood. Many quality of life (QOL) studies have looked at adult HTx patients, but only a few have looked at the population who received a HTx as a child. It is important to examine how this chronic illness affects children's development and eventual transition to adulthood, so that we may improve counseling of future patients. Methods Patients who received a HTx with the pediatric program at Columbia University Medical Center from 1984 through August 1, 2007, who are now > 18 years old and have survived over 10 years since HTx were asked to complete 2 surveys: (1) CHONY Pediatric Heart Transplant Life Achievement Survey, which examines marital and employment status, education level, personal income, health insurance status, and living environment, and (2) Quality of Life Index (QLI) by Ferrans and Powers, which scores life satisfaction from 0 (least satisfied) to 1 (most satisfied) as an overall score, and in 4 distinct domains (health/function, socioeconomic, psych/spiritual, and family). Results The Life Achievement Survey was completed by 54 patients (median age 8 years at transplant and 25 years at time of survey, 44% female, 26% transplanted for congenital heart disease). Fifteen patients have either been married (1 divorced) or part of an unmarried couple, and 7 have children (6 biological including 1 female). Thirty-seven patients are either employed or a student, and 16 earn over $25,000 annually. Almost a third of patients (n=16) live independently, while 5 are homeowners. Fifty patients completed the QLI and reported a mean overall QLI 0.76±0.14. The scores in the health/function, socioeconomic, psych/spiritual, and family domains were 0.77, 0.74, 0.74, and 0.8, respectively. Conclusion Our cohort of adult survivors of pediatric HTx have achieved many standard adult milestones such as receiving an education, maintaining relationships, being employed, and living independently. Additionally, they report a suitable QOL, especially in the health/function and family domains.

Cardiac catheterization is important for the management of patients with pulmonary arterial hypertension (PAH). It is used for diagnosis, assessment, and monitoring of PAH patients, as well as to perform interventions such as balloon atrial septostomy and coil embolization of collateral vessels. Although reports on the risks of catheterization in PAH patients are scarce, many centers hesitate to perform these procedures in such fragile patients. We performed a retrospective chart review of all cardiac catheterizations performed in PAH patients over 10 years at our pulmonary hypertension center. Demographic, hemodynamic, and outcome data were collected. Complication rates were determined, and multivariate proportional hazards modeling was performed to identify predictors of catheterization-related complications. There were 1,637 catheterizations performed in 607 patients over 10 years. Pediatric patients accounted for 50% of these cases, 48% were performed in patients with idiopathic PAH, and 49% were performed under general anesthesia. While the overall complication rate was 5.7%, the rate of major complications was only 1.2% ([Formula: see text]). Although there were 8 deaths during the admission following catheterization, only 4 of these were related to the procedure, yielding a catheterization-related mortality of 0.2%. In conclusion, when performed at a pulmonary hypertension center with expertise in the care of PAH patients, cardiac catheterization is associated with low complication rates and mortality, and it should remain an important tool in the management of these patients.

Pulmonary arterial hypertension (PAH) with increased pulmonary vascular resistance (PVR) is a frequent complication of congenital heart disease (CHD), most commonly occurring with systemic-to-pulmonary shunt lesions. The natural disease progression involves pulmonary endothelial damage due to exposure to increased pulmonary blood flow and pressure, and in its most severe form results in Eisenmenger syndrome (ES), in which there is shunt reversal and cyanosis. Due to anatomic and histopathologic similarities of PAH in patients with CHD, as well as those with idiopathic and other forms of Group 1 PAH, there is an evolving interest and role for the use of the newer targeted PAH therapies in CHD patients. While early closure of shunt lesions is the best preventive measure, the use of targeted medical therapies when a patient is too high risk for surgery, following surgical repair, and even in those with reversal of shunting due to advanced disease, has emerged over the past decade.

Orthotopic heart transplantation remains the definitive treatment of choice for patients with end-stage heart failure, however, elevated pulmonary vascular resistance index (PVRI) is a reported risk factor for mortality after heart transplant, and when severely elevated, is considered an absolute contraindication. Use of a ventricular assist device has been proposed as one treatment for reducing PVRI in potential heart transplant candidates refractory to medical vasodilator therapies. We report on a teenage patient with dilated cardiomyopathy and severely elevated PVRI, unresponsive to pulmonary vasodilator therapy, who underwent left ventricular assist device implantation to safely allow for aggressive pulmonary vasodilator therapy and to decrease PVRI. The resulting dramatic improvement in PVRI in a relatively short period of time allowed for successful heart transplantation, avoiding the need for heart-lung transplant.

Pulmonary arterial hypertension (PAH) with increased pulmonary vascular resistance is a frequent complication of congenital heart disease, most commonly occurring with systemic-to-pulmonary shunt lesions. The natural disease progression involves pulmonary endothelial damage due to exposure to increased pulmonary blood flow and pressure, and in its most severe form results in Eisenmenger syndrome, in which there is shunt reversal and cyanosis. Because of anatomic and pathologic similarities of PAH in the congenital heart disease population compared with idiopathic PAH, there is an evolving role for the use of the newer targeted PAH therapies in these patients. While early closure of shunt lesions is the best preventive measure, the concept of a combined medical-surgical approach for these patients has emerged as well. An additional group of patients that may benefit from the use of targeted PAH therapies includes the rapidly growing subpopulation of patients who have undergone the Fontan operation due to single ventricle anatomy.

No prior reports documenting the safety and diagnostic yield of cardiac catheterization and endomyocardial biopsy (EMB) in heart transplant recipients include multicenter data.Data on the safety and diagnostic yield of EMB procedures performed in heart transplant recipients were recorded in the Congenital Cardiac Catheterization Outcomes Project database at 8 pediatric centers during a 3-year period. Adverse events (AEs) were classified according to a 5-level severity scale. Generalized estimating equation models identified risk factors for high-severity AEs (HSAEs; Levels 3-5) and non-diagnostic biopsy samples.A total of 2,665 EMB cases were performed in 744 pediatric heart transplant recipients (median age, 12 years [interquartile range, 4.8, 16.7]; 54% male). AEs occurred in 88 cases (3.3%), of which 28 (1.1%) were HSAEs. AEs attributable to EMB included tricuspid valve injury, transient complete heart block, and right bundle branch block. Amongst 822 cases involving coronary angiography, 10 (1.2%) resulted in a coronary-related AE. There were no myocardial perforations or deaths. Multivariable risk factors for HSAEs included fewer prior catheterizations (p = 0.006) and longer case length (p0.001). EMB yielded sufficient tissue for diagnosis in 99% of cases. Longer time since heart transplant was the most significant predictor of a non-diagnostic biopsy sample (p0.001).In the current era, cardiac catheterizations involving EMB can be performed in pediatric heart transplant recipients with a low AE rate and high diagnostic yield. Risk of HSAEs is increased in early post-transplant biopsies and with longer case length. Longer time since heart transplant is associated with non-diagnostic EMB samples.

Cardiomyopathies in children encompass a broad range of diseases, both genetic and acquired, which manifest as a primary cardiac disorder or as a cardiomyopathy secondary to systemic disease. The burden of this group of disorders is substantial, and growing on a global scale. The availability of disease altering treatments is limited, and therefore a focused review on the prevention of cardiomyopathies is justified. In this review, we address the prevention of cardiomyopathy in children by dealing with the root causes of disease at a molecular, clinical and population level. Recent years have yielded promising returns in basic research related to gene-targeted therapy, specific anti-viral therapies and modification of the effects of cardiotoxic drugs. Much work remains to be done in the fields of vaccine development, public health and adoption of available treatments. Effective research in this field will require that diagnostic methods are both refined, and made available more broadly, from imaging to gene testing. Much of our knowledge today is derived from the use of registries, which have successfully catalogued the detailed phenotype of affected patients, and provided long-term longitudinal follow up of affected individuals.

Pulmonary hypertension and cor pulmonale have long been known to be complications of sickle cell disease, thought mostly to affect the adult population. Recently, pediatric studies in sickle cell patients have uncovered a similar prevalence of elevated tricuspid regurgitation jet velocities, a finding consistent with increased right-sided pressures, when compared with adult counterparts. Implications on mortality to date, while significant in the adult sickle cell population, appear to be unsubstantiated in children and adolescents. We review the recent literature in an attempt to discuss the prevalence, etiology and implications of an elevated tricuspid regurgitation jet velocity in pediatric sickle cell patients. We also suggest screening, diagnostics, treatment and follow-up plans that may improve the disease burden of pulmonary hypertension in both pediatric and adult sickle cell populations.

Left-ventricular noncompaction (LVNC) is an echocardiographic finding of increasing frequency in pediatrics; however, predictors of outcomes have been difficult to identify. We conducted a retrospective review of pediatric patients at the Morgan Stanley Children’s Hospital of New York from January of 1993 to September of 2009 to identify predictors of the primary outcome of death or heart transplantation. LVNC was identified in 50 patients, 34 of them < 1 year of age. Death or transplantation occurred in 26 patients, with a median survival of 1.17 years after presentation. Patients surviving 1 year after presentation had 75% conditional survival, and patients surviving 2 years after presentation had 92% conditional survival. Hemodynamic instability, poor ventricular function, and LV dilatation were each independent predictors of poor outcome. Of the 21 patients who presented with hemodynamic instability, 17 died or underwent transplantation at a median of 0.08 years after presentation. In conclusion, LVNC is recognized more in younger patients; however, age is not a predictor of outcome. Patients who present with hemodynamic instability and poor ventricular function have decreased transplant-free survival, and most poor outcomes occur within the first year after presentation. Therefore, early listing for transplant may lead to better outcomes in this population.

To describe our experience, at a large pediatric heart transplant center, with percutaneous coronary interventions (PCI) for cardiac allograft vasculopathy (CAV).CAV is a leading cause of late graft failure, mortality, and re-transplantation in pediatric heart transplant (HTx) recipients. Studies of PCI in adult patients have shown some short-term improvements, but no significant change in long-term outcomes. There are limited data on PCI for CAV in pediatric patients. We describe the largest single-center experience to date.We performed a retrospective chart review of all pediatric HTx recipients who underwent PCI for a diagnosis of CAV from 2005 to 2014.Twenty-three procedures were performed in 13 patients, at a median age of 16.4 years (range 5.6-21.2) and median time from HTx to first PCI of 8.3 years (range 2.9-20.3). Three cases consisted of angioplasty alone, two cases had bare metal stents implanted, and the remaining 18 had drug-eluting stents implanted. There was acute procedural success in all but one case, and there was only one procedure-related complication (rebleeding from access site). During the follow-up interval (median 10.4 months, range 0.2-111.8), 7/13 patients had repeat PCI performed, two patients died (at 1.8 and 5.8 months post-PCI), and five were re-transplanted (range 0.2-18 months post-PCI). Freedom from death or retransplant by Kaplan-Meier analysis was 54% at 1 year.PCI can be performed safely and effectively in pediatric HTx recipients with CAV. Similar to the adult experience, there remains a high rate of disease progression and graft failure. © 2016 Wiley Periodicals, Inc.

Organ transplantation from ABO blood group-incompatible (ABOi) donors requires accurate detection, effective removal and subsequent surveillance of antidonor antibodies. Because ABH antigen subtypes are expressed differently in various cells and organs, measurement of antibodies specific for the antigen subtypes in the graft is essential. Erythrocyte agglutination, the century-old assay used clinically, does not discriminate subtype-specific ABO antibodies and provides limited information on antibody isotypes. We designed and created an ABO-glycan microarray and demonstrated the precise assessment of both the presence and, importantly, the absence of donor-specific antibodies in an international study of pediatric heart transplant patients. Specific IgM, IgG, and IgA isotype antibodies to nonself ABH subtypes were detected in control participants and recipients of ABO-compatible transplants. Conversely, in children who received ABOi transplants, antibodies specific for A subtype II and/or B subtype II antigens-the only ABH antigen subtypes expressed in heart tissue-were absent, demonstrating the fine specificity of B cell tolerance to donor/graft blood group antigens. In contrast to the hemagglutination assay, the ABO-glycan microarray allows detailed characterization of donor-specific antibodies necessary for effective transplant management, representing a major step forward in precise ABO antibody detection.

Objective To highlight the success of heart transplantation in patients with complex congenital heart disease and physiologic single lung by providing an update on the world's largest reported cohort. Methods Demographic, perioperative, postoperative, and outcomes data were collected retrospectively on all patients undergoing heart transplant to single lung at Columbia University Medical Center since 1992, and compared with all other patients undergoing transplants performed for single ventricle or tetralogy of Fallot during that time. Results Twenty-two patients (mean age, 20.6 years; range, 5 months-47 years) underwent heart transplant to single lung. Compared with controls (n = 67), the single lung group had more male patients and a greater proportion of tetralogy compared with single ventricle patients, although the single lung group had fewer post-Fontan patients. Age, weight, and body surface area were similar between the groups as were use of mechanical circulatory support and mechanical ventilation before transplant. Median time to extubation, time on inotropes, and length of stay were similar. There were 3 perioperative deaths, including a patient who died during postoperative day 1 from primary graft failure, likely related to a combination of elevated pulmonary vascular resistance and volume load. There were 5 additional mortalities during intermediate- and long-term follow-up, none of which were related to single-lung physiology. There was no significant survival difference between the groups. Conclusions In patients with complex congenital heart disease and single lung physiology, heart transplant alone remains an excellent option, with comparable outcomes to patients undergoing transplant with similar cardiac anatomy and dual lung physiology.