Single Ventricular Assist Device Care and Outcomes for Failed Stage I Palliation: A Single-Center Decade of Experience.

Single ventricular assist device (SVAD) use before and after stage I palliation (S1P) is increasing with limited data on outcomes. To address this knowledge gap, we conducted a single-center retrospective review to assess pre- and post-SVAD clinical status, complications, and outcomes. We leveraged a granular, longitudinal, local database that captures end-organ support, procedural interventions, hematologic events, laboratory data, and antithrombotic strategy. We identified 25 patients between 2013 and 2023 implanted at median age of 53 days (interquartile range [IQR] = 16-130); 80% had systemic right ventricles and underwent S1P. Median SVAD days were 54 (IQR = 29-86), and 40% were implanted directly from ECMO. Compared to preimplant, there was a significant reduction in inotrope use ( p = 0.013) and improved weight gain ( p = 0.008) post-SVAD. Complications were frequent including bleeding (80%), stroke (40%), acute kidney injury (AKI) (40%), infection (36%), and unanticipated catheterization (56%). Patients with in-hospital mortality had significantly more bleeding complications ( p = 0.02) and were more likely to have had Blalock-Thomas-Taussig shunts pre-SVAD ( p = 0.028). Survival to 1 year postexplant was 40% and included three recovered and explanted patients. At 1 year posttransplant, all survivors have technology dependence or neurologic injury. This study highlights the clinical outcomes and ongoing support required for successful SVAD use in failed single-ventricle physiology before or after S1P.

Heart Transplantation in Children With Down Syndrome.

Background Children with Down syndrome (DS) have a high risk of cardiac disease that may prompt consideration for heart transplantation (HTx). However, transplantation in patients with DS is rarely reported. This project aimed to collect and describe waitlist and post- HTx outcomes in children with DS. Methods and Results This is a retrospective case series of children with DS listed for HTx. Pediatric HTx centers were identified by their participation in 2 international registries with centers reporting HTx in a patient with DS providing detailed demographic, medical, surgical, and posttransplant outcome data for analysis. A total of 26 patients with DS were listed for HTx from 1992 to 2020 (median age, 8.5 years; 46% male). High-risk or failed repair of congenital heart disease was the most common indication for transplant (N=18, 69%). A total of 23 (88%) patients survived to transplant. All transplanted patients survived to hospital discharge with a median posttransplant length of stay of 22 days. At a median posttransplant follow-up of 2.8 years, 20 (87%) patients were alive, 2 (9%) developed posttransplant lymphoproliferative disorder, and 8 (35%) were hospitalized for infection within the first year. Waitlist and posttransplant outcomes were similar in patients with and without DS (P=non-significant for all). Conclusions Waitlist and post-HTx outcomes in children with DS selected for transplant listing are comparable to pediatric HTx recipients overall. Given acceptable outcomes, the presence of DS alone should not be considered an absolute contraindication to HTx.

Heterogeneous outcomes of liver disease after heart transplantation for a failed Fontan procedure.

BACKGROUND: Fontan-associated liver disease (FALD) uniformly affects patients with long-term Fontan physiology. The effect of isolated heart transplant (HT) on the course of FALD post-HT is not well understood. METHODS: We evaluated serial liver imaging pre- and post-HT to assess liver changes over time in a single-center retrospective analysis of Fontan HT recipients who had pre- and ≥1-year post-HT liver imaging. Available patient demographic and clinical data were reviewed, including available liver biopsy results. RESULTS: Serial liver imaging was available in 19 patients with a median age at HT of 12 years (range 3-23), the median age from Fontan to HT of 5.7 years (range 0.8-16), and the median time from imaging to follow up of 27 months (range 12-136 months). Pre-HT liver imaging was classified as follows: normal (n=1), congested (n=9), fibrotic (n=7), and cirrhotic (n=2). The majority of transplanted patients (15/19) had improvement in their post-HT liver imaging, including 13 patients with initially abnormal imaging pre-HT having normal liver imaging at follow-up. One patient had persistent cirrhosis at 26-month follow-up, one patient had unchanged fibrosis at 18-month follow-up, and one patient progressed from fibrosis pre-HT to cirrhosis post-HT at 136 months. No patients had overt isolated liver failure during pre- or post-HT follow-up. Liver biopsy did not consistently correlate with imaging findings. CONCLUSIONS: Post-HT liver imaging evaluation in Fontan patients reveals heterogeneous liver outcomes. These results not only provide evidence for the improvement of FALD post-HT but also show the need for serial liver imaging follow-up post-HT.

Quantitative cardiac magnetic resonance T2 imaging offers ability to non-invasively predict acute allograft rejection in children.

BACKGROUND: Monitoring for acute allograft rejection improves outcomes after cardiac transplantation. Endomyocardial biopsy is the gold standard test defining rejection, but carries risk and has limitations. Cardiac magnetic resonance T2 mapping may be able to predict rejection in adults, but has not been studied in children. Our aim was to evaluate T2 mapping in identifying paediatric cardiac transplant patients with acute rejection. METHODS: Eleven paediatric transplant patients presenting 18 times were prospectively enrolled for non-contrast cardiac magnetic resonance at 1.5 T followed by endomyocardial biopsy. Imaging included volumetry, flow, and T2 mapping. Regions of interest were manually selected on the T2 maps using the middle-third technique in the left ventricular septal and lateral wall in a short-axis and four-chamber slice. Mean and maximum T2 values were compared with Student's t-tests analysis. RESULTS: Five cases of acute rejection were identified in three patients, including two cases of grade 2R on biopsy and three cases of negative biopsy treated for clinical symptoms attributed to rejection (new arrhythmia, decreased exercise capacity). A monotonic trend between increasing T2 values and higher biopsy grades was observed: grade 0R T2 53.4 ± 3 ms, grade 1R T2 54.5 ms ± 3 ms, grade 2R T2 61.3 ± 1 ms. The five rejection cases had significantly higher mean T2 values compared to cases without rejection (58.3 ± 4 ms versus 53 ± 2 ms, p = 0.001). CONCLUSIONS: Cardiac magnetic resonance with quantitative T2 mapping may offer a non-invasive method for screening paediatric cardiac transplant patients for acute allograft rejection. More data are needed to understand the relationship between T2 and rejection in children.

Community study to uncover the full spectrum of rheumatic heart disease in Uganda.

OBJECTIVE: Estimates of the prevalence of rheumatic heart disease (RHD) in many endemic countries are limited to samples of children attending schools, which generate an incomplete picture of disease burden in communities. The present study conducted household-based RHD screening in a representative community in Gulu district, Uganda. METHODS: Members of households identified through a two-stage cluster-sampling approach between the ages of 5 years and 50 years were invited to undergo limited cardiac testing with a handheld echocardiogram to assess for the presence of RHD. Suspicious cases underwent confirmatory echocardiogram with a fully functional machine. RESULTS: Of the 2453 community members screened, 2.45% (95% CI 1.87% to 3.14%) showed echocardiographic evidence of RHD with 1.26% (95% CI 0.860% to 1.79%) having definite RHD. The overall prevalence of RHD among participants <20 years was 2.52% (95% CI 1.78% to 3.45%), with a borderline prevalence of 1.97% (95% CI 1.33% to 2.82%) and a definite prevalence of 0.544% (95% CI 0.235% to 1.07%). Prevalence rates among youth increased with age and peaked in the age group of 16-20 years. The overall adult prevalence (>20 years) of RHD was 2.34% (95% CI 1.49% to 3.49%). The majority of definite cases were mild (81%) and marked by mitral regurgitation and associated morphological valve changes (71%). CONCLUSION: Our data reveal a high prevalence of undiagnosed RHD within an endemic community and fill a critical gap in RHD epidemiology in African adults.

Bacterial infections after pediatric heart transplantation: Epidemiology, risk factors and outcomes.

BACKGROUND: Bacterial infections represent a major cause of morbidity and mortality in heart transplant recipients. However, data describing the epidemiology and outcomes of these infections in children are limited. METHODS: We analyzed the Pediatric Heart Transplant Study database of patients transplanted between 1993 and 2014 to determine the etiologies, risk factors and outcomes of children with bacterial infections post-heart transplantation. RESULTS: Of 4,458 primary transplants in the database, there were 4,815 infections that required hospitalization or intravenous therapy, 2,047 (42.51%) of which were bacterial. The risk of bacterial infection was highest in the first month post-transplant, and the bloodstream was the most common site (24.82%). In the early post-transplant period (<30 days post-transplant), coagulase-negative staphylococci were the most common pathogens (16.97%), followed by Enterobacter sp (11.99%) and Pseudomonas sp (11.62%). In the late post-transplant period, community-acquired pathogens Streptococcus pneumoniae (6.27%) and Haemophilus influenzae (2.82%) were also commonly identified. Patients' characteristics independently associated with acquisition of bacterial infection included younger age (p < 0.0001) and ventilator (p < 0.0001) or extracorporeal membrane oxygenation (p = 0.03) use at time of transplant. Overall mortality post-bacterial infection was 33.78%, and previous cardiac surgery (p < 0.001) and multiple sites of infection (p = 0.004) were independent predictors of death. CONCLUSIONS: Bacteria were the most common causes of severe infections in pediatric heart transplant recipients and were associated with high mortality rates. The risk of acquiring a bacterial infection was highest in the first month post-transplant, and a large proportion of the infections were caused by multidrug-resistant pathogens.

Hybrid Exclusion of HeartMate ІІ Left Ventricular Assist Device After Bridge to Recovery.

We present a hybrid technique of left ventricular assist device exclusion after bridge to recovery in a pediatric patient, using percutaneous occlusion of the outflow graft and surgical excision of driveline. This technique has the advantage of avoiding chest reentry and cardiopulmonary bypass.

Simplified rheumatic heart disease screening criteria for handheld echocardiography.

BACKGROUND: Using 2012 World Heart Federation criteria, standard portable echocardiography (STAND) reveals a high burden of rheumatic heart disease (RHD) in resource-poor settings, but widespread screening is limited by cost and physician availability. Handheld echocardiography (HAND) may decrease costs, but World Heart Federation criteria are complicated for rapid field screening, particularly for nonphysician screeners. The aim of this study was to determine the best simplified screening strategy for RHD detection using HAND. METHODS: In this prospective study, STAND (GE Vivid q or i or Philips CX-50) was performed in five schools in Gulu, Uganda; a random subset plus all children with detectable mitral regurgitation or aortic insufficiency also underwent HAND (GE Vscan). Borderline or definite RHD cases were defined by 2012 World Heart Federation criteria on STAND images, by two experienced readers. HAND studies were reviewed by cardiologists blinded to STAND results. Single and combined HAND parameters were evaluated to determine the simplified screening strategy that maximized sensitivity and specificity for case detection. RESULTS: In 1,439 children (mean age, 10.8 ± 2.6 years; 47% male) with HAND and STAND studies, morphologic criteria and the presence of any mitral regurgitation by HAND had poor specificity. The presence of aortic insufficiency was specific but not sensitive. Combined criteria of mitral regurgitation jet length ≥ 1.5 cm or any aortic insufficiency best balanced sensitivity (73.3%) and specificity (82.4%), with excellent sensitivity for definite RHD (97.9%). With a prevalence of 4% and subsequent STAND screening of positive HAND studies, this would reduce STAND studies by 80% from a STAND-based screening strategy. CONCLUSIONS: In resource-limited settings, HAND with simplified criteria can detect RHD with good sensitivity and specificity and decrease the need for standard echocardiography. Further study is needed to validate screening by local practitioners and long-term outcomes.

The utility of handheld echocardiography for early rheumatic heart disease diagnosis: a field study.

AIMS: The World Heart Federation (WHF) guidelines for rheumatic heart disease (RHD) are designed for a standard portable echocardiography (STAND) machine. A recent study in a tertiary care centre demonstrated that they also had good sensitivity and specificity when modified for use with handheld echocardiography (HAND). Our study aimed to evaluate the performance of HAND for early RHD diagnosis in the setting of a large-scale field screening. METHODS AND RESULTS: STAND was performed in 4773 children in Gulu, Uganda, with 10% randomly assigned to also undergo HAND. Additionally, any child with mitral or aortic regurgitation also underwent HAND. Studies were performed by experienced echocardiographers and blindly reviewed by cardiologists using 2012 WHF criteria, which were modified slightly for HAND--due to the lack of spectral Doppler capability. Paired echocardiograms were performed in 1420 children (mean age 10.8 and 53% female), resulting in 1234 children who were normal, 133 who met criteria for borderline RHD, 47 who met criteria for definite RHD, and 6 who had other diagnoses. HAND had good sensitivity and specificity for RHD detection (78.9 and 87.2%, respectively), but was most sensitive for definite RHD (97.9%). Inter- and intra-reviewer agreement ranged between 66-83 and 71.4-94.1%, respectively. CONCLUSIONS: HAND has good sensitivity and specificity for diagnosis of early RHD, performing best for definite RHD. Protocols for RHD detection utilizing HAND will need to include confirmation by STAND to avoid over-diagnosis. Strategies that evaluate simplified screening protocols and training of non-physicians hold promise for more wide spread deployment of HAND-based protocols.

Outcomes of heart transplantation in small children bridged with ventricular assist devices.

BACKGROUND: Ventricular assist devices (VADs) have been used with increasing frequency to bridge small children to heart transplantation (HTx), but outcomes in large cohorts are not well established. METHODS: Small children (≤ 10 kg) bridged to HTx with VADs between 2004 and 2010 were identified in the United Network for Organ Sharing database. Survival was modeled using the Kaplan-Meier method, and 2:1 propensity matching was used to compare outcomes with a well-matched control cohort of nonbridged HTx recipients. RESULTS: Of the 803 small children who underwent HTx during the study period, 59 (7%) were bridged with a VAD. The proportion of recipients that were bridged with a VAD increased from 3% in 2004 to 9% in 2010 (p = 0.03). Kaplan-Meier 30-day, 6-month, and 1-year survival was comparable between those bridged with a VAD and 118 well-matched nonbridged children. Rates of postoperative renal failure, reoperation, infection, and rejection were also comparable. Those bridged with a VAD had a significantly higher rate of postoperative stroke (8.5% vs 0.9%; p = 0.008). CONCLUSIONS: Small children bridged to HTx with a VAD have early survival rates that are comparable to nonbridged children; however, this is achieved at the expense of a higher rate of stroke. Identifying the risk factors for early death and stroke in small children bridged to HTx with VADs is prudent as more experience with this patient population accumulates.

World Heart Federation criteria for echocardiographic diagnosis of rheumatic heart disease--an evidence-based guideline.

Over the past 5 years, the advent of echocardiographic screening for rheumatic heart disease (RHD) has revealed a higher RHD burden than previously thought. In light of this global experience, the development of new international echocardiographic guidelines that address the full spectrum of the rheumatic disease process is opportune. Systematic differences in the reporting of and diagnostic approach to RHD exist, reflecting differences in local experience and disease patterns. The World Heart Federation echocardiographic criteria for RHD have, therefore, been developed and are formulated on the basis of the best available evidence. Three categories are defined on the basis of assessment by 2D, continuous-wave, and color-Doppler echocardiography: 'definite RHD', 'borderline RHD', and 'normal'. Four subcategories of 'definite RHD' and three subcategories of 'borderline RHD' exist, to reflect the various disease patterns. The morphological features of RHD and the criteria for pathological mitral and aortic regurgitation are also defined. The criteria are modified for those aged over 20 years on the basis of the available evidence. The standardized criteria aim to permit rapid and consistent identification of individuals with RHD without a clear history of acute rheumatic fever and hence allow enrollment into secondary prophylaxis programs. However, important unanswered questions remain about the importance of subclinical disease (borderline or definite RHD on echocardiography without a clinical pathological murmur), and about the practicalities of implementing screening programs. These standardized criteria will help enable new studies to be designed to evaluate the role of echocardiographic screening in RHD control.

Decline in rejection in the first year after pediatric cardiac transplantation: a multi-institutional study.

BACKGROUND: Rejection is a major cause of morbidity and mortality after pediatric heart transplantation (HTx). Survival after pediatric HTx has improved over time, but whether there has been an era-related improvement in the occurrence of allograft rejection is unknown. METHODS: The Pediatric Heart Transplant Study (PHTS) database was queried for patients who underwent HTx from January 1993 to December 2005 to determine the incidence of rejection and identify factors associated with the first episode of rejection in the first year after HTx. RESULTS: Data were reviewed in 1,852 patients from 36 centers. The incidence of rejection declined over 13 years at a rate of -2.58 +/- 0.41 (p < 0.001) from approximately 60% to 40% (p < 0.001). The mean number of episodes of rejection also significantly fell at a rate of -0.05 +/- 0.01 per patient/year from 1.19 to 0.66 (p < 0.001). The incidence of rejection with hemodynamic compromise and death from rejection did not change. Multivariate analysis for the risk of a first rejection episode demonstrated decreased risk of rejection with later year of HTx (odds ratio [OR], 0.88; 95% confidence interval [CI], 0.85-0.91; p < 0.001) and use of mechanical support (OR, 0.65; 95% CI, 0.42-0.99; p = 0.046). Increased risk of rejection was associated with positive donor-specific crossmatch (OR, 1.85; 95% CI, 1.18-2.88; p = 0.007) and older recipient age (OR, 1.05; 95% CI, 1.02-1.07; p < 0.001). CONCLUSIONS: Although the overall incidence and prevalence of rejection has substantially decreased over time in pediatric HTx recipients in the first year after HTx, the rate of rejection with hemodynamic compromise or death from rejection remains unchanged.

ABO-incompatible heart transplantation in infants: analysis of the united network for organ sharing database.

INTRODUCTION: ABO-incompatible heart transplantation, traditionally contraindicated because of the risk of hyperacute rejection, has been used selectively in recent years. Infants have limited production of isohemagglutinins, which may lower the risk of hyperacute rejection. A large national database was used to analyze the effect of ABO incompatibility on outcomes after heart transplantation in infants. METHODS: Heart transplant recipients aged younger than 1 year reported to the United Network for Organ Sharing from 1999 to 2007 were divided according to donor-recipient ABO incompatibility or compatibility. Outcomes included Kaplan-Meier survival and hyperacute rejection. Propensity-adjusted Cox regression modeling was used to identify predictors of mortality. RESULTS: Of 591 infants that underwent heart transplantation, 35 (6%) received allografts from ABO-incompatible donors. ABO-incompatible recipients trended toward more congenital heart disease (71% vs 66%; p = 0.06) and were less likely to have dilated cardiomyopathy (11% vs 29%; p = 0.02). One ABO-incompatible infant had hyperacute rejection requiring retransplantation. No ABO-incompatible infant and 2 ABO-compatible infants died from hyperacute rejection. Survival was similar at 3 years. Propensity-adjusted Cox regression analysis demonstrated that ABO-incompatibility did not predict mortality (hazard ratio, 3.61; 95% confidence interval, 0.26-49.0; p = 0.33). CONCLUSION: ABO-incompatible heart transplantation can be performed safely in infants without greater incidence of hyperacute rejection. ABO-incompatible heart transplantation should be strongly considered in infants to maximize donor organ utilization and reduce waiting-list mortality.