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Despite recent data suggesting improved outcomes with bivalirudin vs heparin in pediatric Ventricular assist devices (VAD), higher costs remain a barrier. This study quantified trends in bivalirudin use and compared outcomes, resource utilization, and cost-effectiveness associated with bivalirudin vs heparin.Children age 0 to 6 year who received VAD from 2009 to 2021 were identified in Pediatric Health Information System. Bivalirudin use was evaluated using trend analysis and outcomes were compared using Fine-Gray subdistrubtion hazard ratios (SHR). Daily-level hospital costs were compared due to differences in length of stay. Cost-effectiveness was evaluated using incremental cost-effectiveness ratio (ICER).Of 691 pediatric VAD recipients (median age 1 year, IQR 0-2), 304 (44%) received bivalirudin with 90% receiving bivalirudin in 2021 (trend p-value0.01). Bivalirudin had lower hospital mortality (26% vs 32%; adjusted SHR 0.57, 95% CI 0.40-0.83) driven by lower VAD mortality (20% vs 27%; adjusted SHR 0.46, 95% CI 0.32-0.77) after adjusting for year, age, diagnosis, and center VAD volume. Post-VAD length of stay was longer for bivalirudin than heparin (median 91 vs 64 days, respectively, p0.001). Median daily-level costs were lower among bivalirudin (cost ratio 0.87, 95% CI 0.79-0.96) with higher pharmacy costs offset by lower imaging, laboratory, supply, and room/board costs. Estimated ICER for bivalirudin vs heparin was $61,192 per quality-adjusted life year gained with a range of $27,673 to $131,243.Bivalirudin use significantly increased over the past decade and is now used in 90% young pediatric VAD recipients. Bivalirudin was associated with significantly lower hospital mortality and an ICER$65,000, making it a cost-effective therapy for pediatric VAD recipients.

The existence of a "weekend effect" in heart transplantation (HTx) is understudied. The present study sought to determine whether the odds of (HTx) offer acceptance differed for adult and pediatric candidates depending upon the day on which the offer occurred.United Network for Organ Sharing data were used to identify all HTx offers to adult (listing age ≥18) and pediatric candidates from 2000-2019. Odds of offer acceptance were studied, comparing weekends, holidays, and conferences (Society of Thoracic Surgeons [STS], American Association for Thoracic Surgery [AATS], International Society for Heart and Lung Transplantation [ISHLT]) to "baseline" (all other days). Multivariable binary logistic regression analyses were performed to determine independent predictors of offer nonacceptance, controlling for the impacts of program transplant volume, region, and candidate characteristics.A total of 323,953 offers occurred - 298,405 to adults and 25,548 to pediatric candidates. Clinically significant differences did not exist in donor or candidate characteristics between baseline or other events. The number of offers per day was stable throughout the year for both adults (p = 0.191) and pediatrics (p = 0.976). In adults, independently lower odds of acceptance existed on weekends (OR 0.88 [95% CI 0.84-0.92]), conferences in aggregate (0.86 [0.77-0.95]), and holidays in aggregate (0.81 [0.72-0.91]). In children, independently lower odds of acceptance were seen on weekends (0.88 [0.79-0.98]), during STS (0.46 [0.25-0.83], and during Christmas (0.32 [0.14-0.76]).The day on which a HTx offer occurs significantly impacts its likelihood of acceptance. Further work can determine the impacts of human behavior or resource distribution, but knowledge of this phenomenon can inform efforts to ensure ideal organ allocation throughout the year.

Donor-to-recipient size matching for heart transplantation typically involves comparing donor and recipient body weight; however, weight is not linearly related to cardiac size. Attention has shifted toward the use of computed tomography- (CT-) derived total cardiac volume (TCV), that is, CT-TCV, to compare donor and recipient heart organ size. At this time, TCV size matching is near impossible for most centers due to logistical limitations. To overcome this impediment, echocardiogram-derived TCV (ECHO-TCV) is an attractive, alternative option to estimate CT-TCV. The goal of this study is to test whether ECHO-TCV is an accurate and reliable surrogate for TCV measurement compared with the gold standard CT-TCV.ECHO-TCV and CT-TCV were measured in a cohort spanning the neonatal to young adult age range with the intention to simulate the pediatric heart transplant donor pool. ECHO-TCV was measured using a modified Simpson's summation-of-discs method from the apical 4-chamber (A4C) view. The gold standard of CT-TCV was measured from CT scans using three-dimensional reconstruction software. The relationship between ECHO-TCV and CT-TCV was evaluated and compared with other anthropometric and image-based markers that may predict CT-TCV. Inter-rater reliability of ECHO-TCV was tested among 4 independent observers. Subanalyses were performed to identify imaging views and timing that enable greater accuracy of ECHO-TCV.Banked imaging data of 136 subjects with both echocardiogram and CT were identified. ECHO-TCV demonstrated a linear relationship to CT-TCV with a Pearson correlation coefficient of r = 0.96 (95% CI, 0.95-0.97; P .0001) and mean absolute percent error of 8.6%. ECHO-TCV correlated most strongly with CT-TCV in the subset of subjects4 years of age (n = 33; r = 0.98; 95% CI, 0.96-0.99; P .0001). The single-score intraclass correlation coefficient across all 4 raters is 0.96 (interquartile range, 0.93-0.98). ECHO-TCV measured from a standard A4C view at end diastole with the atria in the plane of view had the strongest correlation to CT-TCV.ECHO-TCV by the A4C view was found to be both an accurate and reliable alternative measurement of CT-TCV and is derived from readily available donor ECHO images. The ECHO-TCV findings in this study make the ECHO method an attractive means of direct donor-to-recipient TCV size matching in pediatric heart transplantation.

Background: Patient- and proxy-reported outcomes (PROs) are an important indicator of healthcare quality and can be used to inform treatment. Despite the widescale use of PROs in adult cardiology, they are underutilised in paediatric cardiac care. This study describes a six-center feasibility and pilot experience implementing PROs in the paediatric and young adult ventricular assist device population. Methods: The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) is a collaborative learning network comprised of 55 centres focused on improving clinical outcomes and the patient/family experience for children with heart failure and those supported by ventricular assist devices. The development of ACTION’s PRO programme via engagement with patient and parent stakeholders is described. Pilot feasibility, patient/parent and clinician feedback, and initial PRO findings of patients and families receiving paediatric ventricular assist support across six centres are detailed. Results: Thirty of the thirty-five eligible patients (85.7%) were enrolled in the PRO programme during the pilot study period. Clinicians and participating patients/parents reported positive experiences with the PRO pilot programme. The most common symptoms reported by patients/parents in the first month post-implant period included limitations in activities, dressing change distress, and post-operative pain. Poor sleep, dressing change distress, sadness, and fatigue were the most common symptoms endorsed >30 days post-implant. Parental sadness and worry were notable throughout the entirety of the post-implant experience. Conclusions: This multi-center ACTION learning network-based PRO programme demonstrated initial success in this six-center pilot study experience and yields important next steps for larger-scale PRO collection, research, and clinical intervention.

Ventricular assist devices (VADs) increase waitlist survival, yet the risk of stroke remains notable. The purpose of this study was to analyze how strokes on VAD support impact post-transplant (post-Tx) outcomes in children.About 520 pediatric (18 years) heart transplant candidates listed from January 2011 to April 2018 with a VAD implant date were matched between the United Network of Organ Sharing and Pediatric Health Information System databases. Patients were divided into pre-Tx Stroke and No Stroke cohorts.About 81% of the 520 patients were transplanted; 28% (n = 146) had a pre-Tx Stroke; and 59% (n = 89) of the Stroke patients were transplanted at a median of 57 (IQR 17-102) days from stroke. Significantly more No Stroke cohort (90%) were transplanted (p 0.001). There was no difference in post-Tx survival between the Stroke and No Stroke cohorts (p = 0.440). Time between stroke and transplant for patients who died within 1 year of transplant was 32.0 days (median) compared to 60.5 days for those alive1 year (p = 0.18). Regarding patients in whom time from stroke to transplant was more than 60 days, one-year survival of Stroke vs. No Stroke patients was 96% vs. 95% (p = 0.811), respectively.Patients with stroke during VAD support, once transplanted, enjoy similar survival compared to No Stroke patients. We hypothesize that allowing Stroke patients more time to recover could improve post-Tx outcomes. Unfortunately, the ideal duration of time between stroke and safe transplantation could not be determined and will require more detailed and larger studies in the future.

Elevated central venous pressure in those with Fontan circulation causes liver congestion and hepatomegaly. We assessed if liver volume by magnetic resonance imaging (MRI) is associated with adverse cardiovascular outcomes. Retrospective study of 122 patients with Fontan circulation who were10 years old and had a liver MRI with magnetic resonance elastography. Liver volume (ml) was measured by manual segmentation from axial T2-weighted images and was indexed to body surface area. The composite outcome included death, heart transplant, ventricular assist device placement, or nonelective cardiovascular hospitalization. The median age at the time of MRI was 18.9 (interquartile range 15.8 to 25.9) years, and 47% of the patients were women. The mean indexed liver volume was 1,133 ± 180 ml/m

The SynCardia temporary total artificial heart (TAH-t) is a pneumatically driven pulsatile device that replaces a patient's ventricles and all four valves. It is now available in two sizes and can be used in patients with sufficient chest size as a bridge to transplant or destination therapy. We describe our experience at a children's hospital with the TAH-t. Seven patients implanted with a TAH-t at Cincinnati Children's Hospital Medical Center from November 2012 to March 2021 were included. Duration of support ranged from 10 to 414 days with a median of 27 days. There was a trend toward decreased time to extubation and intensive care unit (ICU) length of stay, with the three most recent patients being extubated on postoperative day 1. Seventy-one percent of the patients were successfully transplanted following their TAH-t placement. Children and young adults with transplant graft failure requiring durable, biventricular support with a TAH-t had superior outcomes (75% survival to retransplant) than previously described.

Individuals with Fontan circulation are at risk of late mortality from both cardiac and noncardiac causes. Despite the known risk of mortality, referral indications for advanced heart failure care vary between centers, and many individuals die from Fontan circulation-related complications either after late consideration for advanced heart failure therapies or having never seen a heart failure specialist. There is a critical need for guidelines to direct appropriately timed referral for advanced heart failure consultation. The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) Fontan Committee has developed recommended thresholds for advanced heart failure referral to guide primary cardiologists. These recommendations are divided into 4 categories of clinical Fontan circulatory dysfunction including (1) cardiac/systemic ventricular dysfunction, (2) Fontan pathway dysfunction, (3) lymphatic dysfunction, and (4) extracardiac dysfunction.

We previously reported the first successful implantation of the Heartmate 3TM in a Fontan patient. We now report his successful transplantation after 1,104 days of support, the longest reported bridge to transplantation of a Fontan patient. We describe our operative technique complicated by not only the Fontan anatomy and ventricular assist device (VAD) explanation but also a >10cm ascending and aortic arch aneurysm. Additionally, the post-transplant hemodynamics of this patient appear to demonstrate that effective VAD support may induce reversal of chronic effects of the failing Fontan circulation, which in this case was the elimination of his aorto-pulmonary collateral burden.

With improvement in early surgical outcomes in patients with complex congenital heart disease, most patients are now expected to survive to adulthood. As adult congenital heart disease (ACHD) patients age, they are at risk of heart failure, which has become the leading cause of mortality in ACHD. Some who develop advanced heart failure may not be candidates for transplant, and chronic ventricular assist device (VAD) therapy may be the only means of survival. There is limited experience with chronic VAD therapy in ACHD patients, and the outcomes are not well delineated. We describe our center's experience with chronic VAD therapy in ACHD patients receiving care exclusively within our children's hospital.

As women age, they typically experience a progressive decrease in skeletal muscle mass and strength, which can lead to a decline in functional fitness and quality of life. Resistance training (RT) has the potential to attenuate these losses. Although well established for men, evidence regarding the benefits of RT for women is sparse and inconsistent: prior reviews include too few studies with women and do not adequately examine the interactive or additive impacts of workload, modalities, and nutritional supplements on outcomes such as muscle mass (MM), body composition (BC), muscle strength (MS), and functional fitness (FF). The purpose of this review is to identify these gaps. Thirty-eight papers published between 2010 and 2020 (in English) represent 2519 subjects (mean age = 66.89 ± 4.91 years). Intervention averages include 2 to 3 × 50 min sessions across 15 weeks with 7 exercises per session and 11 repetitions per set. Twelve studies (32%) examined the impact of RT plus dietary manipulation. MM, MS, and FF showed positive changes after RT. Adding RT to fitness regimens for peri- to postmenopausal women is likely to have positive benefits.

Mechanical ventilation prior to pediatric heart transplantation predicts inferior post-transplant survival, but the impact of ventilation duration on survival is unclear.Data from the United Network for Organ Sharing and Pediatric Health Information System were used to identify pediatric (18 years) heart transplant recipients from 2003 to 2020. Patients ventilated pretransplant were first compared to no ventilation, then ventilation durations were compared across quartiles of ventilation (≤1 week, 8 days-5 weeks,5 weeks).At transplant, 11% (511/4506) of patients required ventilation. Ventilated patients were younger, had more congenital heart disease, more urgent listing-status, and greater rates of nephropathy, TPN-dependence, and inotrope and ECMO requirements (p .001 for all). Post-transplant, previously ventilated patients experienced longer ventilation durations, ICU and hospital stays, and inferior survival (all p .001). Hospital outcomes and survival worsened with longer pretransplant ventilation. One-year and overall survival were similar between the no-ventilation and ≤1 week groups (p = .703p = .433, respectively) but were significantly worse for ventilation durations1 week (p .001). On multivariable analysis, ventilation ≤1 week did not predict mortality (HR 0.98 [95% CI 0.85-1.43]), whereas ventilation1 week did (HR: 1.18 [1.01-1.39]).Longer pretransplant ventilation portends worse outcomes, although only ventilation1 week predicts mortality. These findings can inform pretransplant prognostication.

Objectives: Six months after withdrawal of the HeartWare HVAD System (HVAD; Medtronic) from sale, approximately 4000 patients continue ongoing support with this device. In light of the diminishing experience, this global consensus document summarizes key management recommendations. Methods: International experts with experience in the management of patients with ongoing HVAD support were invited to summarize key aspects of patient and pump management and highlight differences in the current HeartMate 3 (Abbott Laboratories) ventricular assist device. Clinicians from high-implanting HVAD sites reviewed current literature and reported experience to generate a consensus statement. Results: Specific guidelines to assist in the management of ongoing HVAD patients are developed. Key management protocols and helpful techniques developed from experienced clinicians are combined into a short guideline document. As experience with HeartMate 3 increases, key differences in approach to management are highlighted, where appropriate. Conclusions: With decreasing worldwide experience in the ongoing management of HVAD-supported patients, this consensus guideline provides a summary of best practice techniques from international centers. Differences in HeartMate 3 management are highlighted.

Both gender- and weight-matching between donor and recipient are thought to impact survival in pediatric heart transplantation, with clinical dogma holding that male donor hearts and "ideal" weight-matching yield superior survival. The composite impacts of gender and weight on post-transplant survival (PTS) are understudied.All pediatric (age18) heart recipients between 1989 and 2021 with the complete recipient and donor gender and weight data were identified in the United Network for Organ Sharing database. Patients were grouped by recipient-donor gender (MF) and donor-to-recipient weight ratio (DRWR; undersized [0.8], ideal-sized [0.8-1.5], oversized [1.5]).A total of 10 697 patients were identified. Among male recipients, PTS was greatest with oversized DRWR from either male or female donors (median 22.4 and 20.6 years; p .001 vs. others) and lowest for undersized DRWR from either male or female donors (median 13.4 and 13.2 years; p .001 vs. others). The majority (64%) of male recipients received ideal-sized DRWR, among which female donor hearts yielded superior survival to males (median 18.9 vs. 17.4 years, p = .014). No differences in PTS existed for female recipients on the basis of gender-match, DRWR, and gender/DRWR together (all p .1).When considered together, gender and DRWR pairings impact PTS in male-but not female-pediatric heart transplant recipients. For males receiving ideal-sized DRWR organs (most common pairing,60%), male recipients achieve superior survival when female donor hearts are transplanted. These findings suggest that if weight is being used for size-matching, donor gender should also be considered, particularly for male recipients.

Total cardiac volume (TCV)-based size matching for heart transplantation offers individualization in size matching that increases the number of suitable donors. Here we describe our clinical protocol for using TCV to determine an acceptable donor weight range for heart transplant candidates. We compare candidate imaging-derived TCV to a nomogram of subjects with normal TCV to determine a precise maximum donor weight at the time of listing. For nearly half of our transplant patients, we have increased weight range by an average of 70% with no oversizing related adverse events, such as delayed chest closure to avoid tamponade or bronchial compression. Widespread adoption of TCV-based size matching can lead to a more efficient heart allocation system by the data-driven bypass of poor size matches.

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.

The number of Fontan patients with circulatory failure and systolic dysfunction is growing rapidly. The last decade has demonstrated that ventricular assist device (VAD) is an effective therapy in properly selected patients. Herein, we discuss the current approach to patient selection, implantation, and patient management.

While the use of sports supplements is common among athletes and active adults, the marketing strategies used by these sports supplement manufacturers remain unknown—especially relative to active and athletic women. This descriptive study aims to describe marketing strategies used, such as color choices, structure function claims, and picture choice, and analyze if there are gender-specific differences within the strategies. A total of 100 best-selling sports supplements were selected from three separate third-party websites. Food products, accessories, and kitchen appliances were excluded. Supplement main webpages and sports supplement-specific webpages were explored to determine which marketing strategies were used and if there were gender-specific differences. Of 169 sports supplement webpages reviewed, only 23.1% (n=39) included pictures that were only/majority female. Only 7.1% (n=12) of supplement bottles used feminine colors, while for supplement pages, only 8.3% (n=14) utilized feminine colors and 43.2% (n=73) used masculine colors. While the number of women athletes continues to grow, sports supplement manufacturers are not using strategies targeted to women. Future research should examine the reasons for this discrepancy and women’s perceptions of purchasing sports supplements targeted to men.

Pediatric mechanical circulatory support (MCS) has been successfully used to bridge numerous children to transplantation who otherwise would have been unlikely to survive on the waitlist and, in many cases, make them better transplant candidates. The purpose of this study was to analyze what the pediatric heart failure community has achieved over the last 15 years in reaching 1,200 cases of bridging children to heart transplantation. The United Network for Organ Sharing database was used to identify MCS patients ages 0-18 at the time of listing for heart transplantation between 2005 and 2019, divided into three eras: first (2005-2009), second (2010-2014), and third (2015-2019). From 2005 to 2019, 1,289 pediatric cases were identified. More patients were successfully bridged to transplantation with MCS in the third-era (28%) [vs. first-era (16%), second-era (24%), p ≤ 0.004]. The proportion of discharges on ventricular assist device has increased as well from 3% to 22% (p0.001). Post-transplant survival was significantly better in the third era (1-year survival: 96%; 3-year survival: 89%) compared to the two previous eras (p = 0.006). On MCS, renal dysfunction, ventilator dependence, inotrope use, and functional status improved from the time of listing to transplantation (p0.01). Hepatic dysfunction (p0.001), renal dysfunction (p = 0.004), congenital heart disease (p = 0.023), and infant age (p = 0.002) were risk factors for post-transplant mortality. Over the last 15 years, pediatric MCS has become an accepted and increasingly used strategy for bridging children to transplantation. MCS therapy is associated with improved end-organ function at the time of transplantation, perhaps contributing to the increasing post-transplantation survival of patients bridged with MCS.

Arabidopsis thaliana has a wide elevational range and much of its diversity may be associated with local adaptation to elevation. We took a multi-regional view of the genomics and physiology of elevational adaptation in Arabidopsis, with >200 ecotypes, including 17 newly collected from Africa. We measured plant responses to potential high elevation stressors: low pCO2, high light, and night freezing, and conducted genome-wide association studies (GWAS). We found evidence of an adaptive cline in the western Mediterranean with low δ13C/early flowering at low elevations to high δ13C/late flowering at high elevations. By contrast, central Asian high elevation ecotypes flowered earlier. Antioxidants and pigmentation under high light and freezing showed regional differentiation but not elevational clines and may be associated with maladaptive plasticity. We found natural variation in non-photochemical quenching (NPQ) kinetics in response to chilling and fluctuating light, though with an unclear role in local adaptation. There were several candidate genetic loci mapped, including the ascorbate transporter PHT4;4 (associated with antioxidants) that influences the xanthophyll cycle, and may be involved in local adaptation to Morocco. Our study shows how the ecological strategies and genetic loci causing local adaptation to elevation change across regions and contribute to diversity in Arabidopsis.

Background Portal hypertension in the Fontan circulation is a function of elevated systemic venous pressure and liver fibrosis. Purpose To quantify the prevalence of radiologic evidence of portal hypertension and elevated VAST score (one point each for varices, ascites, splenomegaly, and thrombocytopenia) of 2 or greater in children and adults with Fontan circulation and to determine the association with hemodynamics and adverse outcomes. Materials and Methods This was a retrospective study of individuals with Fontan circulation who underwent abdominal MRI or CT for focal liver lesion surveillance between January 2012 and December 2019. Portal hypertension was defined as the presence of at least two of the following: varices, ascites, or splenomegaly. Fontan deterioration was defined as a composite of heart failure signs or symptoms requiring diuretic escalation, placement of a ventricular assist device, heart transplant, or death. Relationships between variables and the composite end point were assessed using univariable and multivariable logistic regression. Results A total of 123 patients (age range, 9-55 years; 32 children) were evaluated (median age, 23 years; IQR, 17-30 years; 63 male patients). Median time since diagnosis of Fontan circulation was 16 years (IQR, 12-23 years). Twenty-five of the 123 patients (20%) had radiologic evidence of portal hypertension, and 34 (28%) had a VAST score of 2 or greater. Fontan deterioration occurred in 25 of the 123 patients (20%); median follow-up duration was 0.4 year (IQR, 0.1-3.1 years). Compared with patients who had Fontan circulation without deterioration, patients with Fontan deterioration were more likely to have moderate or severe ventricular systolic dysfunction (

Objectives: In this study, we aimed to determine the influence of aesthetics and color of printed nutrition education materials on perceived credibility of the material content. Methods: A randomized 2 x 2 (aesthetics and color) factorial experimental design was completed on a university campus. Undergraduate and graduate students (N=204) were randomly assigned one of 4 types of flyers (high aesthetic-color, high aesthetic-black-and-white, low aesthetic-color, and low aesthetic-black-and-white). Perceptions on the flyer content (accurate, believable, biased, valuable, and trustworthy), perceptions of overall flyer quality (attractive, pleasant, confusing, and interesting), and knowledge of the content within the flyer were assessed via a survey after reading the flyer. Results: A statistically significant main effect of aesthetics was observed for perceived "trustworthiness" of the flyer information (p = .048). Flyers with high aesthetics, regardless of print color, had a higher mean score of "trustworthiness" (M = 6.01) than flyers with low aesthetics (M = 5.71). An interaction effect was seen for perception of the flyer being "confusing" (p = .02). The high aesthetic-black-and-white flyer had the highest mean score for "confusing" (M = 1.66) with the low aesthetic flyer printed in color having the second highest mean score for "confusing" (M = 1.56). Conclusions: The aesthetics of nutrition education materials appear to influence perceived trustworthiness of those materials.

Heart failure metrics specific to the pediatric population are required to successfully implement quality improvement initiatives in children with heart failure. Medication use at the time of discharge following admission for decompensated heart failure has been identified as a potential quality metric in this population. This study aimed to report medication use at discharge in the current era for children admitted with acute decompensated heart failure. All patients

BACKGROUND The HeartMate 3 ventricular assist device (VAD) is a newer centrifugal continuous-flow VAD used for bridge-to-transplant and destination therapy in adults. However, there is limited experience regarding its use in children and adults with complex congenital heart disease (CHD). METHODS The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) is a multicenter learning network comprised of pediatric hospitals implanting VADs in children and adults with complex CHD. We examined the outcomes of patients undergoing HeartMate 3 implantation at an ACTION center between December 2017 and September 2019. RESULTS The HeartMate 3 was implanted in 35 patients at 9 ACTION centers, with a median age of 15.7 (8.8–47.3) years, median weight of 65.7 (19.1–114.1) kg, and median body surface area (BSA) of 1.74 (0.78–2.36) m2. Of the cohort, 14 patients (40%) weighed CONCLUSIONS Use of the HeartMate 3 in ACTION centers was associated with a low incidence of mortality and adverse events. Patients as small as 19 kg (BSA 0.78 m2) were successfully implanted and supported, indicating that this device may be appropriate for older children and small adults.

Ventricular assist devices (VADs) have become a mainstay of advanced heart failure therapy in pediatrics, with outcomes in adolescents that parallel the excellent outcomes in adults. Unfortunately, outcomes for younger children and infants remain suboptimal. This review discusses the patient and device specifics that contribute to this discrepant outcome, highlight current treatment strategies, and suggest areas for future improvement. The field of pediatric mechanical circulatory support (MCS) continues to be driven by advancements in patient selection, timing of VAD implantation, and patient management strategies particularly with regard to anticoagulation. The use of direct thrombin inhibitors, such as bivalirudin, and weight-based antiplatelet therapy has shown promise in reducing the incidence of bleeding and stroke. While the number of devices remains limited, providers continue to leverage current technology and novel cannulation strategies to support smaller and more complex patients. Despite the challenges of supporting infants and small children, it remains a hopeful time for the field of pediatric MCS. While individual centers may only care for small number of VAD patients, the ACTION learning network has sparked collaboration across the field broadly with rapid dissemination of best practices to all centers. This collaboration has led to many advances and holds promise for driving future advancements.

Pediatric cardiomyopathies can be caused by variants in genes encoding the sarcomere and cytoskeleton in cardiomyocytes. Variants are typically inherited in an autosomal dominant manner with variable expressivity. De novo variants have been reported, however their overall frequency is largely unknown. We sought to determine the rate of de novo, pathogenic and likely pathogenic (P/LP) variants in children with a diagnosis of hypertrophic, dilated, or restrictive cardiomyopathy (HCM, DCM, or RCM), and to compare disease outcomes between individuals with and without a de novo variant. A retrospective record review identified 126 individuals with HCM (55%), DCM (37%), or RCM (8%) ≤18 years of age who had genetic testing. Overall, 50 (40%) had positive genetic testing and 18% of P/LP variants occurred de novo. The rate of de novo variation in those with RCM (80%) was higher than in those with HCM (9%) or DCM (20%). There was evidence of germline mosaicism in one family with RCM. Individuals with de novo variants were more likely than those without to have a history of arrhythmia (p = .049), sudden cardiac arrest (p = .024), hospitalization (p = .041), and cardiac transplantation (p = .030). The likelihood of de novo variation and impact on family risk and screening should be integrated into genetic counseling.

BACKGROUND: Studies have shown that the optimal ischemia time (IT) threshold in pediatric heart transplantation (PHT) is up to 4 h, independent of other donor organ factors. The purpose of this study was to examine the relationship between IT and donor left ventricular ejection fraction (LVEF) and study their impact on PHT outcomes. METHODS: This is a retrospective cohort study of PHT (6 h was performed using Kaplan–Meier curves. Cohort was divided according to donor LVEF median value, and survival was analyzed. Cox regression was performed. RESULTS: Median LVEF was 65% in the study cohort (6669 PHT). Overall, IT>6 h was associated with worse survival compared to 6 h (p = .315). For allografts with LVEF > 65%, IT = 4–6 h had similar survival compared with 6 h (p = .003). After adjusting for donor and recipient variables, Cox regression showed that IT = 4–6 h was not associated with increased mortality for LVEF ≥ 65%. CONCLUSIONS: The IT threshold of 4 h does not apply to all allografts. Recipients of hearts with LVEF≥65% can tolerate an IT up to 6 h without any detriment to survival. Routine acceptance of these donor hearts could mitigate longer waiting times and poor donor availability for many candidates.

Cardiac disease results in significant morbidity and mortality in patients with muscular dystrophy (MD). Single centers have reported their ventricular assist device (VAD) experience in specific MDs and in limited numbers. This study sought to describe the outcomes associated with VAD therapy in an unselected population across multiple centers.We examined outcomes of patients with MD and dilated cardiomyopathy implanted with a VAD at Advanced Cardiac Therapies Improving Outcomes Network (ACTION) centers from 9/2012 to 9/2020.A total of 19 VADs were implanted in 18 patients across 12 sites. The majority of patients had dystrophinopathy (66%) and the median age at implant was 17.2 years (range 11.7-29.5). Eleven patients were non-ambulatory (61%) and 6 (33%) were on respiratory support pre-VAD. Five (28%) patients were implanted as a bridge to transplant, 4 of whom survived to transplant. Of 13 patients implanted as bridge to decision or destination therapy, 77% were alive at 1 year and 69% at 2 years. The overall frequencies of positive outcome (transplanted or alive on device) at 1 year and 2 years were 84% and 78%, respectively. Two patients suffered a stroke, 2 developed sepsis, 1 required tracheostomy, and 1 experienced severe right heart failure requiring right-sided VAD.This study demonstrates the potential utility of VAD therapies in patients with muscular dystrophy. Further research is needed to further improve outcomes and better determine which patients may benefit most from VAD therapy in terms of survival and quality of life.

Ventricular assist devices (VADs) support children with heart failure. The objective is to report on the nutrition outcomes of children requiring VAD.This was a retrospective study performed in a tertiary care center. All patients undergoing VAD placement from 2010 to 2018 were included. Exclusion criteria were VAD placement in outside hospitals, missing baseline anthropometrics, and death in the first 15 days post-VAD. Clinical, demographic, and nutrition data were collected from baseline and at 2 months post-VAD. Descriptive statistics were used.Of the 52 patients who had undergone VAD placement, 49 (65% male, 80% with cardiomyopathy; median age at VAD, 8 years) met the study criteria. The median length of stay following VAD was 63 days. Eight patients (16%) had malnutrition at baseline (five mild, two moderate, and one severe). At 2 months post-VAD, seven (of 49) patients had undergone heart transplantation and three (6%) were deceased. The proportion of patients with malnutrition was significantly different at 2 months post-VAD (P = 0.009), with six patients showing evidence of malnutrition (three mild, two moderate, and one severe; body mass index z score at baseline vs 2 months: 0.11 (± 1.72) vs 0.43 (± 0.94), P = 0.049). The percent of required energy consumed increased from 77% at baseline to 90% at 2 months post-VAD (P = 0.021). The proportion of patients fed solely enterally also increased (62% vs 84%, respectively; P = 0.042).VADs are associated with improved nutrition outcomes. Future studies should investigate the impact of VADs on body composition and longer-term outcomes.

We sought to characterize strokes in children with ventricular assist devices. Of 407 patients in the ACTION registry (4/1/18-5/3/2021), 45 (11%) experienced 52 strokes (45 ischemic and 7 hemorrhagic). Median time to stroke was 23.5 days and 19/52 (37%) occurred ≤ 10 days. Stroke rate was 0.09 and 0.63 strokes per patient-year for implantable continuous and paracorporeal devices, respectively. Patients with stroke were younger, more likely to have congenital heart disease and have been on extracorporeal membrane oxygenation at time of VAD. Based on these data, ACTION is now focused on decreasing strokes in these higher-risk patients with particular attention to the peri-implant period.

Supplemental material, sj-docx-2-pch-10.1177_21501351211065360 for Highlights of the Sixteenth International Conference on Pediatric Mechanical Circulatory Support Systems and Pediatric Cardiopulmonary Perfusion by David L. S. Morales, Angela Lorts, Jason W. Greenberg, James A. Reagor and Akif ��ndar in World Journal for Pediatric and Congenital Heart Surgery

Supplemental material, sj-docx-1-pch-10.1177_21501351211065360 for Highlights of the Sixteenth International Conference on Pediatric Mechanical Circulatory Support Systems and Pediatric Cardiopulmonary Perfusion by David L. S. Morales, Angela Lorts, Jason W. Greenberg, James A. Reagor and Akif ��ndar in World Journal for Pediatric and Congenital Heart Surgery