Organizational culture is increasingly recognized as a determinant of team performance, patient safety, and clinician well-being, yet remains poorly characterized in pediatric cardiology. Existing approaches typically assess individual domains rather than culture as a multidimensional construct. We conducted a multinational exploratory study to examine physician perceptions of organizational culture and to develop an integrated conceptual framework. We performed a cross-sectional, anonymized survey of pediatric cardiology physicians across tertiary congenital heart programs internationally. The survey, informed by established constructs including the Safety Attitudes Questionnaire, assessed domains such as psychological safety, leadership, team structure, workload, and medicolegal climate. Analyses were descriptive and hypothesis-generating. A total of 133 physicians from 27 centers participated. Overall, 54.9

Biological sex is closely linked to patterns embedded within the electrocardiogram (ECG) with essential health and disease implications. We report multicenter verification of an AI-enabled ECG model to predict biological sex across pediatric development. A previously published Mayo Clinic model confirmed puberty-linked AUROC gradient during external validation at Texas Children’s Hospital (pre-puberty AUROC 0.64, peri-puberty AUROC 0.84, post-puberty AUROC 0.94). This phenomenon was replicated at Boston Children’s Hospital. Saliency mapping revealed established sex-related electrophysiologic patterns.

BACKGROUND:Patient-reported outcome measures (PROM) are used routinely in adult heart failure (HF) practice but not in pediatric (PHF) practice. Adult PROMs are often not applicable to pediatric patients whose manifestations and experience of living with HF differ from adults. We sought to understand the experience of adolescents living with HF to facilitate development of a PROM for PHF. METHODS AND RESULTS:Between 2019 and 2020, patients aged 12 to 21 years with recent HF and their caregivers were recruited at 2 children's hospitals for qualitative interviews or focus groups about their HF symptoms, physical functioning, and quality of life. Transcripts were analyzed using methods of directed content analysis. Twenty-one patients and 12 caregivers participated. Primary themes spanned 4 domains: (1) functional impairment (eg, inability to participate in activities of daily living); (2) social impact (eg, feeling different or isolated from others); (3) emotional impact (eg, anxiety, depression, frustration); (4) illness burden (eg, taking multiple medications, time burden of clinic visits and procedures). CONCLUSION:The health and social experiences of adolescents with HF differ from adults with HF and warrant development of an adolescent-specific PROM. Such a PROM would be useful to clinicians managing adolescents with HF and to regulators evaluating emerging medical products.

INTRODUCTION:Bicuspid aortic valve (BAV) is one of the most common congenital heart defects (CHDs). However, guidelines for imaging surveillance frequency are not well established among young children, particularly those diagnosed during infancy without hemodynamically significant associated congenital lesions. METHODS:The Mayo Clinic echocardiography database was retrospectively searched for infants (age <1 year) diagnosed with BAV from 2001 to 2019. We initially identified 307 patients, and of these, 204 (66%) patients with BAV and concomitant significant CHD were excluded. Therefore, 103 (34%) patients with isolated BAV without critical valvulopathy and/or BAV with simple CHD (atrial septal defects, muscular ventricular septal defects, and/or patent ductus arteriosus) were included for further analysis. RESULTS:Overall, 103 infants (68% male) with isolated BAV or BAV with simple CHD underwent their first echocardiogram at a median age of 8 days (interquartile range [IQR], 2-84 days; range, 0-349 days). A total of 92 (89%) subjects had at least 1 follow-up visit and most recent echocardiogram at a median age of 8.6 years (IQR, 3.7-13.6 years) with a total clinical follow-up of 791 patient-years. Bicuspid aortic valve was present as an isolated lesion in 43% (44/103), while concomitant simple CHD was present in 57% (59/103) of infants. The most common indication for an echocardiogram was presence of a murmur (68%, 70/103). The first echocardiogram demonstrated aortic regurgitation (AR) in 4% (4/92) and aortic stenosis (AS) in 30% (28/92). A total of 10 aortic valve procedures were performed in 8 patients (9%), with AS (P < .001) and ascending aortic Z score ≥2 (P = .006) on the index echocardiogram significantly associated with risk of aortic valve intervention. Among 61 infants without AS or AR on their first echocardiogram (61/92), none required any valvular intervention at a median follow-up age of 6.0 years (IQR, 2.1-10.8 years). Among 28 infants who had AS (any degree) on their first echocardiogram, 8 (29%) required valvular intervention by a median age of 12.4 years (IQR, 7.2-17.7 years) and all had at least moderate AS at the baseline echocardiogram. Aortic stenosis on the baseline echocardiogram was predictive of not only aortic valve intervention (P < .001) but also progression of AR (P = .007) and ascending aorta dilation (P = .0002) at last follow-up. Among 35 infants with aortopathy at their first echocardiogram, none required intervention on the aorta at a median age of 8.8 years (IQR, 4.0-14.2 years). Overall, 4 patients died, with no deaths related to cardiac disease. CONCLUSION:Infants with normally functioning BAV without severe CHD did not develop significant valvulopathy or aortopathy over the first few years of life and required no interventions on the aortic valve or aorta. Conversely, those with more than mild AS or ascending aortic dilation at first echocardiogram incurred higher risk of valvular intervention, valvulopathy, and aortopathy progression. Therefore, initial valve and aortic pathology should guide frequency of follow-up surveillance imaging.

Background DDX3X- syndrome is a rare neurodevelopmental disorder characterized by varying degrees of intellectual disability, predominantly affecting females. We present an institutional cohort supplemented by a systematic literature review, expanding the cardiovascular phenotype of DDX3X- syndrome. Methods We conducted a retrospective chart review of patients diagnosed with DDX3X -syndrome at Mayo Clinic. Additionally, we performed a systematic literature review to identify studies reporting cardiovascular abnormalities in patients with DDX3X -syndrome. Results A total of 200 patients with DDX3X -syndrome were analyzed, comprising 14 patients from our institutional cohort and 186 patients identified through a systematic review of 9 published studies. Our institutional cohort included 14 patients (12 females and 2 males) from 13 unrelated families diagnosed with DDX3X -syndrome, at a median age of 4.5 years (IQR 1.2–9.5). Echocardiogram was performed on nine patients, and cardiovascular abnormalities were found in 7 out of 9 patients who underwent echocardiography (78%), two of whom had major congenital heart defect (CHD) requiring surgical intervention. At the time of assessment, 13 individuals were still alive, while one had died at age six due to extracardiac complications. The systematic review included 9 studies involving 186 patients, of whom 32 (17.4%, 25 females and 7 males) had reported cardiovascular abnormalities, ranging from simple CHDs to more complex defects. Conclusion DDX3X -syndrome carries a significant cardiovascular burden, which is possibly higher than previously reported, including complex congenital heart disease requiring surgical repair. A thorough cardiovascular assessment, including an electrocardiogram and echocardiogram, should be universally recommended for all patients at the time of diagnosis.

OBJECTIVES:To compare the clinical profile and outcomes after heart failure (HF) hospitalization in adults with and without congenital heart disease. METHODS:Leveraging a national database of commercially insured and Medicare Advantage patients in the United States, this study included patients hospitalized for HF with adult congenital heart disease (ACHD+) and without adult congenital heart disease (ACHD-) between January 1, 2010, and December 31, 2021. The association of baseline characteristics with mortality, major adverse cardiac and cerebrovascular events (MACCE), and health resource utilization was examined using cox proportional hazard regressions. RESULTS:Of 287,616 unique HF admissions, 5805 (2%) were ACHD+ and 281,811 (98%) were ACHD-. Over a mean follow-up period of 1.98±2.04 years, ACHD+ patients had a lower risk of mortality (HR, 0.74; 95% CI, 0.69 to 0.80; P<.001), MACCE (HR, 0.93; 95% CI, 0.89 to 0.97; P=.002), and rehospitalization (HR, 0.91; 95% CI, 0.88 to 0.95; P<.001). One-third (32.6%) of ACHD+ patients experienced a MACCE during follow-up, most commonly due to atrial fibrillation (n=939; 16.1%), recurrent HF (n=696; 12.0%), stroke (n=398; 6.8%) or intracranial bleed (n=102; 1.8%), myocardial infarction (n=276; 4.8%), and cardiac arrest (n=176; 3.0%). CONCLUSION:Compared with the general HF population, ACHD patients had substantially lower mortality risk after HF hospitalization. Despite this, the risk of complications following HF hospitalization was high, reinforcing the importance of discharge planning and post-acute care for improving outcomes in ACHD HF patients.

Purpose of review To summarize important papers published in the pediatric heart transplantation in the last year. Recent findings While advances have been made for pediatric patients who need heart transplantation, considerable challenges remain. Donor availability remains a key challenge, and work to both increase the number of donors and the quality of donor organs is ongoing. The field of pediatric heart transplantation benefits from registries such as the Pediatric Heart Transplant Society (PHTS) and learning networks such as the Advanced Cardiac Therapies Improving Outcomes Network (ACTION), allowing for multisite collaboration to optimize survival both before and after transplant, and to share learning about complex cases. Psychosocial evaluations of potential recipients and families can be challenging, and a consensus framework is now available for these evaluations, to provide appropriate oversight and management of a scarce resource. Looking ahead, heart transplant specialists are anticipating advances in xenotransplantation, which has the potential to revolutionize current standards of care. Summary Donor availability and organ scarcity are the dominant challenges we face, with many recent impactful papers addressing this issue either directly or indirectly. It will be critical to maintain the forward progress made in addressing these challenges, while readying the field for big changes to come.

Fontan-associated liver disease (FALD) occurs in all patients who have undergone Fontan palliation for functional single ventricle congenital heart defects. While liver fibrosis is universal in patients who have undergone Fontan palliation, FALD may lead to more serious consequences including portal hypertension, cirrhosis, and hepatocellular carcinoma. Scientific studies of the pathophysiology and clinical management of FALD have been limited to date by the heterogeneous nature of the disease, relatively small population of patients with Fontan physiology, and inaccuracy of noninvasive staging tests. As survival after the Fontan procedure improves, the population of adults with Fontan physiology is growing, leading to more severe extracardiac complications related to the Fontan circulation and growing demand for heart and liver transplantation. The accurate evaluation, staging, and management of FALD comprises a clinical challenge which requires expert multidisciplinary input.

Background/Objectives: Pediatric heart failure (HF) is a complex clinical syndrome with diverse etiologies and presentations. Primary care providers (PCPs) are critical in early recognition, family education, and coordination of ongoing care in collaboration with pediatric cardiologists. This review provides a comprehensive guide for PCPs to understand the causes, symptoms, treatments, and longitudinal considerations for children with HF. Methods: We conducted a narrative review synthesizing published evidence and expert consensus guidelines, focusing on the clinical presentation, treatment options, and longitudinal care strategies in children with HF. Results: HF in children most commonly results from congenital heart disease and cardiomyopathy, but includes a wide range of etiologies. Presenting symptoms differ by age, with subtle signs often mistaken for common pediatric illnesses. Treatments range from disease-modifying medications to surgical and transplant therapies. Psychosocial support, mental health, and family-centered care are critical components of long-term management. Conclusions: PCPs are essential partners in the care of children with HF. A proactive, collaborative, and informed approach can improve quality of life, reduce hospitalizations, and support families navigating this challenging diagnosis.

INTRODUCTION:Improving organ preservation is essential for expanding the donor pool and enhancing outcomes for pediatric transplant recipients. This report presents three pediatric patients who received donor hearts preserved using the Organ Care System (OCS). All donor hearts were prepared, placed, and monitored on OCS according to standard protocols. Heart function and myocardial edema were visually assessed during monitoring. CASE REPORTS:Patient 1: A 17-year-old female patient with hypertrophic cardiomyopathy utilized OCS technology due to donor distance. She experienced an uncomplicated post-transplant course and was discharged on post-transplant day 10. Patient 2: A 14-year-old male patient with complex congenital heart disease, palliated with Fontan circulation, underwent orthotopic heart transplantation (OHT) with OCS support due to both the complexity of the procedure and donor heart distance. His post-transplant course was complicated by a revision of the ascending aorta anastomosis and prolonged milrinone infusion for diastolic dysfunction. He was discharged on post-transplant day 31. Patient 3: A 16-year-old male patient with hypoplastic left heart syndrome, also palliated with Fontan circulation, underwent OHT using OCS technology due to the complexity of the surgical procedure. His post-transplant recovery was unremarkable, and he was discharged on post-transplant day 15. CONCLUSIONS:All three pediatric patients demonstrated favorable short-term outcomes using OCS technology. The OCS system facilitated shorter ischemic times despite the need for extended transport distances or complex surgical procedures. However, further studies with larger patient cohorts and longer follow-up are needed to better understand the long-term impact of OCS on pediatric heart transplantation outcomes.

ABSTRACTBackgroundIn recent years, transcatheter implantation devices to restrict pulmonary arterial flow have emerged as a potential alternative to surgical pulmonary artery banding.Case PresentationA term male was diagnosed with critical aortic stenosis (AS) and severely reduced left ventricle (LV) systolic function. He underwent aortic balloon valvuloplasty on day 2 of life, resulting in some antegrade flow, but LV ejection fraction only improved to 15%. He remained ductal dependent for systemic perfusion. Pulmonary over‐circulation ensued with systemic steal evident in his second week of life. On day 14, he underwent a transcatheter stage 1 procedure including the placement of bilateral pulmonary flow restrictor (PFR) devices and ductal stenting. Post‐procedure, he improved clinically, weaned off respiratory support, and transitioned to oral feeds while awaiting cardiac transplant.Twelve days later, he received a donor heart, with an uneventful recovery. He is now 3 years post‐transplant and has excellent graft function, and his transplant course has been unremarkable.ConclusionTranscatheter‐based palliation for a neonate with critical AS, utilizing endoluminal PFRs, proved effective in stabilizing the infant and successfully bridging him to transplant. This highlights an opportunity for the utilization of PFRs in neonatal conditions where control of pulmonary blood flow is imperative as a bridge to transplantation.

Ventricular assist devices (VADs) are being increasingly used in pediatric patients as a bridge to heart transplant (HTx ) candidacy, significantly reducing waitlist mortality. We report a case of an 8-year-old girl with high-grade osteosarcoma who developed anthracycline-induced cardiomyopathy (AC), leading to acute systolic heart failure. Due to persistent dual-inotropic dependence, a HeartWare™ left ventricular assist device (HVAD) was placed. Complicating her course, she experienced a local recurrence of her malignancy, necessitating further chemotherapy. While on anticoagulation for her HVAD and due to thrombocytopenia, she developed refractory epistaxis which required discontinuation of anticoagulant and antiplatelet agents for over 110 days. After 21 months of remission, she was listed status 1A for HTx and successfully received a transplant after a 3-month wait. Our experience suggests that with careful clinical and laboratory monitoring, durable HVAD support can be maintained in certain critical situations, without the use of anticoagulant or antiplatelet agents. This case underscores the importance of a multidisciplinary team approach, along with enhanced patient and family education, to minimize complications and optimize outcomes. We also highlight the evolving antithrombotic strategies in VAD patients, with Antiplatelet Removal and Hemocompatibility Events with a HeartMate 3 (ARIES-HM3) trial supporting aspirin avoidance; however, applying these results to other VADs warrants considerable caution.

BACKGROUND:Advancements in organ transplant technology are pushing global transplant capabilities to new heights. However, challenges persist in pediatric surgical and medical transplant fellowship training, primarily due to a limited number of adequate training programs and gaps in education. Addressing these gaps is essential for improving patient outcomes and advancing the field of pediatric transplantation. METHODS:An international survey was distributed to 416 pediatric transplant fellows via multiple global transplant associations. The 29-question survey, available in English and Spanish, assessed current clinical knowledge, training satisfaction, and preferred learning formats. The survey received responses from 237 pediatric transplant fellows. RESULTS:One of the key findings was that respondents felt most confident in their pre-transplantation clinical knowledge. About 75.5% of fellows reported being extremely or very aware of pre-transplant workups and assessments. However, significant gaps were identified in other areas of training. Notably, 24% of respondents felt extremely knowledgeable about the side effects of immunosuppression, and only 22.8% were very confident in managing acute rejection. Fellows expressed strong interest in dynamic educational formats, particularly clinical case discussions (88.8%). Preferred methods to enhance training included continuing medical education (64.6%), hands-on workshops, and support for transition protocols and psychosocial counseling. CONCLUSION:This global survey highlights critical educational gaps in pediatric transplant fellowship training and identifies preferred learning methods among fellows. Targeted improvements such as standardized curricula, case-based learning, interdisciplinary collaboration, and accessible continuing education are essential to enhance training. These strategies will ensure that future transplant professionals are well-equipped to deliver high-quality care across diverse healthcare settings.

BackgroundRisk for medication error increases during care transitions. Error susceptibility increases in pediatrics due to age and weight-based dosing. Governing bodies acknowledge errors endanger patients, and transplant pharmacotherapy experts are mandated by the Center for Medicare and Medicaid Services (CMS). Pharmacist-led medication histories reduce errors; however, pharmacists' roles in the transitions of care between transplant centers are unaddressed by CMS. We assessed the impact of transplant pharmacist involvement in medication histories for pediatrics transitioning between transplant centers.MethodsPediatric patients pre- or post-heart transplant transitioning to our center were proactively identified. Transplant pharmacists conducted medication histories by phone with caregivers and tracked discrepancies between existing medication lists and interview responses. Descriptive statistical analyses were completed.ResultsOf 27 patients identified, seven caregivers were uncontactable, one declined participation, and 19 underwent transplant pharmacist medication history. Medication list errors were detected in 100% of patients (n = 19), with a median [IQR] of 5 [3-11] issues per patient. Of 122 issues, 72 (59%) were inaccurate or missing data, 17.2% (n = 21) listed stopped drugs, 9.8% (n = 12) revealed incorrect administration routes, 7.4% (n = 9) were duplicate medications, and 6.6% (n = 8) of errors were medications needing to be added. Corrections included updating 84 inaccuracies (68.8%); removing/adding drugs (30 [24.6%] and 8 [6.6%], respectively); and team communication.ConclusionsComplex dosing strategies in pediatrics confer error vulnerability. Designing transition of care processes with transplant pharmacist involvement in medication histories can assist in ensuring medication list accuracy, thereby improving pediatric patient safety.

BACKGROUND:The Fontan procedure has transformed the management of congenital heart defects characterized by single ventricle physiology, yet it predisposes individuals to Fontan-associated liver disease. Combined heart and liver transplantation (CHLT) emerges as a therapeutic option, but evidence of its efficacy and safety remains limited. This study aimed to comprehensively evaluate CHLT in Fontan patients, focusing on patient characteristics, perioperative outcomes, and posttransplant morbidity and mortality. METHODS:Following Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines, a systematic search of PubMed, Embase, and the Cochrane Central Register of Controlled Trials was conducted. Studies meeting the intervention of CHLT in Fontan patients were included, and data were collected and synthesized using proportion meta-analysis techniques. Statistical analysis was carried out using R software. RESULTS:Four studies met inclusion criteria, comprising 67 Fontan patients undergoing CHLT. All included studies were observational retrospective cohorts performed in the United States. The 1-y survival rate post-CHLT was 88% (95% confidence interval [CI], 70%-98%). Liver graft rejection rates were low, 4% (95% CI, 0%-22%), and no heart graft rejection greater than mild was reported. Postoperative complications included acute kidney injury 75% (95% CI, 50%-93%), temporary dialysis 27% (95% CI, 9%-51%), neurologic events 7% (95% CI, 0%-26%), infection 23% (95% CI, 3%-55%), and unplanned medical procedures 40% (95% CI, 23%-59%). CONCLUSIONS:CHLT in Fontan patients demonstrates promising survival rates, but graft rejection and postoperative complications pose challenges. The rate of renal complications is particularly notable and requires further evaluation. Future research should prioritize comparative different management strategies and long-term follow-up to refine protocols and optimize outcomes.

IPTA created the SMARTER Initiative to address persistent gaps in pediatric transplant education worldwide. Through webinars, podcasts, case discussions, and interactive resources, SMARTER provides trainees and allied professionals with accessible, peer-reviewed content across all major organ systems. The program is designed for global reach, with downloadable modules, mobile compatibility, and recorded sessions to accommodate different regions and time zones. Since its launch, SMARTER has shown strong engagement, with fellows reporting that the platform helps them prepare for board exams, improve clinical decision-making, and connect learning directly to patient care. Survey feedback highlights high satisfaction and identifies opportunities for further growth, such as expanded case-based modules, procedural videos, and simulation exercises. By reducing disparities in transplant training and supporting collaboration, SMARTER is positioned to strengthen existing curricula and promote a better-prepared, more globally connected transplant workforce.