A Novel Mental Health Curriculum Pilot for Pediatric Cardiology Fellows: Preparing the Subspecialist to Expand the Team.

Mental health (MH) is an important, yet understudied area of care for patients with congenital heart disease (CHD). Pediatric subspecialty fellows believe MH should be within their scope of practice, but few feel confident in their ability to appropriately screen, evaluate, manage, and make treatment referrals for youth with CHD and concurrent MH concerns. A 6-session, didactic-based curriculum was designed by an interprofessional team of experts. It was administered to pediatric cardiology fellows over 9 months during two academic years. Topics included the following: Introduction to MH and CHD, MH Screening and Clinic-Based Interventions, Psychopharmacology, Delirium, Trauma-Informed Care, and Adult CHD and Transitional Care. An investigator-designed survey was developed to assess fellow comfort with MH knowledge, screening, and treatment recommendation domains. Twenty-three fellows participated with 14 completing the pre-post survey (58% participation, 11/17 in year 1, 3/6 in year 2). Most participants were in their first year of training (9/14, 57%). Overall, fellow comfort with MH topics increased significantly (mean score 2.89 ± 0.46 vs. 3.4 ± 0.4, p = 0.0005), with improved comfort in the MH knowledge (p = 0.003) and treatment recommendation domains (p = 0.001). A didactic-based MH curriculum improves the comfort of pediatric cardiology fellows to address the MH concerns of their patients. The topics in this curriculum will continue to be refined over time and could be generalized to training programs, both nationally and across pediatric specialties, to improve MH care for high-risk populations.

School age and adolescent heart failure following the Norwood procedure.

BACKGROUND: Heart failure results in significant morbidity and mortality for young children with hypoplastic left heart syndrome (HLHS) following the Norwood procedure. The trajectory in later childhood is not well described. METHODS: We studied the outcome into adolescence of participants enrolled in the Single Ventricle Reconstruction trial who underwent the Fontan procedure or survived to 6 years without having undergone Fontan procedure. The primary outcome was heart failure events, defined as heart transplant listing or death attributable to heart failure. Symptomatic heart failure for participants surviving 10 or more years was also assessed utilizing the Pediatric Quality of Life Inventory (PedsQL). RESULTS: Of the 345 participants who underwent a Fontan operation or survived to 6 years without Fontan, 25 (7.2%) had a heart failure event before the age of 12 years. Among these, 21 were listed for heart transplant, and 4 died from heart failure. Nineteen participants underwent heart transplant, all of whom survived to age 12 years. Factors associated with a heart failure event included longer Norwood hospital length of stay, aortic atresia, and no Fontan operation by age 6 years. Assessment of heart failure symptoms at 12 years of age revealed that 24 (12.2%) of 196 PedsQL respondents "often" or "almost always" had difficulty walking more than one block. CONCLUSIONS: Heart failure events occur in over 5% of children with palliated HLHS between preschool age and adolescence. Outcomes for children listed for transplant are excellent. However, a substantial portion of palliated HLHS children have significant symptoms of heart failure at 12 years of age.

Experience with SGLT2 Inhibitors in Patients with Single Ventricle Congenital Heart Disease and Fontan Circulatory Failure.

Heart failure is the leading cause of morbidity and mortality in patients with Fontan circulation. Sodium-glucose-cotransporter 2 inhibitors (SGLT2i) have become a mainstay of heart failure therapy in adult patients, however, there remains a paucity of literature to describe its use in pediatric heart failure patients, especially those with single ventricle physiology. We describe our early experience using SGLT2i in patients with single ventricle congenital heart disease surgically palliated to the Fontan circulation. We conducted a single-center retrospective chart review of all patients with Fontan circulation who were initiated on an SGLT2i from January 1, 2022 to March 1, 2023. Patient demographics, diagnoses, clinical status, and other therapies were collected from the electronic medical record. During the study period, 14 patients (median age 14.5 years, range 2.0-26.4 years) with Fontan circulation were started on a SGLT2i. Mean weight was 54 kg (range 11.6-80.4 kg). Median follow-up since SGLT2i initiation was 4.1 months (range 13 days-7.7 months). Four patients had a systemic left ventricle and 10 had a systemic right ventricle. Half the patients had Fontan Circulatory Failure with reduced Ejection Fraction (FCFrEF) of the systemic ventricle and the other half had Fontan Circulatory Failure with preserved Ejection Fraction (FCFpEF) of the systemic ventricle. In addition, 3 patients experienced Protein Losing Enteropathy (PLE) and 2 patients had plastic bronchitis, one of whom also was diagnosed with chylothorax. There were no genitourinary infections, hypoglycemia, ketoacidosis, hypotension or other significant adverse effects noted in our patient population. One patient experienced significant diuresis and transient acute kidney injury. Patients with FCFrEF showed a decrease in natriuretic peptide levels. Given the lack of proven therapies, demonstrated benefits of SGLT2i in other populations, and some suggestion of efficacy in Fontan circulation, further study of SGTLT2i in patients with Fontan circulation is warranted.

Pediatric Heart Transplant Rejection After COVID-19 Infection.

BACKGROUND: Infections have been associated with rejection episodes in solid organ transplant recipients. We report an association between COVID-19 infection and heart transplant (HT) rejection. CASE DESCRIPTION: The patient was 14 years old and 6.5 years post-HT. He developed symptoms of rejection within 2 weeks of COVID exposure and presumed infection. CONCLUSIONS: In this case, COVID-19 infection closely preceded significant rejection and graft dysfunction. Further study is needed to establish a correlation between COVID-19 infection and rejection in HT patients.

Medical and End-of-Life Decision-Making Preferences in Adolescents and Young Adults with Advanced Heart Disease and Their Parents.

Importance: Despite high disease morbidity and mortality and complex treatment decisions, little is known about the medical and end-of-life decision-making preferences of adolescents and young adults (AYA) with advanced heart disease. AYA decision-making involvement is associated with important outcomes in other chronic illness groups. Objective: To characterize decision-making preferences of AYAs with advanced heart disease and their parents and determine factors associated with these preferences. Design, Setting, and Participants: Cross-sectional survey between July 2018 and April 2021 at a single-center heart failure/transplant service at a Midwestern US children's hospital. Participants were AYAs aged 12 to 24 years with heart failure, listed for heart transplantation, or posttransplant with life-limiting complications and a parent/caregiver. Data were analyzed from May 2021 to June 2022. Main Outcomes and Measures: Single-item measure of medical decision-making preferences, MyCHATT, and Lyon Family-Centered Advance Care Planning Survey. Results: Fifty-six of 63 eligible patients enrolled in the study (88.9%) with 53 AYA-parent dyads. Median (IQR) patient age was 17.8 (15.8-19.0) years; 34 (64.2%) patients were male, and 40 patients (75.5%) identified as White and 13 patients (24.5%) identified as members of a racial or ethnic minority group or multiracial. The greatest proportion of AYA participants (24 of 53 participants [45.3%]) indicated a preference for active, patient-led decision-making specific to heart disease management, while the greatest proportion of parents (18 of 51 participants [35.3%]) preferred they and physician(s) make shared medical decisions on behalf of their AYA, representing AYA-parent decision-making discordance (χ2 = 11.7; P = .01). Most AYA participants stated a preference to discuss adverse effects or risks of treatment (46 of 53 participants [86.8%]), procedural and/or surgical details (45 of 53 participants [84.9%]), impact of condition on daily activities (48 of 53 participants [90.6%]), and their prognosis (42 of 53 participants [79.2%]). More than half of AYAs preferred to be involved in end-of-life decisions if very ill (30 of 53 participants [56.6%]). Longer time since cardiac diagnosis (r = 0.32; P = .02) and worse functional status (mean [SD] 4.3 [1.4] in New York Heart Association class III or IV vs 2.8 [1.8] in New York Heart Association class I or II; t-value = 2.7; P = .01) were associated with a preference for more active, patient-led decision-making. Conclusions and Relevance: In this survey study, most AYAs with advanced heart disease favored active roles in medical decision-making. Interventions and educational efforts targeting clinicians, AYAs with heart disease, and their caregivers are needed to ensure they are meeting the decision-making and communication preferences of this patient population with complex disease and treatment courses.

Paediatric cardiology training: burnout, fulfilment, and fears.

BACKGROUND: Burnout is well characterised in physicians and residents but not in paediatric cardiology fellows, and few studies follow burnout longitudinally. Training-specific fears have been described in paediatric cardiology fellows but also have not been studied at multiple time points. This study aimed to measure burnout, training-specific fears, and professional fulfilment in paediatric cardiology fellows with the attention to time of year and year-of-training. METHODS: This survey-based study included the Professional Fulfillment Index and the Impact of Events Scale as well as an investigator-designed Fellow Fears Questionnaire. Surveys were distributed at three-time points during the academic year to paediatric cardiology fellows at a large Midwestern training programme. Fellow self-reported gender and year-of-training were collected. Descriptive analyses were performed. RESULTS: 10/17 (59%) of fellows completed all surveys; 60% were female, 40% in the first-year class, 40% in the second-year class, and 20% in the third-year class. At least half of the fellows reported burnout at each survey time point, with lower mean professional fulfilment scores. The second-year class, who rotate primarily in the cardiac ICU, had higher proportions of burnout than the other two classes. At least half of fellows reported that they "often" or "always" worried about not having enough clinical knowledge or skills and about work-life balance. CONCLUSIONS: Paediatric cardiology fellows exhibit high proportions of burnout and training-specific fears. Interventions to mitigate burnout should be targeted specifically to training needs, including during high-acuity rotations.

Data-driven computational models of ventricular-arterial hemodynamics in pediatric pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a complex disease involving increased resistance in the pulmonary arteries and subsequent right ventricular (RV) remodeling. Ventricular-arterial interactions are fundamental to PAH pathophysiology but are rarely captured in computational models. It is important to identify metrics that capture and quantify these interactions to inform our understanding of this disease as well as potentially facilitate patient stratification. Towards this end, we developed and calibrated two multi-scale high-resolution closed-loop computational models using open-source software: a high-resolution arterial model implemented using CRIMSON, and a high-resolution ventricular model implemented using FEniCS. Models were constructed with clinical data including non-invasive imaging and invasive hemodynamic measurements from a cohort of pediatric PAH patients. A contribution of this work is the discussion of inconsistencies in anatomical and hemodynamic data routinely acquired in PAH patients. We proposed and implemented strategies to mitigate these inconsistencies, and subsequently use this data to inform and calibrate computational models of the ventricles and large arteries. Computational models based on adjusted clinical data were calibrated until the simulated results for the high-resolution arterial models matched within 10% of adjusted data consisting of pressure and flow, whereas the high-resolution ventricular models were calibrated until simulation results matched adjusted data of volume and pressure waveforms within 10%. A statistical analysis was performed to correlate numerous data-derived and model-derived metrics with clinically assessed disease severity. Several model-derived metrics were strongly correlated with clinically assessed disease severity, suggesting that computational models may aid in assessing PAH severity.

The evolution of pediatric heart retransplantation over three decades: An analysis from the PHTS.

BACKGROUND: Retransplantation is rare and associated with worse survival and more morbidity. The study aim is to describe an updated cohort of pediatric retransplants, determine if there has been an era effect on outcomes, and understand if identified trends are explained by changes in patient selection. METHODS: Pediatric Heart Transplant Society database analysis of retransplantation patients <18 years of age (Era 1: 1993-2001, Era 2: 2002-2010, Era 3: 2011-2018). Multivariate analysis identified risk factors for graft loss. Multiphase parametric hazard modeling was used to depict era and risk factor effect. RESULTS: Survival was lower (p < .0001) for retransplant (n = 222) compared to primary transplant (n = 6548) (median 9.3 vs 20.2 years). Median survival increased from Era 1 to 2 (4.8 vs 9.3 years; p < .0001) with no incremental change in Era 3. Era 2 and 3 retransplants had a longer inter-transplant interval (p < .0001), were less frequently for early graft failure (p = .0004) or acute rejection (p = .007), more frequently from a ventricular assist device (p = .0014), and less frequently from extracorporeal membrane oxygenation (p = .0024). Predictors of graft loss included Era 1 (HR 10.55, p = .001), congenital heart disease (HR 4.42, p = .01), inter-transplant interval <1 year (HR 5.34, p = .002), and mechanical support (ventricular assist device HR 7.47, p = .0042; extracorporeal membrane oxygenation HR 10.09, p < .0001). For each 1-year increase in inter-transplant interval, graft loss risk decreased by 1.15 (p = .0002). Retransplantation was associated with more rejection, infection, and allograft vasculopathy. CONCLUSIONS: Graft survival has improved in pediatric retransplants making it a viable option in select patients. Retransplantation should be avoided in the setting of early graft failure especially requiring mechanical support.

Primary Disease, Sex, and Racial Differences in Health-Related Quality of Life in Adolescents and Young Adults with Heart Failure.

Health-related quality of life (HRQOL) is an important clinical and research trial endpoint in adult heart failure and has been shown to predict mortality and hospitalizations in adult heart failure populations. HRQOL has not been adequately studied in the growing pediatric and young adult heart failure population. This study described HRQOL in adolescents and young adults (AYAs) with heart failure and examined primary disease, sex, race, and other correlates of HRQOL in this sample. Participants in this cross-sectional, single-center study included adolescent and young adults with heart failure and a parent/guardian. Patients and their parent/proxies completed the PedsQL, a well-established measure of HRQOL in pediatric chronic illness populations. HRQOL is impaired in AYAs with heart failure resulting from dilated, hypertrophic, or other cardiomyopathy, congenital heart disease, or post-transplant with rejection/complications. Patients identifying as white endorsed poorer total HRQOL than non-white patients (p = 0.002). Subscale analysis revealed significant correlations between female sex (p = 0.01) and white race (p = 0.01) with poorer self-reported physical functioning. Family income was unrelated to HRQOL. Functional status was strongly associated with total (p = 0.0003) and physical HRQOL (p < 0.0001). Sociodemographic and disease-specific risk and resilience factors specific to HRQOL in AYAs with heart failure include primary cardiac disease, race, sex, and functional status. Building upon extensive work in adult heart failure, utilization, and study of HRQOL as a clinical and research trial outcome is necessary in pediatric heart failure. Developing targeted interventions for those at greatest risk of impaired HRQOL is an important next step.

Burnout, professional fulfillment, and post-traumatic stress among pediatric solid organ transplant teams.

BACKGROUND: Adverse effects of clinician burnout have been studied across multiple specialties; however, there have been no studies examining rates of burnout among pediatric solid organ transplant teams. This study aimed to measure burnout, work exhaustion, professional fulfillment, and post-traumatic stress symptoms among clinicians and administrators practicing in this high-stress field. METHODS: This cross-sectional study utilized a 50 item web-based survey that included the Personal Fulfillment Index and the IES-R. This survey was distributed across four pediatric solid organ transplant centers in North America. Basic demographics, clinician characteristics, and information regarding wellness and self-care activities were collected. Descriptive and correlational analyses were performed. RESULTS: One hundred and thirty five participants completed the survey, 76% were female and 78% were Caucasian. One-third (34%) of participants endorsed burnout, while 43% reported professional fulfillment. Approximately 15% of respondents endorsed clinically significant levels of post-traumatic stress symptoms related to patient deaths, with female clinicians more likely to endorse symptoms (p = .01). Nearly 80% of participants reported engaging in self-care activities outside of work and only 10% of participants reported participation in hospital-sponsored wellness programs. CONCLUSIONS: Pediatric solid organ transplant team members exhibited moderate levels of burnout, professional fulfillment, and post-traumatic stress. Female clinicians were the most likely to experience both work exhaustion and post-traumatic stress symptoms. Transplant centers are encouraged to consider interventions and programming to improve clinician wellness.

Psychosocial outcomes in pediatric patients with ventricular assist devices and their families: A systematic review.

Ventricular assist device (VAD) therapy has provided pediatric patients with severe heart failure new therapeutic options. However, pediatric patients and families receiving VAD therapy also experience psychosocial challenges. No synthesis of the literature on psychosocial outcomes within the pediatric VAD population has been conducted; thus, the current review sought to systematically investigate the impact of pediatric VAD on patient, parent, and family psychosocial outcomes and assess variables associated with poorer outcomes. Literature searches were conducted in PsycInfo, PubMed, and Cumulative Index to Nursing and Allied Health Literature databases and full texts were assessed according to pre-established inclusion criteria. Main findings and study quality were reviewed. Sixteen studies were included in the present review. Findings highlighted the likelihood for psychological sequelae among pediatric patients and families receiving VAD therapy, including decreased psychological and emotional well-being, elevated stress, and difficulty coping with changes to family dynamics and responsibilities. Health-related quality of life was reported comparable to that of healthy peers and children with other cardiac conditions. Patients and families on VAD therapy experience significant difficulties in psychological well-being and challenges related to adjustment. The current review underscores the importance of ongoing support for families and continued assessment of psychosocial functioning across VAD support. Three critical periods for increased distress were identified: 1) immediately following VAD implantation, 2) discharge home and the weeks following discharge, and 3) long-term VAD therapy.

Acute Hemodynamic Effects of Negative Extrathoracic Pressure in Fontan Physiology.

We sought to assess acute hemodynamic changes after implementation of negative extrathoracic pressure (NEP) in spontaneously breathing ambulatory Fontan patients with symptomatic heart failure. We hypothesized that application of NEP would result in an acute decrease in pulmonary artery pressure. Ten patients with clinical evidence of Fontan failure underwent baseline hemodynamic catheterization while breathing spontaneously. Hemodynamic measurements were then repeated after 30 min of continuous NEP. After 30 min of continuous NEP, 4/10 patients had a decrease in their Fontan pressure by 2 mmHg and one patient had a decrease by 1 mmHg. There were three patients that had an increase in Fontan pressure by 2 mmHg. In 7/10 patients, indexed pulmonary vascular resistance decreased by an average of 31%. In symptomatic Fontan patients with a favorable hemodynamic response to NEP during catheterization, potential benefit of longer-term NEP to improve clinical status should be explored.

Use of Hemodynamic Ramp Study to Optimize Continuous-Flow Ventricular Assist Device in a Fontan Patient.

The utility of hemodynamic ramp studies in single ventricle patients supported by continuous flow left ventricular assist devices (CF-LVADs) is largely unknown. We report the novel use of the hemodynamic ramp test to optimize a 26 year-old Fontan-palliated single-ventricle patient supported with a continuous-flow ventricular assist device (Heartware HVAD). Modest improvements in Fontan hemodynamics resulted in significant and persistent clinical improvement and symptomatic relief.

Quartiles of peak troponin are associated with long-term risk of death in type 1 and STEMI, but not in type 2 or NSTEMI patients.

BACKGROUND: The prognostic value of peak cardiac troponin (cTn) in different types of acute myocardial infarction (AMI) under the universal clinical classification is unknown. HYPOTHESIS: We tested the hypothesis that the prognostic value of cTn varies with its peak level and type of AMI. METHODS: We studied 345 consecutive patients with AMI with mean follow-up of 30.6 months according to quartiles of peak cTn level (QPTL) and the type of AMI. The study outcomes were the major adverse cardiovascular events (MACE; composite of all causes of mortality and recurrent AMI) and the individual components of MACE. RESULTS: The study included patients with AMI Type 1 (n = 276), type 2 (n = 54), ST-segment elevation myocardial infarction (STEMI; n = 159), and non-ST-segment elevation myocardial infarction (NSTEMI; n = 186). Overall, peak cTn level was an independent predictor of MACE (hazard ratio [HR]: 1.001, 95% confidence interval [CI]: 1.000-1.003, P = 0.01) and death (HR: 1.002, 95% CI: 1.001-1.004, P = 0.003), but not of recurrent AMI. The highest risk of MACE and death was in the highest QPTL (61.6%, P = .016 and 66.3%, P = 0.021, respectively) while the highest risk of recurrent AMI was in the lowest QPTL (83.7%, P = 0.04). Quartiles of peak cTn level were significantly associated with increased risk of MACE and death in patients with Type 1 (all P = 0.01) and STEMI (P = 0.01 and P = 0.02, respectively), but no association existed in type 2 or NSTEMI patients. CONCLUSIONS: Overall, peak cTn predicts the risk of MACE and death but not the risk of AMI. While in Type 1 and STEMI patients, QPTL are associated with risk of MACE and death, no association exists in type 2 or NSTEMI patients.