Life and Death: A Multicenter Study Evaluating Cardiologists' Approach to Difficult Conversations with Fontan Patients and Families.

Outpatient cardiologists provide longitudinal care for Fontan patients. As these patients age, they face mounting morbidities, necessitating challenging conversations about prognosis and goals of care. We created a novel survey to evaluate cardiologists' attitudes surrounding risk counseling for patients/caregivers. Cardiologists were recruited during concomitant outpatient enrollment of individuals with Fontan operation > age 10. Physician demographic data, expectations of timing in discussing adverse event risk, and perceived barriers were collected. Barriers were analyzed using a thematic approach. 40 cardiologists (9 institutions) responded regarding 155 patients (mean age 21.2 years, SD 7.7). Physicians were mostly male (58%) with mean practice of 21 years post-fellowship (SD 12). Most felt the time was right to have a conversation with patient (55%) and family (62%), and majority thought patient (53%) and family (75%) were ready for such a conversation. Most had previously discussed prognosis with patient (72%) and family (75%). Providers were inclined to discuss risk with caregivers earlier (mean patient age 9 years, SD 11) than patients (mean patient age 17 years, SD 6.4). Nevertheless, 42% of physicians perceived significant barriers and provided 58 narrative comments categorized into 4 major themes: (1) Patient-related (53.4%), including cognitive limitations and mental health; (2) Provider-related (16.4%), including lack of familiarity, preservation of happiness, and discomfort; (3) Family related (12.3%), including protection/denial and psychosocial stressors; (4) Other (26%), including social barriers. Experienced cardiologists are willing to have difficult conversations; nearly half reported largely patient-related barriers. Facilitating these conversations is critical for the adolescent/young adult with Fontan physiology.

How Good Are Cardiologists at Predicting Major Adverse Events in Fontan Patients?

Background: It is unknown how well cardiologists predict which Fontan patients are at risk for major adverse events (MAEs). Objectives: The purpose of this study was to examine the accuracy of cardiologists' ability to identify the "good Fontan" patient, free from MAE within the following year, and compare that predicted risk cohort to patients who experienced MAE. Methods: This prospective, multicenter study included patients ≥10 years with lateral tunnel or extracardiac Fontan. The cardiologist was asked the yes/no "surprise" question: would you be surprised if your patient has a MAE in the next year? After 12 months, the cardiologist was surveyed to assess MAE. Agreement between cardiologist predictions of MAE and observed MAE was determined using the simple kappa coefficient. Multivariable generalized linear mixed effects models were performed to identify factors associated with MAE. Results: Overall, 146 patients were enrolled, and 99/146 (68%) patients w`ere predicted to be a "good Fontan." After 12 months, 17 (12%) experienced a MAE. The simple kappa coefficient of cardiologists' prediction was 0.17 (95% CI: 0.02-0.32), suggesting prediction of MAE was 17% better than random chance. In the multivariable cardiologist-predicted MAE (N = 47) model, diuretic/beta-blocker use (P ≤ 0.001) and systolic dysfunction (P = 0.005) were associated with MAE. In the observed multivariable MAE (N = 17) model, prior unplanned cardiac admission (P = 0.006), diuretic/beta-blocker use (P = 0.028), and ≥moderate atrioventricular valve regurgitation (P = 0.049) were associated with MAE. Conclusions: Cardiologists are marginally able to predict which Fontan patients are at risk for MAE over a year. There was overlap between factors associated with a cardiologist's prediction of risk and observed MAE, namely the use of diuretic/beta-blocker.

Impact of Age-Related Change in Caval Flow Ratio on Hepatic Flow Distribution in the Fontan Circulation.

BACKGROUND: The Fontan operation is a palliative technique for patients born with single ventricle heart disease. The superior vena cava (SVC), inferior vena cava (IVC), and hepatic veins are connected to the pulmonary arteries in a total cavopulmonary connection by an extracardiac conduit or a lateral tunnel connection. A balanced hepatic flow distribution (HFD) to both lungs is essential to prevent pulmonary arteriovenous malformations and cyanosis. HFD is highly dependent on the local hemodynamics. The effect of age-related changes in caval inflows on HFD was evaluated using cardiac magnetic resonance data and patient-specific computational fluid dynamics modeling. METHODS: SVC and IVC flow from 414 patients with Fontan were collected to establish a relationship between SVC:IVC flow ratio and age. Computational fluid dynamics modeling was performed in 60 (30 extracardiac and 30 lateral tunnel) patient models to quantify the HFD that corresponded to patient ages of 3, 8, and 15 years, respectively. RESULTS: SVC:IVC flow ratio inverted at ≈8 years of age, indicating a clear shift to lower body flow predominance. Our data showed that variation of HFD in response to age-related changes in caval inflows (SVC:IVC, 2, 1, and 0.5 corresponded to ages, 3, 8, and 15+, respectively) was not significant for extracardiac but statistically significant for lateral tunnel cohorts. For all 3 caval inflow ratios, a positive correlation existed between the IVC flow distribution to both the lungs and the HFD. However, as the SVC:IVC ratio changed from 2 to 0.5 (age, 3-15+) years, the correlation's strength decreased from 0.87 to 0.64, due to potential flow perturbation as IVC flow momentum increased. CONCLUSIONS: Our analysis provided quantitative insights into the impact of the changing caval inflows on Fontan's long-term HFD, highlighting the importance of SVC:IVC variations over time on Fontan's long-term hemodynamics. These findings broaden our understanding of Fontan hemodynamics and patient outcomes.

A Deep Learning Pipeline for Assessing Ventricular Volumes from a Cardiac MRI Registry of Patients with Single Ventricle Physiology.

Purpose To develop an end-to-end deep learning (DL) pipeline for automated ventricular segmentation of cardiac MRI data from a multicenter registry of patients with Fontan circulation (Fontan Outcomes Registry Using CMR Examinations [FORCE]). Materials and Methods This retrospective study used 250 cardiac MRI examinations (November 2007-December 2022) from 13 institutions for training, validation, and testing. The pipeline contained three DL models: a classifier to identify short-axis cine stacks and two U-Net 3+ models for image cropping and segmentation. The automated segmentations were evaluated on the test set (n = 50) by using the Dice score. Volumetric and functional metrics derived from DL and ground truth manual segmentations were compared using Bland-Altman and intraclass correlation analysis. The pipeline was further qualitatively evaluated on 475 unseen examinations. Results There were acceptable limits of agreement (LOA) and minimal biases between the ground truth and DL end-diastolic volume (EDV) (bias: -0.6 mL/m2, LOA: -20.6 to 19.5 mL/m2) and end-systolic volume (ESV) (bias: -1.1 mL/m2, LOA: -18.1 to 15.9 mL/m2), with high intraclass correlation coefficients (ICCs > 0.97) and Dice scores (EDV, 0.91 and ESV, 0.86). There was moderate agreement for ventricular mass (bias: -1.9 g/m2, LOA: -17.3 to 13.5 g/m2) and an ICC of 0.94. There was also acceptable agreement for stroke volume (bias: 0.6 mL/m2, LOA: -17.2 to 18.3 mL/m2) and ejection fraction (bias: 0.6%, LOA: -12.2% to 13.4%), with high ICCs (>0.81). The pipeline achieved satisfactory segmentation in 68% of the 475 unseen examinations, while 26% needed minor adjustments, 5% needed major adjustments, and in 0.4%, the cropping model failed. Conclusion The DL pipeline can provide fast standardized segmentation for patients with single ventricle physiology across multiple centers. This pipeline can be applied to all cardiac MRI examinations in the FORCE registry. Keywords: Cardiac, Adults and Pediatrics, MR Imaging, Congenital, Volume Analysis, Segmentation, Quantification Supplemental material is available for this article. © RSNA, 2023.

Health Care Providers Working Cross-Culturally: Pitfalls, Pearls, and Preparation Resources for Culture Shock.

Health care providers engaging in cross-cultural work will likely experience culture shock, a psychological, behavioral, and physiologic response to new cultural environments that can significantly affect travelers. Culture shock has the potential for both negative and positive outcomes. Well-being, health, and professionalism can be negatively influenced during the peak of culture shock, but the experience may also positively promote transformative learning and professional identity formation. Culture shock has been carefully researched for different types of sojourners, such as undergraduate students and business personnel, but minimally for health care providers. This article defines culture shock, describes different health care-related cross-cultural opportunities, identifies factors contributing to culture shock, describes complexities related to measuring culture shock, depicts common cross-cultural challenges encountered by traveling health care providers, and offers tangible guidance to help prepare for culture shock. We conclude with a call for further research and resource development to support the well-being of an increasingly global health care workforce. [Pediatr Ann. 2023;52(9):e335-e343.].

Craving Sweet Success: A Recipe for Developing a Global Health Education Consortium (or Any Consortium, for That Matter).

More than a decade ago, two faculty met at a conference. Each talked about how they were charged with leading global health education efforts at their institutions and longed to have an opportunity to share resources and learn from each other. After reaching out to a few other Midwestern colleagues and finding a date, the first Midwest Consortium of Global Child Health Educators meeting was held in Madison, WI. Now, after a dozen annual meetings, more than 30 articles, chapters, abstracts, and workshops, as well as the creating, piloting, and sharing of several widely used curricula in global health education, the founding consortium members share the practical steps for faculty looking to form similar regional consortia around shared interests. In this article, the authors provide a recipe for the successful formation of an academic consortium based on the lessons learned from their experience. [Pediatr Ann. 2023;52(9):e351-e356.].

More Than Just a Passport: Resources and Implementation Strategies to Optimize Preparation for Global Health Work.

During the past decade, many resources have been developed to support trainees and clinicians seeking to prepare for global health work. For time-constrained health care providers, figuring out how to prepare can be overwhelming. Given the wide variation in types of travelers and work plans, there is not a "one size fits all" preparation resource. This article offers a summary of preparation topics that all travelers should consider; compiles curated, high-yield resources designed to prepare health care providers for global health experiences; and provides implementation strategies to best meet the unique needs of each traveler, taking into consideration factors such as provider expertise (trainee vs practicing clinician), solo versus group travel, and time available before departure. These curated resources include a variety of training modalities (self-directed, group-based, train-the-trainer, and in-person courses), all summarized here to empower health care providers to create individualized, comprehensive preparation plans before engaging globally. [Pediatr Ann. 2023;52(9):e330-e334.].

Magnetic resonance three-dimensional steady-state free precession imaging of the thoracic duct in patients with Fontan circulation and its relationship to outcomes.

BACKGROUND: Lymphatic complications are common in patients with Fontan circulation. Three-dimensional balanced steady-state free precession (3D bSSFP) angiography by cardiovascular magnetic resonance (CMR) is widely used for cardiovascular anatomical assessment. We sought to determine the frequency of thoracic duct (TD) visualization using 3D bSSFP images and assess whether TD characteristics are associated with clinical outcomes. METHODS: This was a retrospective, single-center study of patients with Fontan circulation who underwent CMR. Frequency matching of age at CMR was used to construct a comparison group of patients with repaired tetralogy of Fallot (rTOF). TD characteristics included maximum diameter and a qualitative assessment of tortuosity. Clinical outcomes included protein-losing enteropathy (PLE), plastic bronchitis, listing for heart transplantation, and death. A composite outcome was defined as presence of any of these events. RESULTS: The study included 189 Fontan patients (median age 16.1 years, IQR 11.0-23.2 years) and 36 rTOF patients (median age 15.7 years, IQR 11.1-23.7 years). The TD diameter was larger (median 2.50 vs. 1.95 mm, p = 0.002) and more often well visualized (65% vs. 22%, p < 0.001) in Fontan patients vs. rTOF patients. TD dimension increased mildly with age in Fontan patients, R = 0.19, p = 0.01. In Fontan patients, the TD diameter was larger in those with PLE vs. without PLE (age-adjusted mean 4.11 vs. 2.72, p = 0.005), and was more tortuous in those with NYHA class ≥ II vs. class I (moderate or greater tortuosity 75% vs. 28.5%, p = 0.02). Larger TD diameter was associated with a lower ventricular ejection fraction that was independent of age (partial correlation = - 0.22, p = 0.02). More tortuous TDs had a higher end-systolic volume (mean 70.0 mL/m2 vs. 57.3 mL/m2, p = 0.03), lower creatinine (mean 0.61 mg/dL vs. 0.70 mg/dL, p = 0.04), and a higher absolute lymphocyte count (mean 1.80 K cells/µL vs. 0.76 K cells/µL, p = 0.003). The composite outcome was present in 6% of Fontan patients and was not associated with TD diameter (p = 0.50) or tortuosity (p = 0.09). CONCLUSIONS: The TD is well visualized in two-thirds of patients with Fontan circulation on 3D-bSSFP images. Larger TD diameter is associated with PLE and increased TD tortuosity is associated with an NYHA class ≥ II.

Encephalopathy With Akinetic Mutism in a Child With COVID-19 Infection: A Case Report.

INTRODUCTION: COVID-19 has been associated with neurological complications, including encephalopathy and akinetic mutism. CASE PRESENTATION: A 7-year-old unvaccinated boy presented with visual hallucinations, urinary incontinence, and akinetic mutism 13 days after he was exposed to COVID-19. He had minimal respiratory symptoms, including just 1 day of fever and cough. Evaluations showed slowing on electroencephalogram, normal cerebrospinal fluid, normal brain magnetic resonance imaging, and mild sinus bradycardia. He recovered rapidly to baseline after 5 days of intravenous methylprednisolone. DISCUSSION: COVID-19-related encephalopathy including akinetic mutism is usually found in older adult patients with more severe COVID-19 illness. Our case demonstrates that akinetic mutism can present in children with mild COVID-19 illness and that it can respond rapidly and completely to intravenous methylprednisolone. CONCLUSIONS: COVID-19-related encephalopathy may be immune mediated. A heightened awareness of its association with COVID-19 illness should lead to earlier diagnosis and consideration of immunomodulatory therapy.

Transaminase Elevation in Nutritional Infantile Failure to Thrive.

INTRODUCTION: Laboratory investigations pursued for infants with failure to thrive (FTT) often show mild transaminase elevations, the incidence and significance of which are unknown. METHODS: This retrospective chart review included infants diagnosed with simple nutritional FTT at a single academic tertiary care system. Comparisons of diagnostic studies and outcomes between children with and without transaminase elevation were performed using chi-square and Wilcoxon rank sum tests. RESULTS: None of the infants with abnormal transaminases required additional follow-up or developed alternative diagnoses in the following year. DISCUSSION: Transaminase elevation may be common in infants with FTT and may not warrant further investigation if the history indicates an isolated etiology of insufficient nutrition.

The Immigrant Partnership and Advocacy Curricular Kit: A Comprehensive Train-the-Trainer Curriculum in Immigrant and Refugee Health.

The number of immigrants and refugees in the United States is growing, yet many trainees and clinicians feel unprepared to manage the diverse needs of this population. This perspective piece describes the development of the Immigrant Partnership and Advocacy Curricular Kit (I-PACK) by the Midwest Consortium of Global Child Health Educators. I-PACK is an adjunct to the Consortium's sugarprep.org global health curricular materials. Using Kern's six-step approach to curriculum development, they developed eight modules in immigrant and refugee health that incorporate interactive learning activities. The I-PACK was launched as an open-access resource in September 2020. As of September 2021, the curriculum has been freely available at sugarprep.org/i-pack and downloaded from educators in 15 countries. The I-PACK curriculum can address a growing need in medical education to empower learners and clinicians to provide competent and compassionate care for immigrants and refugees.

A Passport for a New Era of Learning.

Over the last several years, there has been a surge of readily available curricular resources for global health (GH) educators that theoretically has enabled them to overcome the barrier of needing to create new content for their programs. Despite this increase in available resources, integrating GH education into the already busy schedule of residency is a common challenge to the growing number of GH track directors. In this perspectives piece, GH educators from multiple institutions will share a novel model for packaging, administering, and monitoring GH educational curricula. This model transposes traditional GH learning objectives into self-paced, longitudinal maps of opportunities suitable for the time-intensive demands of residency, with flexibility for individual learning preferences and built-in tracking mechanisms.

Mitigating the Impacts of COVID-19 on Global Child Health: a Call to Action.

PURPOSE OF REVIEW: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the cause of coronavirus disease 2019 (COVID-19), continues to affect individuals, communities, and health systems worldwide. Here, we highlight how COVID-19 threatens to jeopardize the tremendous gains made over the last few decades on improving children's health globally. RECENT FINDINGS: In contrast to adults, children with COVID-19 are less likely to develop severe disease requiring hospitalization or die as a direct result of infection. However, the pandemic will likely have other important health impacts disproportionately affecting vulnerable children globally. Possible effects include worsening of poverty and food insecurity; disruption of already strained routine child health services; damage to already imperiled healthcare workforces; a wave of mental health challenges; interruption of education; and increased risks of violence, abuse, exploitation, and neglect. These challenges notwithstanding, the response to COVID-19 may also provide opportunities, such as for health system strengthening, that could improve child health after the pandemic. SUMMARY: The negative impacts of COVID-19 on global child health may be substantial. However, these are not foregone conclusions and much can be done to mitigate the worst outcomes. Child health providers should advocate for an equitable response to COVID-19 that prioritizes the health of vulnerable children and furthers the gains made in global child health.

Establishment and Retrospective Analysis of a Pilot Peer Mentorship Program.

BACKGROUND: Studies suggest widespread advantages to peer mentoring programs; however, there is minimal data pertaining to medical students mentoring undergraduate students. OBJECTIVES: To determine the feasibility and perceived effectiveness of a medical student-undergraduate student peer mentorship program. METHODS: A needs assessment guided the development of Pre-Med Pair Up, a program connecting medical student mentors from the Medical College of Wisconsin and other US medical schools to undergraduates at Marquette University and the University of Wisconsin-Oshkosh to provide peer mentorship, premedical resources, and global health information. After 6 months, surveys were distributed to 43 premedical and 26 medical students to evaluate the program. Descriptive statistics and Pearson correlations (r) were used to assess the relational strength between program components and student confidence and knowledge. RESULTS: Eleven undergraduate and 26 medical students completed surveys. Most undergraduates expressed increased confidence in abilities as premedical students associated with program involvement (18.2% great, 27.3% moderate, 45.5% minimal, 9.1% no improvement). Increased confidence was strongly correlated with knowledge of volunteer opportunities (r = 0.887, P < 0.001) and feelings of preparedness for the medical school application process (r = 0.854, P = 0.001) and curriculum (r = 0.871, P < 0.001). CONCLUSION: While self-reported confidence improved and overall positive program outcomes were statistically significant, the number of participants was low and the number who completed mid-year surveys was even lower. Therefore, no conclusions about program effectiveness were made. Instead, a lessons-learned approach was used to discuss the pilot development, implementation, and suggestions for future program installment.

Integrated Clinical and Magnetic Resonance Imaging Assessments Late After Fontan Operation.

BACKGROUND: Several clinical and cardiac magnetic resonance (CMR)-derived parameters have been shown to be associated with death or heart transplant late after the Fontan operation. OBJECTIVES: The objective of this study was to identify the relative importance and interactions of clinical and CMR-based parameters for risk stratification after the Fontan operation. METHODS: Fontan patients were retrospectively reviewed. Clinical and CMR parameters were analyzed using univariable Cox regression. The primary endpoint was time to death or (listing for) heart transplant. To identify the patients at highest risk for the endpoint, classification and regression tree survival analysis was performed, including all significant variables from Cox regression. RESULTS: The cohort consisted of 416 patients (62% male) with a median age of 16 years (25th, 75th percentiles: 11, 23 years). Over a median follow-up of 5.4 years (25th, 75th percentiles: 2.4, 10.0 years) after CMR, 57 patients (14%) reached the endpoint (46 deaths, 7 heart transplants, 4 heart transplant listings). Lower total indexed end-diastolic volume (EDVi) was the strongest predictor of transplant-free survival. Among patients with dilated ventricles (EDVi ≥156 ml/BSA1.3), worse global circumferential strain (GCS) was the next most important predictor (73% vs. 44%). In patients with smaller ventricles (EDVi <156 ml/BSA1.3), New York Heart Association functional class ≥II was the next most important predictor (30% vs. 4%). CONCLUSIONS: In this cohort of patients late after Fontan operation, increased ventricular dilation was the strongest independent predictor of death or transplant (listing). Patients with both ventricular dilation and worse GCS were at highest risk. These data highlight the value of integrating CMR and clinical parameters for risk stratification in this population.

Hemosuccus Pancreaticus Following Acute Pancreatitis in a 12-years-old Boy Secondary to Pancreatic Pseudoaneurysm Treated With Endovascular Coil Embolization.

Hemosuccus pancreaticus is a very rare cause of upper gastrointestinal bleeding in children. It is defined as bleeding from the pancreatic or peripancreatic vessels into the main pancreatic duct and may be life-threatening. We present the case of a 12-year-old boy with hematemesis and severe anemia that developed following an episode of acute pancreatitis. Upper endoscopy did not reveal a bleeding source. An endoscopic retrograde cholangiopancreatography performed for the evaluation of common bile duct obstruction identified bleeding from the pancreatic duct. Subsequently, the bleeding source, a pseudoaneurysm of the splenic artery, was identified by conventional angiography and occluded with coil embolization. The diagnosis of hemosuccus pancreaticus may be difficult in children due to rare occurrence and the unusual anatomical site; hence, a high index of suspicion is needed in a patient with a history of pancreatitis who presents with intermittent upper gastrointestinal bleeding and normal upper endoscopy.

Global Health Opportunities in Pediatric Fellowships.

BACKGROUND: Interest in global health (GH) among pediatric residents continues to grow. GH opportunities in pediatric fellowship programs in the United States are poorly described. We aimed to evaluate GH offerings among accredited general and subspecialty pediatric fellowship programs and identify implementation barriers. METHODS: This was a cross-sectional study by pediatric GH educators from the Association of Pediatric Program Directors Global Health Learning Community and the American Board of Pediatrics Global Health Task Force. Fellowship program directors and GH educators at accredited US pediatric fellowship programs were surveyed. Data were analyzed by using descriptive and comparative statistics. RESULTS: Data were obtained from 473 of 819 (57.8%) fellowship programs, representing 111 institutions. Nearly half (47.4%) offered GH opportunities as GH electives only (44.2%) or GH tracks and/or fellowships (3.2%) (GHT/Fs). Pretravel preparation and supervision were variable. Programs offering GH opportunities, compared to those without, were more likely to report that GH training improves fellow education (81.9% vs 38.3%; P < .001) and recruitment (76.8% vs 35.9%; P < .001). Since 2005, 10 programs with GHT/Fs have graduated 46 fellows, most of whom are working in GH. Of those with GHT/Fs, 71% believe national accreditation of GH fellowships would define minimum programmatic standards; 64% believe it would improve recruitment and legitimize GH as a subspecialty. CONCLUSIONS: GH experiences are prevalent in accredited US pediatric fellowship programs, and programs offering GH perceive that these opportunities improve fellow education and recruitment. Responses suggest that standards for GH opportunities during fellowship would be useful, particularly regarding pretravel preparation and mentorship for trainees.