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Background In a recent multicenter study of perinatal outcome in fetuses with Ebstein anomaly or tricuspid valve dysplasia, we found that one third of live-born patients died before hospital discharge. We sought to further describe postnatal management strategies and to define risk factors for neonatal mortality and circulatory outcome at discharge. Methods and Results This 23-center, retrospective study from 2005 to 2011 included 243 fetuses with Ebstein anomaly or tricuspid valve dysplasia. Among live-born patients, clinical and echocardiographic factors were evaluated for association with neonatal mortality and palliated versus biventricular circulation at discharge. Of 176 live-born patients, 7 received comfort care, 11 died

This article discusses the chalk talk's potential as an active learning method. Although chalk talks are a form of interactive lecture, they have received little attention in the medical education literature compared with other active learning methods such as team-based learning and simulation. One of the authors (C. K. L. Phoon) has used chalk talks to teach congenital heart defects to first- and third-year NYU medical students for many years. His chalk talks have consistently earned among the highest teaching scores, and students have noted their strengths of being more interesting, clear, and tangible than didactic lectures. Using the teacher and student perspectives, we examine the chalk talk's strengths and weaknesses compared with common passive and active learning methods. Chalk talks create a real-time, shared space that facilitates the active learning goals of helping students build, test, and revise mental models (conceptual frameworks). The limited amount of information that can be presented and the ability to solicit and arrange students' ideas on the board lead to the cocreation of valuable conceptual frameworks. Chalk talks require less restructuring of teaching sessions than other active learning methods and are best suited to topics that hinge on understanding of concepts. We advocate for the chalk talk to be reexamined as a promising educational tool given its strengths and the successes that other active learning methods have shown. Furthermore, we provide guidance to help educators deliver chalk talks and discuss future studies that would advance understanding of this powerful teaching tool.

BackgroundEbstein anomaly and tricuspid valve dysplasia are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era.Methods and resultsFetuses diagnosed with Ebstein anomaly and tricuspid valve dysplasia from 2005 to 2011 were included from 23 centers. The primary outcome was perinatal mortality, defined as fetal demise or death before neonatal discharge. Of 243 fetuses diagnosed at a mean gestational age of 27±6 weeks, there were 11 lost to follow-up (5%), 15 terminations (6%), and 41 demises (17%). In the live-born cohort of 176 live-born patients, 56 (32%) died before discharge, yielding an overall perinatal mortality of 45%. Independent predictors of mortality at the time of diagnosis were gestational age

Cardiolipins are phospholipids that are central to proper mitochondrial functioning. Because mitochondria play crucial roles in differentiation, development, and maturation, we would also expect cardiolipin to play major roles in these processes. Indeed, cardiolipin has been implicated in the mechanism of three human diseases that affect young infants, implying developmental abnormalities. In this review, we will: (1) Review the biology of cardiolipin; (2) Outline the evidence for essential roles of cardiolipin during organismal development, including embryogenesis and cell maturation in vertebrate organisms; (3) Place the role(s) of cardiolipin during embryogenesis within the larger context of the roles of mitochondria in development; and (4) Suggest avenues for future research. Key Findings: As the "signature" phospholipid in mitochondria, we would expect cardiolipin to play a major role in developmental processes.Indeed, three human mitochondrial diseases now implicate cardiolipin primarily in their pathogenesis, affecting young infants and therefore displaying developmental abnormalities.Here, we will review the biology of cardiolipin, and the evidence for evolving and essential roles of cardiolipin during organismal development, including embryogenesis and cell maturation.In addition to studies in cellular model systems, we will establish that cardiolipin plays a role in vertebrate development and outline the evidence for possible underlying mechanisms. [ABSTRACT FROM AUTHOR]

Context : Cardiac injury has been described in both acute COVID-19 and the multisystem inflammatory syndrome in children (MIS-C). Echocardiographic strain has been shown to be a sensitive measure of systolic function. Aims : We sought to describe strain findings in both the groups on initial presentation and follow-up. Settings and Design : A retrospective study analyzing echocardiograms of all patients presenting with acute COVID-19 infection and MIS-C at our institution between March 2020 and December 2020 was performed. Subjects and Methods : TOMTEC software was used for strain analysis in both the study groups (COVID-19 and MIS-C) and age-matched healthy controls. Strain was correlated with LV ejection fraction (EF) and serum troponin levels. Results : Forty-five patients (34 -- MIS-C and 11 -- COVID-19) met the inclusion criteria. There was a statistically significant decrease in LV longitudinal strain (P < 0.001), LV circumferential strain (P < 0.001), and left atrial strain (P = 0.014) in the MIS-C group when compared to the control group. There was a statistically significant decrease in LV longitudinal strain (P = 0.028) in the acute COVID-19 group. All patients with abnormal left ventricular EF (LVEF) had abnormal strain. However, 14 (41%) patients in the MIS-C group and 3 (27%) in the acute COVID-19 group had preserved LVEF but abnormal strain. There was a significant correlation with LV longitudinal strain (P = 0.005) and LVEF (P = 0.002) and troponin in patients with MIS-C. Abnormal strain persisted in one-third of patients in the MIS-C and acute COVID-19 groups on outpatient follow-up. Conclusions : Patients with MIS-C and acute COVID-19 can develop myocardial dysfunction as seen by abnormal strain. LV longitudinal strain correlates with cardiac injury as measured by serum troponin in patients with MIS-C. Strain may provide an additional tool in detecting subtle myocardial dysfunction. It can be routinely employed at diagnosis and at follow-up evaluation of these patients. [ABSTRACT FROM AUTHOR]

Barth syndrome is a multisystem disorder caused by an abnormal metabolism of the mitochondrial lipid cardiolipin. In this review, we discuss physical properties, biosynthesis, membrane assembly, and function of cardiolipin. We hypothesize that cardiolipin reduces packing stress in the inner mitochondrial membrane, which arises as a result of protein crowding. According to this hypothesis, patients with Barth syndrome are unable to meet peak energy demands because they fail to concentrate the proteins of oxidative phosphorylation to a high surface density in the inner mitochondrial membrane. [ABSTRACT FROM AUTHOR]

Background: The overall severity of cardiac disease secondary to acute SARS-CoV-2 (severe acute respiratory syndrome coronavirus-2) infection in children appears to be much lower when compared with adults. However, the newly described multisystem inflammatory syndrome in children (MIS-C) associated with coronavirus disease 2019 (COVID-19) has been associated with cardiac complications. Methods: We reviewed the clinical course and cardiac testing results in pediatric patients hospitalized with MIS-C at 2 large hospital systems in the New York City metropolitan area over a 3-month period. Results: Of the 33 patients (median age 2.8 years) in the study cohort, 24 (73%) had at least one abnormality in cardiac testing: abnormal electrocardiogram (48%), elevated brain natriuretic peptide (43%), abnormal echocardiogram (30%), and/or elevated troponin (21%). Electrocardiogram and echocardiogram abnormalities all resolved by the 2-week outpatient follow-up cardiology visit. Conclusion: While 73% of pediatric patients with MIS-C had evidence of abnormal cardiac testing on hospital admission in our study, all cardiac testing was normal by outpatient hospital discharge follow-up. Cardiac screening tests should be performed in all patients diagnosed with MIS-C given the high rate of abnormal cardiac findings in our study cohort. [ABSTRACT FROM AUTHOR]

Pediatric heart failure remains poorly understood, distinct in many aspects from adult heart failure. Limited data point to roles of altered mitochondrial functioning and, in particular, changes in mitochondrial lipids, especially cardiolipin. Barth syndrome is a mitochondrial disorder caused by tafazzin mutations that lead to abnormal cardiolipin profiles. Patients are afflicted by cardiomyopathy, skeletal myopathy, neutropenia, and growth delay. A mouse model of Barth syndrome was developed a decade ago, which relies on a doxycycline-inducible short hairpin RNA to knock down expression of tafazzin mRNA (TAZKD). Our objective was to review published data from the TAZKD mouse to determine its contributions to our pathogenetic understanding of, and potential treatment strategies for, Barth syndrome. In regard to the clinical syndrome, the reported physiological, biochemical, and ultrastructural abnormalities of the mouse model mirror those in Barth patients. Using this model, the peroxisome proliferator-activated receptor pan-agonist bezafibrate has been suggested as potential therapy because it ameliorated the cardiomyopathy in TAZKD mice, while increasing mitochondrial biogenesis. A clinical trial is now underway to test bezafibrate in Barth syndrome patients. Thus the TAZKD mouse model of Barth syndrome has led to important insights into disease pathogenesis and therapeutic targets, which can potentially translate to pediatric heart failure. [ABSTRACT FROM AUTHOR]