A Novel Approach to Mentorship in Pediatric Cardiology: A Group for Women.

Gender disparities for female physicians in academic medicine are longstanding. Female pediatric cardiologists experience inequities in scholarship opportunities, promotion, leadership positions, and compensation. Mentorship groups have been successfully implemented in other subspecialities with promising results. We created a peer mentorship group for female pediatric cardiologists in the Northeast and completed a needs assessment survey of eligible participants. Our goal was to better understand the current challenges and identify resources to overcome these barriers. Our objectives were to (1) describe the creation of a novel mentorship program for female pediatric cardiologists and trainees in the Northeast United States, and (2) report the results of a formal needs assessment survey of all eligible participants. All female pediatric cardiology fellows and practicing pediatric and adult congenital heart disease specialists from 15 academic centers in New England were invited to join a free group with virtual meetings. A formal needs assessment survey was provided electronically to all eligible members. The vast majority of respondents agreed that the Women in Pediatric Cardiology (WIPC) group is a valuable networking and mentorship experience (90%) and would recommend this group to a colleague (95%). Members have witnessed or experienced inequities in a broad range of settings. Common challenges experienced by respondents include dependent care demands, lack of mentorship, inadequate research support, and inequitable clinical responsibilities. Resources suggested to overcome these barriers include mentorship, sponsorship, transparency in compensation, and physician coaching. Mentorship groups have the potential to address many challenges faced by women in medicine. The WIPC Northeast program provides a forum for community, collaboration, education, and scholarship.

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.

Cardiac MRI Findings in Male Patients with Acute Myocarditis in the Presence or Absence of COVID-19 Vaccination.

By comparing phenotypic clinical characteristics and cardiovascular magnetic resonance (CMR) findings in 14 patients with COVID-19 mRNA vaccine-associated myocarditis to 14 patients with acute myocarditis from other causes, we found that patients with COVID-19 vaccination- associated acute myocarditis have higher left ventricular ejection fraction, higher left ventricular global circumferential and radial strain, and less involvement of late gadolinium enhancement in the septal segments with less involvement of midmyocardial pattern of late gadolinium enhancement, compared to patients with acute myocarditis from other causes.

Clinically Suspected Myocarditis Temporally Related to COVID-19 Vaccination in Adolescents and Young Adults: Suspected Myocarditis After COVID-19 Vaccination.

BACKGROUND: Understanding the clinical course and short-term outcomes of suspected myocarditis after the coronavirus disease 2019 (COVID-19) vaccination has important public health implications in the decision to vaccinate youth. METHODS: We retrospectively collected data on patients <21 years old presenting before July 4, 2021, with suspected myocarditis within 30 days of COVID-19 vaccination. Lake Louise criteria were used for cardiac MRI findings. Myocarditis cases were classified as confirmed or probable on the basis of the Centers for Disease Control and Prevention definitions. RESULTS: We report on 139 adolescents and young adults with 140 episodes of suspected myocarditis (49 confirmed, 91 probable) at 26 centers. Most patients were male (n=126, 90.6%) and White (n=92, 66.2%); 29 (20.9%) were Hispanic; and the median age was 15.8 years (range, 12.1-20.3; interquartile range [IQR], 14.5-17.0). Suspected myocarditis occurred in 136 patients (97.8%) after the mRNA vaccine, with 131 (94.2%) after the Pfizer-BioNTech vaccine; 128 (91.4%) occurred after the second dose. Symptoms started at a median of 2 days (range, 0-22; IQR, 1-3) after vaccination. The most common symptom was chest pain (99.3%). Patients were treated with nonsteroidal anti-inflammatory drugs (81.3%), intravenous immunoglobulin (21.6%), glucocorticoids (21.6%), colchicine (7.9%), or no anti-inflammatory therapies (8.6%). Twenty-six patients (18.7%) were in the intensive care unit, 2 were treated with inotropic/vasoactive support, and none required extracorporeal membrane oxygenation or died. Median hospital stay was 2 days (range, 0-10; IQR, 2-3). All patients had elevated troponin I (n=111, 8.12 ng/mL; IQR, 3.50-15.90) or T (n=28, 0.61 ng/mL; IQR, 0.25-1.30); 69.8% had abnormal ECGs and arrhythmias (7 with nonsustained ventricular tachycardia); and 18.7% had left ventricular ejection fraction <55% on echocardiogram. Of 97 patients who underwent cardiac MRI at a median 5 days (range, 0-88; IQR, 3-17) from symptom onset, 75 (77.3%) had abnormal findings: 74 (76.3%) had late gadolinium enhancement, 54 (55.7%) had myocardial edema, and 49 (50.5%) met Lake Louise criteria. Among 26 patients with left ventricular ejection fraction <55% on echocardiogram, all with follow-up had normalized function (n=25). CONCLUSIONS: Most cases of suspected COVID-19 vaccine myocarditis occurring in persons <21 years have a mild clinical course with rapid resolution of symptoms. Abnormal findings on cardiac MRI were frequent. Future studies should evaluate risk factors, mechanisms, and long-term outcomes.

NOVEL XRCC4 MUTATIONS IN AN INFANT WITH MICROCEPHALIC PRIMORDIAL DWARFISM, DILATED CARDIOMYOPATHY, SUBCLINICAL HYPOTHYROIDISM, AND EARLY DEATH: EXPANDING THE PHENOTYPE OF XRCC4 MUTATIONS.

OBJECTIVE: Microcephalic primordial dwarfism (MPD) is a group of clinically and genetically heterogeneous disorders which result in severe prenatal and postnatal growth failure. X-ray repair cross-complementing protein 4 (XRCC4) is a causative gene for an autosomal recessive form of MPD. The objective of this report is to describe novel XRCC4 mutations in a female infant with MPD, dilated cardiomyopathy, and subclinical hypothyroidism. METHODS: Genetic testing was performed using a comprehensive next generation sequencing panel for MPD, followed by targeted XRCC4 gene sequencing. RESULTS: We report the case of a 970-gram, 35-cm, female infant (weight z score -5.05, length z score -4.71) born at 36 weeks and 3 days gestation. Physical examination revealed triangular facies, micrognathism, clinodactyly, and second and third toe syndactyly. Initial echocardiogram at birth was normal. Follow-up echocardiogram at 60 days of life revealed dilated cardiomyopathy with moderate left ventricular systolic dysfunction (ejection fraction was 40 to 45%), and anticongestive therapy was initiated. Thyroid testing revealed subclinical hypothyroidism with elevated thyroid-stimulating hormone of 13.0 µIU/mL (reference range is 0.3 to 5.0 µIU/mL) and normal free thyroxine by dialysis of 1.6 ng/dL (reference range is 0.8 to 2.0 ng/dL). Levothyroxine was initiated. Postnatal growth remained poor (weight z score at 3 months -4.93, length z score at 3 months -6.48), including progressive microcephaly (head circumference z score at 3 months -10.94). Genetic testing revealed novel compound heterozygous XRCC4 variants in trans: c.628A>T and c.638+3A>G. The child ultimately had cardiopulmonary arrest and died at 6 months of life. CONCLUSION: Molecular diagnosis in MPD is key to defining the natural history, management, and prognosis for patients with these rare disorders.

Work-Life Balance and Career Experiences of Part-Time Versus Full-Time Faculty at the Warren Alpert Medical School of Brown University.

INTRODUCTION: Part-time faculty are an important part of the academic medical workforce, comprising 11-21% of faculty in some institutions. OBJECTIVE: To describe the part-time faculty experience at a single institution across four domains: work-life balance, work environment, leadership and advancement, and mentorship. METHODS: Faculty from the Division of Biology and Medicine at Brown University were invited to participate in an electronic survey. The authors compared responses between full-time and part-time faculty across the four domains.   Results: Survey response rate was 43% (437/1025). Of the 363 who answered the question about employment status, 333 (92%) were full-time and 30 (8%) were part-time. Part-time faculty were less likely to report forgoing personal activities for professional responsibilities, that work conflicted with personal life, that their division director took interest in their careers, and having a leadership position was important to them. CONCLUSION: Part-time and full-time faculty reported significant differences in perception of work impact on personal life, division director support, and desire for leadership positions.

Maternal monitoring and safety considerations during antiarrhythmic treatment for fetal supraventricular tachycardia.

Fetal tachycardia is a rare complication during pregnancy. After exclusion of maternal and fetal conditions that can result in a secondary fetal tachycardia, supraventricular tachycardia is the most common cause of a primary sustained fetal tachyarrhythmia. In cases of sustained fetal supraventricular tachycardia, maternal administration of digoxin, flecainide, sotalol, and more rarely amiodarone, is considered. As these medications have the potential to cause significant adverse effects, we sought to examine maternal safety during transplacental treatment of fetal supraventricular tachycardia. In this narrative review we summarize the literature addressing pharmacologic properties, monitoring, and adverse reactions associated with medications most commonly prescribed for transplacental therapy of fetal supraventricular tachycardia. We also describe maternal monitoring practices and adverse events currently reported in the literature. In light of our findings, we provide clinicians with a suggested maternal monitoring protocol aimed at optimizing safety.

Aortic stiffness and left ventricular diastolic function in children with well-functioning bicuspid aortic valves.

AIMS: Aortic stiffness and diastolic function are abnormal in adults with bicuspid aortic valves (BAVs). The goal of this study was to determine the relationship between aortic stiffness and left ventricular (LV) diastolic impairment in children with well-functioning BAV and no associated congenital heart disease. METHODS AND RESULTS: This is a retrospective review of echocardiograms in children with isolated BAV (group BAV; N = 50) and healthy frequency-matched controls (group Control; N = 50). We analysed LV systolic and diastolic function, proximal and distal ascending aortic stiffness index (SI), distensibility, and strain. Age range was 0.2-20 (median 11) years. There was no significant difference in blood pressure, normalized LV size and systolic function between the groups. Several parameters of LV diastolic function were lower in group BAV compared with group Control (e.g. septal E': BAV 12 ± 2.3 cm/s; Control 13.5 ± 1.8 cm/s, P < 0.001). All parameters of proximal and distal ascending aortic elasticity were abnormal in group BAV vs. Control (SI proximal ascending aorta: BAV 4.2 ± 1.6; Control 3.0 ± 0.9, P < 0.001). There was no significant correlation between parameters of aortic elasticity and diastolic function. In a subgroup analysis of children with fusion of the right-non vs. right-left coronary cusps, there was no significant difference for any of the parameters analysed. CONCLUSION: Even children with well-functioning isolated BAV have abnormalities in aortic elasticity and diastolic function when compared with the Control group. However, a relationship between the two could not be established.

Aortic stiffness and left ventricular diastolic function in children following early repair of aortic coarctation.

Aortic stiffness and diastolic function are abnormal in adults with repaired coarctation of the aorta (CoA). The goal of this study was to determine the relation between aortic stiffness and left ventricular (LV) diastolic impairment in children who had undergone CoA repair very early in life. This is a retrospective review of echocardiograms in children with isolated repaired CoA (group CoA; n = 24) and healthy matched controls (group Normal; n = 24). We analyzed systolic and LV diastolic functions, proximal and distal ascending aortic stiffness indices (SIs), distensibility, and strain. Age range was 0.3 to 21 (median 9) years. Age at time of CoA repair was 0 to 24 (median 0.5) months. Median time since CoA repair was 6 years. There was no significant difference in blood pressure, LV size, and systolic function between the groups. LV diastolic function was impaired in group CoA compared with group Normal (septal E': CoA 10.3 ± 1.6 cm/s and Normal 13.4 ± 1.9 cm/s, p <0.001). All parameters of proximal and distal ascending aortic elasticities were abnormal in group CoA versus Normal (SI of proximal ascending aorta: CoA 4.9 ± 1.6 and Normal 2.7 ± 0.6, p <0.001). Across all patients, there was a strong correlation between septal E' and proximal ascending aortic SI (r = -0.72, p <0.001). In conclusion, even children who underwent CoA repair at a very young age have abnormal LV diastolic function and aortic elasticity compared with controls and there is a linear relation between the 2. LV diastolic dysfunction in patients with repaired CoA may be due to chronically increased afterload.