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Approximately 1% of all live births in the USA are affected by congenital heart disease (CHD), the leading cause of congenital defect-related illness and infant death. Although technological innovations have improved CHD diagnosis in utero, variation among fetal cardiac counseling practices persists. Our study aims to evaluate physician counseling content based on cardiac defect complexity. We conducted an anonymous survey of providers who perform fetal cardiac counseling. Participants stated their counseling practices for 21 CHD lesions, choosing from postnatal surgical intervention, termination of pregnancy, and/or postnatal palliative comfort care. CHD lesions were divided by severity into three groups. Chi-square tests were performed to assess whether counseling content varied based on provider response to the statement "some life is always better than no life at all." There were 138 respondents, with postnatal surgical intervention counseling frequency consistent among all lesions. Inclusion of termination and palliative/comfort care in counseling increased in frequency as disease severity increased, with the former being counseled more frequently. For tetralogy of Fallot (p = 0.02), interrupted aortic arch (p = 0.03), hypoplastic left heart syndrome (p = 0.03), and pulmonary atresia with intact ventricular septum (p = 0.03), those providers who disagreed with the statement that "some life is always better than no life at all" were more likely to counsel termination of pregnancy and/or palliative care compared to their counterparts. Our study found that the interventions and options included in fetal cardiac counseling vary based on complexity. Moreover, physicians' personal beliefs tended to influence counseling practices in some moderate or severe defects., Competing Interests: Declarations. Conflict of interest: The authors declare no conflict of interests. Ethical Approval: The authors assert that all procedures contributing to this work comply with the ethical standards of the relevant national guidelines on human experimentation (please name) and with the Helsinki Declaration of 1975, as revised in 2008, and has been approved by the institutional committees., (© 2025. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: Fetal cardiac anomalies are the most commonly diagnosed structural anomalies. In these cases, Maternal-Fetal Medicine (MFM) specialists are tasked with counseling patients on a spectrum of diagnoses as well as their prognostic implications. A recent study of pediatric cardiologists demonstrated that personal beliefs regarding termination impact the counseling provided to patients. Our objective was to study whether the personal beliefs of MFMs impact counseling of patients with severe fetal cardiac anomalies and to compare these providers to their cardiology counterparts., Methods: We conducted an anonymous cross-sectional survey of MFMs in New England that assessed personal beliefs and counseling practices when diagnosing hypoplastic left heart syndrome (HLHS). We subsequently compared these providers to the previously surveyed cardiologists., Results: A total of 34 respondents representing a broad spectrum of age and experience across several states in New England were analyzed. When presented with the statement "some life is always better than no life at all," 79% ( n  = 27) of respondents disagreed and all respondents ( n  = 34) offered termination, palliative care, and treatment options when counseling patients with HLHS. Additionally, while 74% ( n  = 25) of providers would personally support a decision to terminate a pregnancy with HLHS, 94% ( n  = 32) would professionally support the decision to pursue termination.MFMs and cardiologists differed in their responses to "some life is better than no life" and the belief that termination should be offered, though differences did not reach statistical significance. However, with respect to the providers' personal and professional support of the decision to terminate the pregnancy, the groups of respondents varied significantly in their level of support, both professionally and personally with fewer cardiologists supporting this decision., Conclusion: When diagnosing a severe and potentially fatal congenital cardiac anomaly, counseling by MFMs was largely unaffected by personal beliefs regarding termination of pregnancy. While this is consistent with previously published data on counseling practices among pediatric cardiology specialists, some important differences between the specialties were seen.

Background: Prenatal counseling following a fetal diagnosis of critical congenital heart disease (CCHD) is challenging and requires delivery of detailed and complete information to ensure appropriate decision making and pregnancy support., Methods: An anonymous 50-item survey was distributed to providers who performed fetal cardiac counseling. The primary purpose was to evaluate on a national scale physician beliefs and attitudes toward available choices after a fetal diagnosis of CCHD and their association with reported counseling practices. Participants were specifically asked to indicate how likely they were to recommend postnatal interventional and/or surgical treatment, postnatal palliative care, or termination of pregnancy. The primary outcome variables were provider responses to statements about pregnancies complicated by CCHD diagnosed in utero., Results: There were 138 respondents, with most somewhat or strongly agreeing that providing counseling on surgical and catheter-based interventions anticipated in the short- and long-term (99%, 136/138), counseling on a "comfort care" alternative with limited intervention (97%, 134/138), and options for pregnancy termination at different stages (91%, 126/138) should be included. Physicians that disagreed with the statement "some life is better than no life at all" were statistically more likely (p < 0.001) to report that prenatal counseling regarding "options for pregnancy termination services at different stages of pregnancy" was important. There was a statistically significant difference in the counseling provided to parents based on the physician's personal and professional views on palliative care and termination of pregnancy., Conclusion: Physicians' self-reported beliefs impact the counseling content provided to families in the setting of a prenatal diagnosis of CCHD., (© 2024 John Wiley & Sons Ltd.)

Ebstein anomaly (EA) is a rare congenital heart defect characterized by abnormal development of the tricuspid valve (TV) and right ventricular myocardium. This study documents 2 dramatic cases of fetal EA characterized by hydrops and cardiomegaly, leading to intrauterine or early neonatal death. These clinical outcomes were associated with morphological abnormalities including severe tricuspid regurgitation, unguarded TV orifice, pulmonary atresia, and flattened right ventricular myocardium. This study highlights that these adverse anatomical features may result in unfavorable clinical outcomes in fetal EA. While timely identification of such features by prenatal ultrasound is crucial for providing accurate prognostic stratification and guiding treatment decisions, fetopsy may be necessary to discern EA among the spectrum of right-heart anomalies., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Pediatric cardiology fellows often deliver serious news to families. Effective clinician-patient communication is the basis of strong therapeutic relationships and improves health outcomes, increases patient adherence, and enhances patient satisfaction. Communication training improves physicians' communication skills, ability to deliver serious news, and meet the informational and emotional needs of patients and family members. However, there is little data surrounding pediatric cardiology fellows competencies or training in communication skills., Methods: Pediatric cardiology fellows participated in a 3-hour communication training session. The session used VitalTalk methodology and was facilitated by two VitalTalk facilitators. Fellows spent 1 h learning the skills of delivering serious news and responding to emotion and 2 h in role play with standardized actors followed by a brief group wrap-up activity. Participants took an anonymous, electronic pre- and post-survey and an 8-month follow-up survey via REDCap. Participants were asked about their preparedness and comfort performing certain communication skills and leading challenging conversations specific to pediatric cardiology. Response options used a combination of 0 (low comfort/preparedness) to 100 (high comfort/preparedness) point scales and multiple choice., Results: 9 fellows participated in the training and 100% completed all three surveys. Eight were first-year fellows and 1 was a third-year fellow. Finding the right words, balancing honesty with hope, and clinical and prognostic uncertainty were the top three factors that contributed to making conversations difficult. Following the course, there was a significant increase in fellow preparedness to communicate a new diagnosis of congenital heart disease, discuss poor prognoses, check understanding, and respond to emotion and an increase in fellow comfort responding to emotions. Four fellows reported using the skills from this training course in various clinical settings at 8-month follow up., Conclusions: Communicating serious news effectively is a skill that can be learned in a sustainable way and is essential in the field of pediatric cardiology. Our study demonstrates that an interactive, VitalTalk course can improve preparedness and comfort to deliver serious news in a cohort of pediatric cardiology trainees. Future studies are needed to evaluate translation of skills to clinical practice and durability of these skills in larger cohorts., (© 2024. The Author(s).)

The novel Coronavirus Disease 2019 (COVID-19), caused by the SARS-CoV-2 virus, created a need for evidence-based guidelines for the evaluation, management, and follow-up after infection. Data have become rapidly available, creating a challenge for medical providers to stay abreast of the ever-evolving recommendations. This document, written collaboratively by pediatric cardiovascular experts, pediatricians, and sports medicine specialists, is focused on SARS-- CoV-2-related pediatric cardiac manifestations. It aims to provide a systemic review of high-yield literature related to all cardiovascular entities as a tool for primary pediatric clinicians to utilize as they consider the cardiac consequences of acute SARS-CoV-2 infection, MIS-C, vaccine-related myocarditis, return-to-play, and long COVID-19 syndrome., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Advances in fetal cardiac imaging over the last few decades have allowed for increased prenatal detection and detailed counseling of congenital heart disease (CHD). When CHD is detected, fetal cardiologists are faced with the challenge of providing nuanced prenatal counseling. Studies in other specialties have shown that differences in physician attitudes exist around termination of pregnancy and correlate with variations in the counseling provided to parents. We conducted an anonymous cross-sectional survey of fetal cardiologists in New England (n = 36) regarding attitudes toward termination of pregnancy and the counseling provided to parents with a fetal diagnosis of hypoplastic left heart syndrome. Using a screening questionnaire, there was no significant difference in the counseling provided to parents regardless of the physician's personal or professional views on termination of pregnancy, age, gender, location, type of practice, or years of experience. There were, however, differences among physicians on reasons to consider termination and their perceived professional responsibility to the fetus or mother. Further investigation on a larger geographic scale may reveal additional insights on variations in physician beliefs and whether such beliefs affect variability in counseling practices., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: Impairments in the maternal-fetal environment are associated with adverse postnatal outcomes among infants with congenital heart disease. Therefore, we sought to investigate placental anomalies as they related to various forms of fetal congenital heart disease (FCHD)., Methods: We reviewed the placental pathology in singleton pregnancies with and without FCHD. FCHD was divided into separate categories (transposition physiology, obstructive left, obstructive right, biventricular without obstruction, and others). Exclusion criteria included other prenatally known structural malformations and/or aneuploidy. The significance threshold was set at p < 0.05 or False Discovery rate q < 0.05 when multiple tests were performed., Results: The cohort included 215 FCHD and 122 non-FCHD placentas. FCHD placentas showed increased rates of maternal vascular malperfusion (24% vs. 5%, q < 0.001) and cord anomalies (27% vs. 1%, q < 0.001). Placentas with fetal TGA demonstrated a lower rate of hypoplasia when compared with other FCHD types (1/39 vs. 51/176, Fisher's exact p = 0.015)., Conclusion: Placental maternal vascular malperfusion is increased in FCHD. The prevalence of vascular malperfusion did not differ by FCHD type, indicating that CHD type does not predict the likelihood of placental vascular dysfunction. Further investigation of the placental-fetal heart axis in FCHD is warranted given the importance of placental health., (© 2024 John Wiley & Sons Ltd.)

While significant progress has been made in reducing disparities within the US health care system, notable gaps remain. This article explores existing disparities within pediatric congenital heart disease care. Congenital heart disease, the most common birth defect and a leading cause of infant death, has garnered substantial attention, revealing certain disparities within the US health care system. Factors such as race, ethnicity, insurance coverage, socioeconomic status, and geographic location are all commonalities that significantly affect health disparities in pediatric congenital heart disease. This comprehensive review sheds light on disparities from diverse perspectives in pediatric care, demonstrates the inequities and inequalities leading to these disparities, presents effective solutions, and issues a call to action for providers, institutions, and the health care system. Recognizing and addressing these disparities is imperative for ensuring equitable care and enhancing the long-term well-being of children affected by congenital heart disease. Implementing robust, evidence-based frameworks that promote responsible and safe interventions is fundamental to enduring change.

Background: Prior literature has described an association between preeclampsia and offspring congenital heart disease (CHD), while suggesting there may be a stronger relationship in individuals with early preeclampsia., Objectives: The authors sought to explore the relationship between offspring CHD and preeclampsia among pregnancies in a population-based study., Methods: Retrospective cohort study all singleton pregnancies delivered in the state of California 2000 to 2012. We included singleton births with gestational ages of 23 to 42 weeks and excluded pregnancies complicated by pre-existing diabetes or identified fetal chromosomal anomalies. We used multivariable logistic regression to estimate ORs for associations between offspring CHD and preeclampsia. Further subanalyses examined the relationships in deliveries <34 weeks and >34 weeks to analyze if there was a difference according to timing of preeclampsia development., Results: Preeclampsia was strongly associated with offspring CHD (aOR: 1.38; 99% CI: 1.29-1.49) in the same pregnancy. Among patients with preeclampsia in the index pregnancy, there was an increased risk of fetal CHD in the subsequent pregnancy (aOR: 1.39; 99% CI: 1.20-1.61). Among patients with offspring CHD in the index pregnancy, there was an increased risk of preeclampsia in the subsequent pregnancy (aOR: 1.39; 99% CI: 1.15-1.68). In all 3 analyses, results remained significant when stratified by <34 weeks and ≥34 weeks., Conclusions: Our findings suggest a need for further investigation into the etiology of preeclampsia and its relationship to embryologic development of cardiovascular structures., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

Congenital heart disease (CHD) is increasingly diagnosed prenatally and the ability to screen and diagnose the genetic factors involved in CHD have greatly improved. The presence of a genetic abnormality in the setting of prenatally diagnosed CHD impacts prenatal counseling and ensures that families and providers have as much information as possible surrounding perinatal management and what to expect in the future. This review will discuss the genetic evaluation that can occur prior to birth, what different genetic testing methods are available, and what to think about in the setting of various CHD diagnoses.

Mental health conditions are a common comorbidity among children living with heart disease. Children with congenital heart disease are more likely to have a mental health condition than their unaffected peers or peers with other chronic illnesses, and mental health risk persists across their lifetime. While poorer mental health in adults with congenital heart disease is associated with worse overall health outcomes, the association between mental health and cardiac outcomes for children with heart disease remains unknown. Despite this, it is suspected that mental health conditions go undiagnosed in children with heart disease and that many affected children and adolescents do not receive optimal mental health care. In this article, we review mental health in congenital heart disease across the lifespan, across domains of care, and across diagnoses. Further directions to support mental health care for children and adolescents with heart disease include practical screening and access to timely referral and mental health resources., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Evidence from medicine and other fields has shown that gender diversity results in better decision making and outcomes. The incoming workforce of congenital heart specialists (especially in pediatric cardiology) appears to be more gender balanced, but past studies have shown many inequities. Gender-associated differences in leadership positions, opportunities presented for academic advancement, and recognition for academic contributions to the field persist. In addition, compensation packages remain disparate if evaluated based on gender with equivalent experience and expertise. This review explores these inequities and has suggested individual and institutional changes that could be made to recruit and retain women, monitor the climate of the institution, and identify and eliminate bias in areas like salary and promotions.

Objective: Trisomy 13 (T13) and 18 (T18) are aneuploidies associated with multiple structural congenital anomalies and high rates of fetal demise and neonatal mortality. Historically, patients with either one of these diagnoses have been treated similarly with exclusive comfort care rather than invasive interventions or intensive care, despite a wide phenotypic variation and substantial variations in survival length. However, surgical interventions have been on the rise in this population in recent years without clearly elucidated selection criterion. Our objective was to create a standardized approach to counseling expectant persons and parents of newborns with T13/T18 in order to provide collaborative and consistent counseling and thoughtful approach to interventions such as surgery., Study Design: This article describes our process and presents our resulting clinical care guideline., Results: We formed a multi- and interdisciplinary committee. We used published literature when available and otherwise expert opinion to develop an approach to care featuring individualized assessment of the patient to estimate qualitative mortality risk and potential to benefit from intensive care and/or surgeries centered within an ethical framework., Conclusion: Through multidisciplinary collaboration, we successfully created a patient-centered approach for counseling families facing a diagnosis of T13/T18. Other institutions may use our approach as a model for developing their own standardized approach., Key Points: · Trisomy 13 and trisomy 18 are associated with high but variable morbidity and mortality.. · Research on which patients are most likely to benefit from surgery is lacking.. · We present our institution's framework to counsel families with fetal/neonatal T13/T18.., Competing Interests: None declared., (Thieme. All rights reserved.)

Background: For infants with single ventricle heart disease, the time after stage 2 procedure (S2P) is believed to be a lower risk period compared with the interstage period; however, significant morbidity and mortality still occur., Objectives: This study aimed to identify risk factors for mortality or transplantation referral between S2P surgery and the first birthday., Methods: Retrospective cohort analysis of infants in the National Pediatric Cardiology Quality Improvement Collaborative who underwent staged single ventricle palliation from 2016 to 2022 and survived to S2P. Multivariable logistic regression and classification and regression trees were performed to identify risk factors for mortality and transplantation referral after S2P., Results: Of the 1,455 patients in the cohort who survived to S2P, 5.2% died and 2.3% were referred for transplant. Overall event rates at 30 and 100 days after S2P were 2% and 5%, respectively. Independent risk factors for mortality and transplantation referral included the presence of a known genetic syndrome, shunt type at stage 1 procedure (S1P), tricuspid valve repair at S1P, longer time to extubation and reintubation after S1P, ≥ moderate tricuspid regurgitation prior to S2P, younger age at S2P, and the risk groups identified in the classification and regression tree analysis (extracorporeal membrane oxygenation after S1P and longer S2P cardiopulmonary bypass time without extracorporeal membrane oxygenation)., Conclusions: Mortality and transplantation referral rates after S2P to 1 year of age remain high ∼7%. Many of the identified risk factors after S2P are similar to those established for interstage factors around the S1P, whereas others may be unique to the period after S2P., Competing Interests: Dr Bucholz is funded by a grant from the 10.13039/100005627Thrasher Research Fund to apply machine learning techniques for risk prediction of outcomes in single ventricle heart disease. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

Background: Timely prenatal diagnosis of CHD allows families to participate in complex decisions and plan for the care of their child. This study sought to investigate whether timing of initial fetal echocardiogram and the characteristics of fetal counselling were impacted by parental socio-economic factors., Methods: Retrospective chart review of fetal cardiac patients from 1 January, 2017 to 31 December, 2018. We reviewed gestational age at first fetal echo, maternal age and ethnicity, zip code, rurality index, and hospital distance. Counselling was evaluated based on documentation regarding use of interpreter, time billed for counselling, and treatment option chosen., Results: Total of 139 maternal-fetal dyads were included, and 29 dyads had single-ventricle heart disease. There was no difference in income, hospital distance or rurality index, and first fetal echo timing. There was no significant difference between maternal ethnicity and maternal age, gestational age at initial visit, or follow-up. Patients in rural areas had increased counselling time (p < .05). There was no difference between socio-economic factors and ultimate parental choices (termination, palliative delivery, or cardiac interventions)., Conclusion: Oregon comprises a heterogeneous population from a large geographical catchment. While prenatal counselling and family decision-making are multifaceted, we demonstrated that dyads were referred from across the state and received care in a uniformly timely manner, and once at our centre received consistent counselling despite differences in parental socio-economic factors.

Introduction: The prenatal diagnosis of congenital heart disease (CHD) is a traumatic event that can cause expectant parents to experience anxiety, depression, and toxic stress. Prenatal exposure to stress may impact neonatal postoperative outcomes. In addition, expectant parents may have other psychosocial stressors that may compound maternal stress. We investigated the relationship between stress in pregnancies complicated by prenatally diagnosed CHD and their neonatal outcomes., Methods: A pilot retrospective cohort study of pregnancies with prenatally diagnosed critical CHD (2019-2021) was performed. The collected data included pregnancy characteristics and neonatal and postoperative outcomes (including the need for exogenous corticosteroid treatment (ECT)). In order to quantify prenatal stressors, a composite prenatal stress score (PSS) was established and utilized., Results: In total, 41 maternal-fetal dyads were evaluated. Thirteen (32%) neonates had single-ventricle anatomy. The need for ECT after CHD surgery was associated with higher pregnant patient PSS ( p = 0.01). PSS did not correlate with birthweight, infection, or hypoglycemia in the neonatal period., Conclusions: Prenatal stress is multifactorial; higher PSS is correlates with post-bypass ECT, suggesting that a stressful intrauterine environment may be associated with worse neonatal postoperative outcomes.

Background Fetal diagnosis of congenitally corrected transposition of the great arteries (ccTGA) has been increasingly reported; however, predictors of clinical outcomes remain underexplored. We undertook a multicenter, retrospective study to investigate natural history, associated anomalies, and outcomes of fetal ccTGA. Methods and Results Fetuses with ccTGA diagnosed from January 2004 to July 2020 within 20 North American programs were included. Fetuses with severe ventricular hypoplasia thought to definitively preclude biventricular repair were excluded. We included 205 fetuses diagnosed with ccTGA at a median gestational age of 23 (interquartile range, 21-27) weeks. Genetic abnormalities were found in 5.9% tested, with extracardiac anomalies in 6.3%. Associated cardiac defects were diagnosed in 161 (78.5%), with atrioventricular block in 23 (11.3%). On serial fetal echocardiogram, 39% demonstrated a functional or anatomic change, most commonly increased tricuspid regurgitation (6.7%) or pulmonary outflow obstruction (11.1%). Of 194 fetuses with follow-up, 26 were terminated, 3 experienced fetal death (2 with atrioventricular block), and 165 were live-born. Of 158 with postnatal data (median follow-up 3.7 years), 10 (6.6%) had death/transplant before 1 year. On univariable analysis, fetal factors associated with fetal death or death/transplant by 1 year included ≥ mild tricuspid regurgitation, pulmonary atresia, aortic obstruction, fetal arrhythmia, and worsening hemodynamics on serial fetal echocardiogram (defined as worse right ventricular function, tricuspid regurgitation, or effusion). Conclusions Associated cardiac lesions and arrhythmias are common in fetal ccTGA, and functional changes commonly occur through gestation. Worse outcomes are associated with fetal tricuspid regurgitation (≥mild), any arrhythmia, pulmonary atresia, aortic obstruction, and worsening hemodynamics on serial echocardiograms. These findings can inform prenatal counseling and perinatal management planning.

Prenatal diagnosis of congenital heart disease (CHD) allows for thoughtful multidisciplinary planning about location, timing, and need for medical interventions at birth. We sought to assess the accuracy of our prenatal cardiac diagnosis, and postnatal needs for patients with CHD utilizing a multidisciplinary approach. We performed a retrospective chart review of fetal CHD patients between 1/1/18 and 4/30/19. Maternal and infant charts were reviewed for delivery planning, subspecialty care needs, genetic evaluation, prenatal and postnatal cardiac diagnoses, need for prostaglandin (PGE) and neonatal cardiac intervention. 82 maternal-fetal dyads met inclusion criteria during the study period and delivered at a median of 38w2d gestation. 32 (39%) dyads had CHD and other anomalies or genetic abnormalities. All dyads met with a genetic counselor and neonatologist. 11 patients delivered at outside hospitals as planned (all with isolated CHD not requiring neonatal intervention), and 5 chose a palliative delivery. 30 patients were counseled to expect a neonatal cardiac intervention and 25 (83%) underwent an intervention within the expected time period. No neonates required an uncounseled cardiac intervention. 29 patients planned for PGE at birth and 31 received PGE. Of the 79 postnatal echocardiograms, 60 (76%) were entirely consistent with the fetal diagnosis. A multidisciplinary approach to the prenatal diagnosis of CHD in maternal-fetal dyads is optimal and utilizing this method we were able to accurately predict postnatal physiology and ensure that patients delivered in the correct location with an appropriate supportive structure in place., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: We sought to identify associations between prenatal care coordination (PNC) and outcomes in hypoplastic left heart syndrome (HLHS)., Study Design: We hypothesized that suboptimal PNC is associated with worse pre-operative status. HLHS patients from 2016 through 2019 were identified using a multicenter registry. Optimal PNC was defined as (1) a completed interdisciplinary conference and (2) closed-loop communication with the obstetric team. Associations between PNC and outcomes were identified., Results: Of 1441 patients, 1242 (86%) had prenatal diagnosis. Among those with a prenatal diagnosis, PNC was achieved in only 845 (68%). Suboptimal PNC was associated with adverse events (50% vs 40%, p < 0.001), inotrope need (19% vs 13%, p = 0.007), mechanical ventilation (22% vs 16%, p = 0.016), and parenteral feeding (60% vs 46%, p < 0.001). African-American race and non-commercial insurance were associated with a lower likelihood of optimal PNC (p = 0.006 and p < 0.001, respectively)., Conclusion: Improving PNC and overcoming racial and socioeconomic barriers are important targets to improve HLHS perinatal care., (© 2022. The Author(s), under exclusive licence to Springer Nature America, Inc.)

Purpose: To assess the accuracy of maternal understanding of fetal cardiac defects following initial fetal counseling., Methods: Pregnant women with a fetal diagnosis of congenital heart disease (CHD) were surveyed regarding understanding of their fetus's heart defect. The survey asked: (1) for a description of the heart condition; (2) how confident they were in the diagnosis; (3) whether their fetus would require heart surgery. Two fetal cardiologists evaluated the maternal qualitative description. Partners were excluded from the study., Results: Fifty-one participants consented and 39 completed the survey. Mean age was 31 years, 60% had some college level or post-graduate education, 48% had Medicaid insurance, and 81% were Caucasian. More than three-quarters of participants, stated they had either "quite a bit" or "very much" understanding of their fetus's diagnosis. Maternal assessment matched the physician's assessment of accuracy with 77% ( N  = 30) demonstrating either "quite a bit" or a "very accurate" description of the diagnosis. All women correctly understood if their fetus would require heart surgery. Highest level of maternal education positively correlated with the accuracy of diagnosis (regression coefficient 0.48, p < .002). However, confidence in the diagnosis was independent of both education (0.30, p = .167) and maternal age (-0.03, p = .234)., Conclusions: Fetal counseling is effective in conveying anatomy and the need for surgery; however, accuracy amongst women with lower levels of education and maternal confidence in understanding can be improved.

Importance: Paediatricians play an integral role in the lifelong care of children with CHD, many of whom will undergo cardiac surgery. There is a paucity of literature for the paediatrician regarding the post-operative care of such patients., Observations: The aim of this manuscript is to summarise essential principles and pertinent lesion-specific context for the care of patients who have undergone surgery or intervention resulting in a biventricular circulation., Conclusions and Relevance: Familiarity with common issues following cardiac surgery or intervention, as well as key details regarding specific lesions and surgeries, will aid the paediatrician in providing optimal care for these patients.

Importance: Single ventricle CHD affects about 5 out of 100,000 newborns, resulting in complex anatomy often requiring multiple, staged palliative surgeries. Paediatricians are an essential part of the team that cares for children with single ventricle CHD. These patients often encounter their paediatrician first when a complication arises, so it is critical to ensure the paediatrician is knowledgeable of these issues to provide optimal care., Observations: We reviewed the subtypes of single ventricle heart disease and the various palliative surgeries these patients undergo. We then searched the literature to detail the general paediatrician's approach to single ventricle patients at different stages of surgical palliation., Conclusions and Relevance: Single ventricle patients undergo staged palliation that drastically changes physiology after each intervention. Coordinated care between their paediatrician and cardiologist is requisite to provide excellent care. This review highlights what to expect when these patients are seen by their paediatrician for either well child visits or additional visits for parental or patient concern.

In this survey study of institutions across the US, marked variability in evaluation, treatment, and follow-up of adolescents 12 through 18 years of age with mRNA coronavirus disease 2019 (COVID-19) vaccine-associated myopericarditis was noted. Only one adolescent with life-threatening complications was reported, with no deaths at any of the participating institutions., (Copyright © 2021 Elsevier Inc. All rights reserved.)

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.

Late diagnosis of critical congenital heart disease (CCHD) is associated with higher levels of morbidity and mortality in neonates. Nearly all states have passed laws mandating universal pulse oximetry screening (POxS) of newborns to improve early detection rates of CCHD. We performed a retrospective chart review of all transthoracic echocardiograms (TTEs) interpreted by our institution on patients between 0 and 30 days of life in the years 2010 (prior to POxS) and 2015 (after POxS). Between 2010 and 2015, the number of neonatal TTEs interpreted by our institution decreased by 18.2%. In 2015, there were 46 neonates diagnosed with CCHD with a 78% prenatal detection rate. There was only one case of a true-positive POxS. Our study demonstrated that the initiation of POxS coincided with a significant decrease in neonatal TTEs, suggesting universal POxS may impart reassurance to primary providers leading to a decrease in TTE utilization.

Due to the COVID-19 pandemic, there has been an increased interest in telehealth as a means of providing care for children by a pediatric cardiologist. In this article, we provide an overview of telehealth utilization as an extension of current pediatric cardiology practices and provide some insight into the rapid shift made to quickly implement these telehealth services into our everyday practices due to COVID-19 personal distancing requirements. Our panel will review helpful tips into the selection of appropriate patient populations and specific cardiac diagnoses for telehealth that put patient and family safety concerns first. Numerous practical considerations in conducting a telehealth visit must be taken into account to ensure optimal use of this technology. The use of adapted staffing and billing models and expanded means of remote monitoring will aid in the incorporation of telehealth into more widespread pediatric cardiology practice. Future directions to sustain this platform include the refinement of telehealth care strategies, defining best practices, including telehealth in the fellowship curriculum and continuing advocacy for technology.

Objectives: Critical pulmonary stenosis (PS) and pulmonary atresia with intact ventricular septum (PAIVS) require urgent neonatal intervention. Since PS may be more insidious than PAIVS during gestation, we hypothesized that neonates with PS would have lower rates of prenatal detection than PAIVS., Methods: We performed a retrospective chart review of all neonates who underwent diagnostic or interventional cardiac catheterization between 2000 and 2014 for critical PS or PAIVS. The rates of prenatal diagnosis were calculated for PS and PAIVS. Prenatal and postnatal echocardiographic data were reviewed., Results: 178 patients met inclusion criteria: 91 with critical PS and 87 with PAIVS. The prenatal diagnosis rate for critical PS was lower than for PAIVS at 37% (34/91) vs 60% (52/87) (P = .003). At the time of diagnosis at a median gestational age of 25 weeks, the median TV z-score for patients with critical PS was larger than in PAIVS (-0.15 vs -3.0 P = .004)., Conclusion: Critical PS had a lower prenatal detection rate than PAIVS, likely due to a relatively normal 4-chamber view at the time of routine second trimester screening in patients with PS. Color flow Doppler of the outflow tracts may improve detection, since outflow tracts may appear normal by 2D imaging., (© 2020 John Wiley & Sons, Ltd.)

Objective: Hypoplastic left heart syndrome is a single ventricle defect. While staged surgical palliative treatments have revolutionised care, patients with hypoplastic left heart syndrome continue to have significant morbidity and mortality. In 2017, the National Pediatric Cardiology Quality Improvement Collaborative recommended all single ventricle patients to receive a prenatal palliative care consult. This study aimed to elucidate provider perspectives on the implementation of prenatal palliative care consults for families expecting a child with hypoplastic left heart syndrome., Methods: An online survey was administered to obstetric and paediatric providers of relevant disciplines to assess their experience with palliative care involvement in hypoplastic left heart syndrome cases., Results: Nearly, all physicians (97%) and most registered nurses (79%) agreed that the initial palliative care consult for patients with hypoplastic left heart syndrome should occur during the prenatal period. Respondents also indicated that prenatal palliative care consults should also be offered in a variety of other CHD conditions. Participants believed positive aspects of this new referral protocol included an expanded support network for families, decreased family stress during the postnatal period, increased patient education about what to expect during the postnatal period, and continuity of care., Conclusion: Multidisciplinary healthcare professionals believe that prenatal palliative care consults provide a variety of benefits for patients and families with hypoplastic left heart syndrome. Additional, multi-centre research is necessary to evaluate whether prenatal palliative care consults should become standard of care for families expecting a child with a single ventricle defect.

The objective of this study was to evaluate the utility of transthoracic echocardiography (TTE) in children with structurally normal hearts suspected of having infective endocarditis (IE). We hypothesized that the diagnostic yield of TTE is minimal in low-risk patients with normal hearts. We performed a retrospective chart review of TTEs performed for concern for endocarditis at a pediatric tertiary care referral center in Portland, Oregon. Three hundred patients met inclusion criteria (< 21 years old, completed TTE for IE from 2005 to 2015, no history of congenital heart disease or endocarditis). We recorded findings that met the modified Duke criteria (MDC) including fever, positive blood culture, and vascular/immunologic findings; presence of a central line; whether or not patients were diagnosed with IE clinically; and if any changes to antibiotic regimens were made based on TTE. Ten patients (3%) had echocardiograms consistent with IE. When compared to the clinical diagnosis of IE, the positive predictive value (PPV) of one positive blood culture without other major/minor MDC was 0. Similarly, the PPV of two positive blood cultures without other major/minor criteria was 0.071. Patients should be evaluated using the MDC to assess the clinical probability of IE prior to performing a TTE. Patients with a low probability for IE should not undergo TTE as it has a low diagnostic yield and patients are unlikely to be diagnosed with disease.

Objectives: Children with tetralogy of Fallot (TOF) require initial intervention at varying times reflecting a spectrum of obstruction to pulmonary blood flow. In utero, fetal patients with right sided obstructive lesions demonstrate increased middle cerebral artery (MCA) pulsatility index (PI). We sought to determine whether MCA flow patterns are associated with the degree of pulmonary obstruction and the consequential need for early surgical intervention (ESI)., Methods: We performed a multi-center retrospective study evaluating patients with a prenatal diagnosis of TOF. All echocardiographic fetal studies performed from 16 weeks' gestational age through delivery were reviewed between 2001 and 2015 with subsequent postnatal medical chart review., Results: 82 patients met inclusion criteria. Patients who ultimately required ESI (n=30) demonstrated an increased MCA PI z-score (-0.68 vs -1.32, p=0.02) compared to those who did not (n=52). An elevated MCA PI in patients with TOF was associated with ESI after 24 weeks' gestational age (-0.36 vs -1.22, p=0.02)., Conclusions: Fetal patients with TOF demonstrate altered MCA flow that correlates with need for ESI. This effect is blunted earlier in gestation which may reflect cerebral autoregulation. MCA PI may be a beneficial tool in helping determine perinatal management and counseling for patients with TOF., (© 2019 John Wiley & Sons, Ltd.)

Background: Early detection of right ventricular dysfunction after transannular patch for tetralogy of Fallot (TOF-TAP) is essential for management., Objectives: To evaluate echocardiographic metrics of ventricular function correlate with functional MRI measurements, in patients with TOF-TAP., Methods: A retrospective review of patients with TOF-TAP between 2007 and 2017 who had an echocardiogram and MRI within six months were analyzed. Systolic to diastolic ratio (SD ratio) was measured from the tricuspid regurgitation and adjusted for heart rate. Tricuspid Annular Plane Systolic Excursion (TAPSE), Fractional Area Change (FAC), and shortening fraction (SF) were additionally measured. Echocardiographic measurements were correlated with MRI assessment of right ventricular ejection fraction (RVEF), left ventricular ejection fraction (LVEF), right ventricular end-diastolic volume index (RVEDi), and right to left ventricle volume ratio (RV/LV)., Results: Of the 53 patients (mean age 12.8 years) that met inclusion criteria, 45 (85%) had available TR jets for SD ratio analysis. The HR adjusted SD ratio negatively correlated with RVEF (r = -.359, P = .016), LVEF (r = -.317, P = .038) and positively with RV/LV EDV ratio (r = .347, P = .024). TAPSE, FAC, and SF measurements did not show significant correlation., Conclusion: In patients with TOF-TAP, there is a moderate negative correlation between heart rate adjusted SD ratio and MRI metrics of ventricular function, suggesting that decreased filling time is a marker for reduced right ventricular function. The SD ratio may be a useful echocardiographic tool for serial evaluation of in this population., (© 2019 Wiley Periodicals, Inc.)

Transient left ventricular (LV) dysfunction occurs in some infants born with critical pulmonary stenosis (PS) or membranous pulmonary atresia with intact ventricular septum (PAIVS) after pulmonary valve (PV) balloon dilation (BD). The cause for this is not well understood. We sought to characterize this LV dysfunction by investigating regional differences in this cohort using myocardial strain imaging. Patients who underwent neonatal (<2 weeks age) PV BD for critical PS or PAIVS from Jan, 2007 to March, 2014 with echocardiographic images suitable for strain analysis were identified; infants with ≥moderate post-BD LV dysfunction (ejection fraction <40%, n = 8) were matched 1:1 with controls who underwent PV BD but did not develop LV dysfunction. Longitudinal and circumferential global and segmental strain were analyzed before and after PV BD. For the 8 LV dysfunction cases, LV global longitudinal strain worsened after PV BD (-16% pre- vs -8% post-PV BD, p = 0.008) with similar impairment in global LV circumferential strain (-17% vs -8%, p = 0.008); there was no significant change in RV global or segmental longitudinal strain pre- vs post-PVBD. No significant pre/post-BD differences in global or circumferential strain were found in control pts. Segmental analysis of longitudinal and circumferential LV strain before and after balloon dilation in cases demonstrated decreased strain in all segments, but more pronounced and statistically significant in septal segments as compared with the free wall. In conclusion, transient LV dysfunction post-PV BD for critical PS/PAIVS is characterized by impaired global longitudinal and circumferential LV strain, with the most significant reductions in strain at the interventricular septum; longitudinal RV strain remains unchanged. These findings suggest that the mechanism of LV dysfunction post-PV BD is adverse ventricular-ventricular interactions specifically involving the interventricular septum., (Copyright © 2018. Published by Elsevier Inc.)

Background: In 2003, work-hour regulations were implemented by the Accreditation Council for Graduate Medical Education. Much has been published regarding resident rest and quality of life as well as patient safety. There has been no examination on the effect of work-hour restrictions on academic productivity of fellows in training. Paediatric subspecialty fellows have a scholarly requirement mandated by the American Board of Pediatrics. We have examined the impact of work-hour restrictions on the scholarly productivity of paediatric cardiology fellows during their fellowship., Methods: We conducted a literature search for all paediatric cardiology fellows between 1998 and 2007 at a single academic institution as first or senior authors on papers published during their 3-year fellowship and 3 years after completion of their categorical fellowship (n=63, 30 fellows before 2003 and 33 fellows after 2003). The numbers of first- or senior-author fellow publications before and after 2003 were compared. We also collected data on final paediatric cardiology subspecialty career choice., Results: There was no difference in the number of fellow first-author publications before and after 2003. Before work-hour restrictions, the mean number of publications per fellow was 2.1 (±2.2), and after work-hour restrictions it was 2.0 (±1.8), (p=0.89). By subspecialty career choice, fellows who select electrophysiology, preventative cardiology, and heart failure always published within the 6-year time period., Conclusions: Since the implementation of work-hour regulations, total number of fellow first-authored publications has not changed. The role of subspecialty choice may play a role in academic productivity of fellows in training.

Introduction New paediatric cardiology trainees are required to rapidly assimilate knowledge and gain clinical skills to which they have limited or no exposure during residency. The Pediatric Cardiology Fellowship Boot Camp (PCBC) at Boston Children's Hospital was designed to provide incoming fellows with an intensive exposure to congenital cardiac pathology and a broad overview of major areas of paediatric cardiology practice., Methods: The PCBC curriculum was designed by core faculty in cardiac pathology, echocardiography, electrophysiology, interventional cardiology, exercise physiology, and cardiac intensive care. Individual faculty contributed learning objectives, which were refined by fellowship directors and used to build a programme of didactics, hands-on/simulation-based activities, and self-guided learning opportunities., Results: A total of 16 incoming fellows participated in the 4-week boot camp, with no concurrent clinical responsibilities, over 2 years. On the basis of pre- and post-PCBC surveys, 80% of trainees strongly agreed that they felt more prepared for clinical responsibilities, and a similar percentage felt that PCBC should be offered to future incoming fellows. Fellows showed significant increase in their confidence in all specific knowledge and skills related to the learning objectives. Fellows rated hands-on learning experiences and simulation-based exercises most highly., Conclusions: We describe a novel 4-week-long boot camp designed to expose incoming paediatric cardiology fellows to the broad spectrum of knowledge and skills required for the practice of paediatric cardiology. The experience increased trainee confidence and sense of preparedness to begin fellowship-related responsibilities. Given that highly interactive activities were rated most highly, boot camps in paediatric cardiology should strongly emphasise these elements.

Background: Coronary artery (CA) Z scores are commonly used for clinical decisions in Kawasaki disease, including treatment, anticoagulation, and duration and frequency of follow-up. The aim of this study was to evaluate CA measurement reproducibility, Z score calculation variability, and the impact of variability on management., Methods: Twenty-one patients with Kawasaki disease with right CA (RCA) or left anterior descending CA (LAD) Z scores of 1.5 to 3 (group 1) were randomly selected, and all patients with Kawasaki disease with Z scores of 7 to 14 for either the RCA or LAD (n = 20; group 2) were included from March 2008 to May 2014. Two echocardiographers measured left main CA, LAD, and RCA dimensions. The inter- and intraobserver reliability of absolute measurements was calculated, and the CA Z scores derived from three commonly used formulas were compared., Results: Median age at echocardiography was 1.2 years (range, 0.2-11.5 years), and 68% of subjects (n = 28) were male. Interobserver reliability was high for the LAD (intraclass correlation coefficient [ICC], 96.79%) and RCA (ICC, 93.31%) and lower for the left main CA (ICC, 73.54%). Intraobserver reliability was also high for the LAD and RCA (ICC, 99.08% and 97.74%) and lower for the left main CA (ICC, 80.88%). Calculated Z scores were similar among the three formulas for group 1 but varied markedly in group 2. Calculated Z scores using the same CA measurement in each of the three formulas resulted in different clinical management in up to seven of 21 group 1 patients (22%) and in up to 10 of 20 group 2 patients (50%)., Conclusions: Although CA measurements have high inter- and intraobserver agreement, CA Z scores vary dramatically on the basis of the Z score formula at larger CA dimensions. Discrepancies in CA Z score calculators may affect clinical decision making., (Copyright © 2016 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

Pulmonary valve (PV) balloon dilation (BD) is the primary therapy for infants born with critical pulmonary stenosis (PS) or membranous pulmonary atresia with intact ventricular septum (PAIVS). We observed left ventricular (LV) dysfunction in patients following BD and sought to determine its incidence, clinical course and associated risk factors. Clinical, echocardiographic and catheterization data for all patients who underwent neonatal (<2 weeks age) PV BD for critical PS or PAIVS between January 2000 and February 2014 were retrospectively analyzed (n = 129). Post-procedure LV dysfunction was defined as ejection fraction (EF) <54 %. Median age at PV BD was 1 day. Most (71 %) patients had critical PS. Median PV diameter pre-BD was 6.0 mm with PV z-scores -4.1 to 0.9, median LV EF pre-BD was 58 %. Post-BD LV dysfunction developed in 45 patients (35 %); 15 patients had LV EF ≤40 %. Median time to normalization of LV EF was 10 days (range 2-72). In univariate analysis, diagnosis (critical PS or PAIVS), right ventricle to LV pressure ratio pre-BD, acute procedural complication and post-BD inotropic support were not associated with post-BD LV dysfunction. In multivariable analysis, the predictors of post-procedure LV dysfunction were lower PV z-score (OR 1.81, p 0.04), tricuspid regurgitation pre-BD ≥ moderate (OR 3.73, p 0.008) and larger right ventricular apical area (OR 1.99, p 0.04). LV dysfunction post-neonatal PV BD develops in a significant number of patients (35 %) and can be severe, but resolves. The risk of developing LV dysfunction post-PV BD is highest in patients with larger right ventricles.

In this article I have explored why I love and hate my mother. It is a retrospective and ongoing participant observation of the phenomenon of being the daughter of a mother with mental retardation. In it, I make use of a layered account--an experimental, postmodern, ethnographic reporting format that enables researchers to use varied resources, such as social theory, lived experience, and emotions. By using my own experience, I explore, through first-person narrative, the complex issues and emotions involved. My conclusion is that the situation is fraught with ambivalence because my present interactions with my mother are cast in the light of a past where my mother simultaneously neglected and protected me.