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Over 4 decades, short- and long-term survival for children with congenital heart disease (CHD) has significantly increased. Those with complex CHD have a 1-year mortality rate of approximately 25%. Prenatal access to palliative care ensures a comprehensive approach to the infant's physical, emotional, spiritual, and psychosocial well-being and offers enhanced support for parents and families while providing an interprofessional approach to care. Pediatric cardiac provider attitudes, knowledge, patterns of prenatal palliative care, and consultation for fetuses with complex heart disease to guide increased and appropriate prenatal consultation were explored for a quality improvement project completed at a single institution. A retrospective cardiac database review identified that 21.5% of maternal patients seen prenatally and carrying a fetus with complex CHD would have qualified for prenatal palliative care consultation. To increase prenatal palliative care consultation, a pathway for referral was developed. A pre-evaluation of pediatric cardiac provider attitudes regarding palliative care was measured. Survey data informed the intervention phase of prenatal palliative care educational material development, which was presented to pediatric cardiac providers. The number of prenatal palliative care consults was tracked. A postintervention survey completed by pediatric cardiac providers showed increased understanding and utilization of palliative care for comfort and support and not just reserved for death preparation. Greater than 93% agreed or strongly agreed that they gained a better understanding of appropriate use of palliative/supportive care. As pediatric cardiac providers become more knowledgeable about early integration of palliative care, parents and infants will benefit greatly from referrals and initiation of palliative care at the time of prenatal diagnosis.

Multiple studies have shown that quantitative evaluation of right ventricular (RV) systolic function in children with hypoplastic left heart syndrome (HLHS) is associated with outcomes. However, the most widely used method is qualitative, or subjective echocardiographic evaluation. Tricuspid annular displacement (TMAD) is a quantitative method and has been shown to be associated with transplant/death in a cohort of pediatric patients with HLHS. In this study, the same echocardiograms used in the quantitative TMAD study were qualitatively evaluated to see if the assessment correlated with midterm outcomes. We hypothesized that TMAD measures would outperform qualitative measurements. A previously published retrospective study of patients with systemic right ventricle demonstrated that TMAD measurements of function prior to the Glenn procedure were associated with midterm mortality/transplant (mean TMAD 12.4% for survivors and 10.0% for non-survivors/transplant, p = 0.03). Echocardiographic images used in that study were re-evaluated using qualitative assessment of function. A score between severely depressed (0) and supra-normal (7) was assigned independently by three cardiologists. A chart review included short-term clinical outcome measures in addition to midterm mortality/transplant. Spearman correlations and logistic regression were used to estimate the associations between function scores and clinical outcomes. Function scores were measured with acceptable inter- and intra-rater reliabilities in 47 patients who had echocardiograms used in the prior analysis before their bidirectional Glenn. The mean functional score was 5.3 ± 1.32 in survivors and 5.0 ± 1.02 in non-survivors/transplant. Subjective echocardiographic scores of function were not predictive of mortality (p = 0.23). Scores were correlated with length of cardiac intensive care unit stay (Spearman's rho = - 0.31, p = 0.04), but not post-Glenn total length of hospital stay (p = 0.4). A sub-analysis was performed for each individual operator. Only one of three operators produced scores with a significant association with mortality/transplant (p = 0.01, p = 0.25, p = 0.22, respectively). Averaged subjective measurement of function by three pediatric cardiologists in children with single right ventricle prior to the Glenn procedure was not associated with midterm outcomes. Previous work based on the same echocardiograms showed that quantitative analysis was associated with midterm outcomes. This suggests quantitative analysis may be more useful in prognostication. Future studies could confirm these results and identify which quantitative methods are most helpful.

To identify caregivers' views on preferred surrogate decision makers for their children.A respondent-anonymous survey was distributed to a convenience sample of adults who accompanied a child to general and subspecialty pediatric care at 2 different institutions or were at the bedside of a child in the pediatric intensive care unit at a third institution in Chicago.We collected 462 valid surveys. The average age of the legal guardian and accompanying child was 36.8 years and 6.6 years, respectively. Most legal guardians designated "other parent with legal authority" as their first choice surrogate decision maker (70%). Respondent's sex, respondent's age, child's age, and child's ethnicity had no effect on first choice surrogate decision maker. "Other parent with legal authority" was less likely to be first choice surrogate if respondents had Medicaid insurance, less than a college degree, or lived in a non-nuclear household (P.01 for all factors). The surrogacy ladder selected by 31% of legal guardians was "other parent with legal authority," "child's grandparent(s)," and "child's aunt(s) or uncle(s)." No other sequence received more than 10% designation. Study site had no effect on surrogate preference (P = .30).A surrogacy priority ladder for minors needs to include relatives who are often not included in state surrogacy statutes (eg, grandparents, aunts and uncles). The most popular surrogacy ladder will not be ideal for many families. Parents need to be informed and empowered to choose alternate surrogates, and documented preferences must be easily and widely accessible.

Background Coronavirus disease 2019 (COVID-19) vaccine hesitancy in healthcare workers (HCWs) contributes to personal and patient risk in contracting COVID-19. Reasons behind hesitancy and how best to improve vaccination rates in HCWs are not clear. Methods We adapted a survey using the Health Belief Model framework to evaluate HCW vaccine hesitancy and reasons for choosing for or against COVID-19 vaccination. The survey was sent to three large academic medical centers in the Chicagoland area between March and May 2021. Results We received 1974 completed responses with 85% of HCWs receiving or anticipating receiving COVID-19 vaccination. Multivariable logistic regression found HCWs were less likely to receive COVID-19 vaccination if they were Black (OR 0.34, 95% CI 0.15-0.80), Republican (OR 0.54, 95% CI 0.31-0.91), or allergic to any vaccine component (OR 0.27, 95% CI 0.10-0.70) and more likely to receive if they believed people close to them thought it was important for them to receive the vaccine (OR 5.2, 95% CI 3-8). Conclusions A sizable number of HCWs remain vaccine hesitant one year into the COVID-19 pandemic. As HCWs are positively influenced by colleagues who believe COVID-19 vaccination, development of improved communication across HCW departments and roles may improve vaccination rates.

As COVID-19 related hospitalizations surged in other countries and began to rise sharply in the United States, hospitals, healthcare systems, and states began to grapple with the terrifying questio...

A couple and their five-year-old daughter are in a car accident. The parents are not expected to survive. The child is transported to a children's hospital, and urgent treatment decisions must be made. Whom should the attending physician approach to make decisions for the child? When such cases arise in, for example, the hospitals where we work, the social worker or chaplain is instructed to use the Illinois Health Care Surrogacy Act as a guidepost to identify a decision-maker. But in our state and the country overall, the limitations of such statutes leave hospital workers to make a judgment call among friends, family, and clergy who may come forward. While surrogate decision-making statutes comprehensively address surrogate decision-makers for adults, a patchwork of laws-permanency statutes, kinship provider statutes, standby guardianship statutes, and, in some cases, surrogate decision-making statutes-provide variable decision-making pathways for children.

Background:Tetralogy of Fallot is a congenital heart defect diagnosed in infancy. Assessment of right ventricular size and function is important for evaluation of patients with tetralogy of Fallot, but these quantitative measures are challenging by echocardiography. This study evaluates a semi-automated software (EchoInsight®, Epsilon Imaging) by comparing its measures to manual measures in children with tetralogy of Fallot.Methods:Echocardiographic measurements were performed using manual techniques and semi-automated software. Right ventricular measurements included end-diastolic and end-systolic area, fractional area change, chamber dimensions, and tricuspid annular plane systolic excursion. Reliability, correlation, and agreement between manual and semi-automated measures were assessed.Results:Echocardiograms for 46 patients were analysed. Intra- and inter-observer reliabilities for semi-automated measures were good with intraclass correlation coefficients all over 0.95 and 0.85, respectively. There was high correlation between manual and semi-automated methods for areas and dimensions (r = 0.91–0.98). Tricuspid annular plane systolic excursion measures and fractional area change also correlated, albeit less strongly. The semi-automated measurements of end-systolic and end-diastolic area were a 20 and 47% higher than manual methods, respectively.The semi-automated method yielded a relative 52% lower fractional area change compared to the manual method.Conclusions:The semi-automated software generates quantitative right ventricular measures in children with tetralogy of Fallot with good reliability and good correlation with manual methods for all measures, but with significant difference between manual and semi-automated techniques for area and functional measures. The specific right ventricular geometry in tetralogy of Fallot children may be why, compared to normal anatomy, greater differences were observed between the two techniques.

Objective Medical education experienced widespread disruption during the first peak of the COVID-19 pandemic. In response, members of the Society of Pediatric Cardiology Training Program Directors (SPCTPD) created a series of live, Zoom-based lectures for pediatric cardiology fellows. The goals were to support fellow didactic education and connect fellows and faculty during the pandemic. We sought to determine the impact of the lecture series on pediatric cardiology fellows around the country. Methods Pediatric cardiology experts from across the country volunteered to give lectures in the Pediatric Cardiology National Education Series (PC-NES). Lectures were held online once or twice weekly for 3 months during Spring 2020, while many fellows had modified clinical responsibilities. Fellows voluntarily completed an online survey before and after the lecture series. Results On the initial survey, a majority of fellows responded they would benefit from more didactic education while their training was impacted by COVID-19. After the series concluded, almost all (95%) fellows responded that PC-NES lectures were valuable supplements to their training during COVID-19. They included quality of lectures, board-relevance, and a way to connect to the pediatric cardiology community as key reasons for participating in PC-NES, and 100% responded they felt PC-NES should continue in some capacity. Conclusion The PC-NES was a low-cost, simple way to deliver high-quality supplemental education to pediatric cardiology fellows, help trainees build a network of peers and learn from experts outside their home institution. Similar models could connect other pediatric trainees at local, regional, or national levels, and provide valuable supplemental education opportunities.

BackgroundScarce resource allocation policies vary across the United States. Little is known about regional variation in hospital-level resource allocation protocols and variation in their application.ObjectiveTo evaluate how scarce resource allocation policies throughout the Chicagoland area vary and whether there are differences in policy application within and amongst hospitals.DesignTwo cross-sectional surveys.SettingChicagoland hospitals.ParticipantsRepresentatives from Chicagoland hospitals and triage officers at these hospitals.MeasurementsSurvey responses and categorical variables are described by frequency of occurrence. Intra- and interhospital variation in ranking of hypothetical patients was assessed using Kappa coefficients.ResultsEight Chicago area hospitals responded to the survey assessing scarce resource allocation protocols. For hospitals willing to describe their specific ventilator allocation protocol (n=7), the initial scoring system varied with most utilizing the sequential organ failure assessment (SOFA) score (86%) and medical comorbidities (57%). A majority gave priority to pre-defined groups in their initial scoring system (86%), all discussed withdrawal of mechanical ventilation for adult patients (100%), and a minority had exclusion criteria (43%). Forty-nine triage officers from nine hospitals responded to the second survey. Triage officer rankings of hypothetical patients had slight agreement amongst all hospitals (Kappa 0.158) and fair agreement within two hospitals with the most respondents (Kappa 0.21 and 0.25). Almost half of triage officer respondents reported using tiebreakers to rank patients (N=23/49, 47%).ConclusionAlthough most Chicago area hospitals surveyed created guidelines for resource allocation during the COVID-19 pandemic, these guidelines and application of these protocols by triage officers varied significantly.Funding SourceNone.

Background Scarce resource allocation policies vary across the United States. Little is known about regional variation in resource allocation protocols and variation in their application. We sought to evaluate how Covid-19 scarce resource allocation policies vary throughout the Chicago metropolitan area and whether there are differences in policy application within hospitals when prioritizing hypothetical patients who need critical care resources. Methods Two cross-sectional surveys were distributed to Chicago metropolitan area hospital representatives and triage officers. Survey responses and categorical variables are described by frequency of occurrence. Intra- and interhospital variation in ranking of hypothetical patients was assessed using Fleiss's Kappa coefficients. Results Eight Chicago-area hospitals responded to the survey assessing scarce resource allocation protocols (N = 8/18, response rate 44%). For hospitals willing to describe their ventilator allocation protocol (N = 7), most used the sequential organ failure assessment (SOFA) score (N = 6/7, 86%) and medical comorbidities (N = 4/7, 57%) for initial scoring of patients. A majority gave priority in initial scoring to pre-defined groups (N = 5/7, 71%), all discussed withdrawal of mechanical ventilation for adult patients (N = 7/7, 100%), and a minority had exclusion criteria (N = 3/7, 43%). Forty-nine triage officers from nine hospitals responded to the second survey (N = 9/10 hospitals, response rate 90%). Their rankings of hypothetical patients showed only slight agreement amongst all hospitals (Kappa 0.158) and fair agreement within two hospitals with the most respondents (Kappa 0.21 and 0.25). Almost half used tiebreakers to rank patients (N = 23/49, 47%). Conclusions Although most respondents from Chicago-area hospitals described policies for resource allocation during the COVID-19 pandemic, the substance and application of these protocols varied. There was little agreement when prioritizing hypothetical patients to receive scarce resources, even among people from the same hospital. Variations in resource allocation protocols and their application could lead to inequitable distribution of resources, further exacerbating community distrust and disparities in health. Supplemental data for this article is available online at https://doi.org/10.1080/23294515.2021.1983667.

Patients with congenital heart disease (CHD) often have other comorbidities including extracardiac and genetic anomalies that can negatively impact their outcomes after cardiac surgery. In this chapter, we provide the historical context and describe an era when children with disabilities were denied life-saving procedures. We explore modern examples of trisomies 13 and 18 to show how, although attitudes towards some genetic anomalies have shifted, conflicts about others continue to arise. We discuss the use of the best interest standard, appropriate ethical justifications for not offering surgery, and how to balance justice and societal burdens when a child has significant extracardiac anomalies that may limit his or her lifespan appreciably and/or impact quality of life. Finally, we explore the perspectives of the clinician and the parents, the importance of shared decision-making, and acknowledge the important role of the parent in cases where complexity is high, and prognostication is difficult.

Clinical ethics consultation is an emerging field whereby an individual or group of individuals with expertise in ethics provides a set of services to clinicians and families to provide guidance about ethical issues that arise during medical treatment. Ethicists may help to resolve disputes when parties disagree about treatment decisions, offer historical context regarding issues that medical providers and families may think are novel, and provide insights into the current state of the issue in the bioethics literature. Multiple methodologies and approaches exist by which an ethics consultant can scrutinize, analyze, and provide recommendations of an ethically acceptable option. In this chapter, we will focus on the role of the ethics consultant, explore various approaches to performing a formal ethics consultation, review cases from the literature, and describe two clinical ethical consultations that involve pediatric patients with congenital heart disease. Finally, we provide an update on the credentialing of ethics consultants and the potential utility of a national ethics database in the future.

Informed Consent in the setting of complex fetal congenital heart disease such as hypoplastic left heart syndrome involves many concerned individuals who include: the mother, the father, the obstetrician, the pediatric cardiologist, the pediatric cardiac surgeon, and many others. It is the duty of the physician to administer and perform informed consent under high risk pre- and postnatal circumstances that require high risk surgical options without which the newborn baby would most certainly die. We explore the intricate roll that physicians play in the informed consent process from prenatal to post-natal circumstances which include making decisions about pregnancy termination, comfort care, and staged palliation. Important considerations such as the importance of the woman’s bodily integrity, autonomy, and the ethics of comfort care are discussed with reference to other complex congenital heart lesions. We conclude that informed consent for HLHS decisions is best practiced by a multi-disciplinary organized approach that will allow comprehensive counseling by multiple care-givers in a timely fashion. Ultimately, the obstetrician/pediatric cardiologist team continue to be the primary physicians that assist with shared decision-making.

Aortopulmonary septal defect (AP window) is a rare congenital cardiac anomaly comprising only 0.1% of all congenital heart defects and over 50% are associated with additional significant congenital heart defects requiring surgical repair. A 1.9 kg, 34-week premature infant presented with

Anomalous right pulmonary artery (RPA) is a rare form of congenital heart disease associated with development of pulmonary hypertension (PH). Our patient was born at 36 weeks gestation with an anomalous RPA from a right patent ductus arteriosus (PDA), right aortic arch and left PDA. Prostaglandin

Pulmonary artery, and rarely aortic, calcifications have been reported in sporadic case reports in the recipient twin of twin-to-twin transfusion syndrome. This presentation is more likely to be secondary to the haemodynamic alterations in the recipient twin, but must be differentiated from idiopathic infantile arterial calcification as the clinical implications, treatment, and prognosis may be drastically different.

Palliative procedures are often used in patients with congenital heart disease (CHD) to improve survival when corrective surgery is deemed unfavorable. We present a 4 month old ex preemie with complex CHD (dextrocardia, transposition of the great vessels, atrial septal defect (ASD), inlet

Objective Infants undergoing heart transplantation have the highest early posttransplant mortality of any age group. We sought to determine the pretransplantation factors associated with in-hospital mortality in transplanted infants in the current era. Methods All infants under 12 months of age who underwent primary heart transplantation during a recent 10-year period (1999–2009) in the United States were identified using the Organ Procurement and Transplant Network database. Multivariable logistic regression was used to identify independent pretransplantation factors associated with in-hospital mortality. Results Of 730 infants in the study (median age 3.8 months), 462 (63%) had congenital heart disease, 282 (39%) were supported by a ventilator, 94 (13%) with extracorporeal membrane oxygenation, and 22 (3%) with a ventricular assist device at the time of transplantation. Overall, 82 (11.2%) infants died before their initial hospital discharge. In adjusted analysis, in-hospital mortality was associated with repaired congenital heart disease (odds ratio [OR], 3.6; 95% confidence interval [CI], 1.8, 7.2), unrepaired congenital heart disease not on prostaglandin E (OR, 2.8; CI, 1.3, 6.1), extracorporeal membrane oxygenator support (OR, 6.1; CI, 2.8, 13.4), ventilator support (OR, 4.4; CI, 2.3, 8.3), creatinine clearance less than 40 mL · min −1 · 1.73 m −2 (OR, 3.1; CI, 1.7, 5.3), and dialysis (OR, 6.2; CI, 2.1, 18.3) at transplantation. Conclusions One in 9 infants undergoing heart transplantation dies before hospital discharge. Pretranplantation factors associated with early mortality include congenital heart disease, extracorporeal membrane oxygenator support, mechanical ventilation, and renal failure. Risk stratification for early posttransplant mortality among infants listed for heart transplantation may improve decision-making for transplant eligibility, organ allocation, and posttransplant interventions to reduce mortality.