Perspectives of Challenges in Counseling for Congenital Heart Defects.

Prior work regarding counseling patients about congenital heart defects (CHD) has focused on their perceptions about accurate communication of cardiac anatomy, and the emotional support received from the provider. The objectives of this study were to identify the additional CHD counseling-specific challenges and areas for future intervention, using a practical communication framework. This is a secondary analysis of qualitative data provided by caretakers of infants who received congenital heart surgery from 2019 to 2020 in the Chicagoland area. While the survey in the primary study pertained to barriers in obtaining prenatal diagnosis, respondents with both prenatal and postnatal diagnosis reported challenges to effective counseling. Qualitative data measuring counseling challenges were collected from semi-structured phone interviews. Thematic analysis was performed using an inductive approach. Themes were organized into five domains using SPIKES (Setting, Perception, Invitation, Knowledge, Empathy, and Summarize/Strategy), a previously validated framework to help clinicians effectively break bad news. Among 160 survey respondents, 35 (21.9%) reported a challenge during CHD counseling that they received. In total, 12 challenges were identified and spanned all six SPIKES domains. The three most common challenges were as follows: perception of repeated imaging studies for accurate diagnosis or management (n = 19, Knowledge), the lack of cardiologist presence at the time of initial CHD detection (n = 8, Setting), and insufficient information provided about the CHD diagnosis (n = 7, Knowledge). Patients perceive counseling as a key component of prenatal diagnosis of CHD and identify the challenges that exist at all stages of the counseling process. These findings suggest that effective counseling extends beyond conveying information about anatomy and prognosis.

Ethical Considerations in Fetal Cardiology.

Fetal cardiology has evolved over the last 40 years and changed the timing of diagnosis and counseling of congenital heart disease, decision-making, planning for treatment at birth, and predicting future surgery from the postnatal to the prenatal period. Ethical issues in fetal cardiology transect multiple aspects of biomedical ethics including improvement in prenatal detection and diagnostic capabilities, access to equitable comprehensive care that preserves a pregnant person's right to make decisions, access to all reproductive options, informed consent, complexity in shared decision-making, and appropriate use of fetal cardiac interventions. This paper first reviews the literature and then provides an ethical analysis of accurate and timely diagnosis, equitable delivery of care, prenatal counseling and shared decision-making, and innovation through in utero intervention.

Incidence of Fetal Arrhythmia Before and During the COVID-19 Pandemic: A Single-Center Experience.

Fetal arrhythmias are rare and carry significant morbidity and mortality without appropriate treatment. Initial reports exist of fetal arrhythmia in the setting of maternal COVID-19 infection. Our study sought to evaluate incidence of fetal arrhythmia before and during the COVID-19 pandemic at our institution. This retrospective cohort study from a tertiary care fetal cardiac center utilized the institutional REDCap database to search fetal arrhythmia diagnostic codes. Medical records of mother-fetus dyads were reviewed and data were collected on diagnoses, gestational age, treatment regimen, and postnatal outcomes. Patients were divided into pre-COVID and peri-COVID segments. 8368 total pregnancies were evaluated during the 7.3 years of study period. Forty-five patients (0.5%) had a significant fetal arrhythmia and were included in this study: 19 (42%) in the pre-COVID-19 group and 26 (58%) in the peri-COVID-19 group. No patients had associated congenital heart disease. There was a notable increase in the incidence of fetal supraventricular tachycardia (SVT) (1.82 per 1000 vs 2.65 per 1000 pregnancies) and complete heart block (1.04 per 1000 vs 1.77 per 1000 pregnancies) but no apparent change in other tachyarrhythmias during the COVID era. The proportion of antibody-mediated complete heart block increased from 50 to 87.5%. There was also an increase in the percentage of SVT patients requiring postnatal treatment during COVID-19 (53.8% vs 62.5%). Our experience shows an increased incidence of some fetal arrhythmia diagnoses during the COVID-19 pandemic. Additional multi-center studies will be necessary to fully evaluate the increased burden of fetal arrhythmias during the COVID-19 era as well as to elucidate etiology.

Tricuspid Atresia with Absent Pulmonary Valve with Nearly Discontinuous Branch Pulmonary Arteries.

Absent pulmonary valve with tricuspid atresia or tricuspid stenosis (APV-TA/TS) is an extremely rare congenital heart defect associated with significant morbidity and mortality. Compared to Tetralogy of Fallot with Absent Pulmonary Valve Syndrome, branch pulmonary arteries are not typically significantly dilated. We present the case of a newborn male prenatally diagnosed APV-TA with intact ventricular septum (IVS) and nearly discontinuous branch pulmonary arteries, the surgical strategy employed, and the salient hemodynamic factors considered in the medical decision-making.

Burden with No Benefit: Prior Authorization in Congenital Cardiology.

Prior authorization is a process that health insurance companies use to determine if a patient's health insurance will cover certain medical treatments, procedures, or medications. Prior authorization requests are common in adult congenital and pediatric cardiology (ACPC) due to need for advanced diagnostics, complex procedures, disease-specific medications, and the heterogeneity of the ACPC population. Prior authorizations in ACPC are rarely denied, but nonetheless, they are often accompanied by significant administrative burden on clinical care teams and delays in patient care. Prior authorizations have been implicated in worsening care inequities. The prior authorization process is insurer specific with differences between commercial and public insurers. Prior authorization rejections were previously found to be more common for women, racial minorities, those with low education, and in low-income groups. Prior authorization unduly burdens routine diagnostics, routine interventional and surgical procedures, and routine cardiac specific medication use in the ACPC population. This manuscript highlights the burdens of prior authorization and advocates for the elimination of prior authorization for ACPC patients.

Patient-reported barriers to prenatal diagnosis of congenital heart defects: A mixed-methods study.

OBJECTIVE: To ascertain patient-reported, modifiable barriers to prenatal diagnosis of congenital heart defects (CHDs). METHODS: This was a mixed-methods study among caretakers of infants who received congenital heart surgery from 2019 to 2020 in the Chicagoland area. Quantitative variables measuring sociodemographic characteristics and prenatal care utilization, and qualitative data pertaining to patient-reported barriers to prenatal diagnosis were collected from electronic health records and semi-structured phone surveys. Thematic analysis was performed using a convergent parallel approach. RESULTS: In total, 160 caretakers completed the survey, 438 were eligible for survey, and 49 (31%) received prenatal care during the COVID-19 pandemic. When comparing respondents and non-respondents, there was a lower prevalence of maternal Hispanic ethnicity and a higher prevalence of non-English/Spanish-speaking households. Of all respondents, 34% reported an undetected CHD on ultrasound or echocardiogram, while 79% reported at least one barrier to prenatal diagnosis related to social determinants of health. Among those social barriers, the most common were difficulty with appointment scheduling (n = 12, 9.5%), far distance to care/lack of access to transportation (n = 12, 9.5%) and difficulty getting time off work to attend appointments (n = 6, 4.8%). The latter two barriers were correlated. CONCLUSION: While technical improvements in the detection of CHDs remain an important area of research, it is equally critical to produce evidence for interventions that mitigate barriers to prenatal diagnosis due to social determinants of health.

Addressing Social Determinants of Health and Mitigating Health Disparities Across the Lifespan in Congenital Heart Disease: A Scientific Statement From the American Heart Association.

Despite the overall improvement in life expectancy of patients living with congenital heart disease (congenital HD), disparities in morbidity and mortality remain throughout the lifespan. Longstanding systemic inequities, disparities in the social determinants of health, and the inability to obtain quality lifelong care contribute to poorer outcomes. To work toward health equity in populations with congenital HD, we must recognize the existence and strategize the elimination of inequities in overall congenital HD morbidity and mortality, disparate health care access, and overall quality of health services in the context of varying social determinants of health, systemic inequities, and structural racism. This requires critically examining multilevel contributions that continue to facilitate health inequities in the natural history and consequences of congenital HD. In this scientific statement, we focus on population, systemic, institutional, and individual-level contributions to health inequities from prenatal to adult congenital HD care. We review opportunities and strategies for improvement in lifelong congenital HD care based on current public health and scientific evidence, surgical data, experiences from other patient populations, and recognition of implicit bias and microaggressions. Furthermore, we review directions and goals for both quantitative and qualitative research approaches to understanding and mitigating health inequities in congenital HD care. Finally, we assess ways to improve the diversity of the congenital HD workforce as well as ethical guidance on addressing social determinants of health in the context of clinical care and research.

Contemporary Provider Management Practices and Attitudes Toward Referral for Advanced Heart Failure Therapies in Fontan Patients Across North America.

BACKGROUND: To date, no reports have described clinicians' management practices for patients with Fontan circulatory failure or their understanding of risk factors for mortality and transplant outcomes in these patients. METHODS AND RESULTS: A cross-sectional survey of caregivers across North America was conducted from February to September 2020. Responses were compared by primary specialty (heart failure/transplant vs non-heart failure/transplant), years of experience (early, mid, and late career), and Fontan center volume (low, medium, and high). Of 400 responses, the majority were from general cardiologists (111, 28%) followed by heart failure/transplant specialists (93, 23%). Although most agreed that patients with Fontan physiology will have signs/symptoms of heart failure (369 [93%]) and eventuate in heart transplant (286 [72%]), many disagreed (180 [45%]) that routine evaluation by a transplant cardiologist is needed without symptoms. Transplant providers were more likely than non-transplant providers to suggest referral for manifestations of Fontan circulatory failure such as protein-losing enteropathy, plastic bronchitis, liver fibrosis/cirrhosis, and worsening valve regurgitation. Non-transplant providers were more likely to suggest that protein-losing enteropathy, plastic bronchitis, and Fontan-associated liver disease lead to inferior outcomes after transplantation. Early career and transplant providers more favorably viewed ventricular assist device use for Fontan patients failing traditional heart failure therapy (P < .05 for all). CONCLUSIONS: There is significant variation in the management of Fontan patients, including heterogeneous timing of referral of such patients to the heart failure/transplant team, which may have implications for future outcomes.

Parents' decision-making for their foetus or neonate with a severe congenital heart defect.

BACKGROUND: Parents who receive a diagnosis of a severe, life-threatening CHD for their foetus or neonate face a complex and stressful decision between termination, palliative care, or surgery. Understanding how parents make this initial treatment decision is critical for developing interventions to improve counselling for these families. METHODS: We conducted focus groups in four academic medical centres across the United States of America with a purposive sample of parents who chose termination, palliative care, or surgery for their foetus or neonate diagnosed with severe CHD. RESULTS: Ten focus groups were conducted with 56 parents (Mage = 34 years; 80% female; 89% White). Results were constructed around three domains: decision-making approaches; values and beliefs; and decision-making challenges. Parents discussed varying approaches to making the decision, ranging from relying on their "gut feeling" to desiring statistics and probabilities. Religious and spiritual beliefs often guided the decision to not terminate the pregnancy. Quality of life was an important consideration, including how each option would impact the child (e.g., pain or discomfort, cognitive and physical abilities) and their family (e.g., care for other children, marriage, and career). Parents reported inconsistent communication of options by clinicians and challenges related to time constraints for making a decision and difficulty in processing information when distressed. CONCLUSION: This study offers important insights that can be used to design interventions to improve decision support and family-centred care in clinical practice.

Provider insights on shared decision-making with families affected by CHD.

BACKGROUND AND OBJECTIVES: Little data exist on provider perspectives about counselling and shared decision-making for complex CHD, ways to support and improve the process, and barriers to effective communication. The goal of this qualitative study was to determine providers' perspectives regarding factors that are integral to shared decision-making with parents faced with complex CHD in their fetus or newborn; and barriers and facilitators to engaging in effective shared decision-making. METHODS: We conducted semi-structured interviews with providers from different areas of practice who care for fetuses and/or children with CHD. Providers were recruited from four geographically diverse centres. Interviews were recorded, transcribed, and analysed for key themes using an open coding process with a grounded theory approach. RESULTS: Interviews were conducted with 31 providers; paediatric cardiologists (n = 7) were the largest group represented, followed by nurses (n = 6) and palliative care providers (n = 5). Key barriers to communication with parents that providers identified included variability among providers themselves, factors that influenced parental comprehension or understanding, discrepant expectations, circumstantial barriers, and trust/relationship with providers. When discussing informational needs of parents, providers focused on comprehensive short- and long-term outcomes, quality of life, and breadth and depth that aligned with parental goals and needs. In discussing resources to support shared decision-making, providers emphasised the need for comprehensive, up-to-date information that was accessible to parents of varying situations and backgrounds. CONCLUSIONS: Provider perspectives on decision-making with families with CHD highlighted key communication issues, informational priorities, and components of decision support that can enhance shared decision-making.

Applicability and Durability of Valve-Sparing Tetralogy of Fallot Repair.

BACKGROUND: Although valve-sparing repair remains ideal for patients with tetralogy of Fallot, the durability of valve-sparing repair and which patients may have been better served with a transannular patch remain unclear. METHODS: Retrospective review was performed of tetralogy of Fallot operations at our institution from January 2008 to December 2018. Standard demographic data were collected, including echocardiographic parameters, operative details, and clinical outcomes. Statistical analysis was performed comparing the transannular patch and valve-sparing repair groups. RESULTS: Sixty-seven patients underwent tetralogy of Fallot repair with a median age of 4.5 (3.2-6.0) months and weight of 5.8 (5.2, 6.7) kg. Seventeen (25%) patients underwent transannular patch repair and 50 (75%) patients underwent valve-sparing repair. There was no difference in age or weight between patients who underwent a transannular patch repair and those who underwent a valve-sparing repair. At last follow-up (median 42 months), there was a trend of a higher peak pulmonary valve/right ventricular outflow tract gradient (P = .06) in the valve-sparing group, but no difference in the pulmonary valve annulus z-scores. Additionally, the pulmonary valve z-scores in the valve-sparing group decreased from -2.3 ± 1.0 on predischarge echocardiogram of to -1.2 ± 1.6 on last follow-up, with the peak gradient on predischarge 23 (0-37) mm Hg remaining stable on last follow-up at 18 (0-29) mm Hg. There was one reoperation: pulmonary valve replacement six years after a transannular patch. CONCLUSIONS: Obtaining a postrepair pulmonary valve z-score of -2 yields satisfactory, stable valve-sparing repair with pulmonary valve growth, acceptable gradients, minimal regurgitation, and high freedom from reintervention during follow-up.

Health Care Policy and Congenital Heart Disease: 2020 Focus on Our 2030 Future.

The congenital heart care community faces a myriad of public health issues that act as barriers toward optimum patient outcomes. In this article, we attempt to define advocacy and policy initiatives meant to spotlight and potentially address these challenges. Issues are organized into the following 3 key facets of our community: patient population, health care delivery, and workforce. We discuss the social determinants of health and health care disparities that affect patients in the community that require the attention of policy makers. Furthermore, we highlight the many needs of the growing adults with congenital heart disease and those with comorbidities, highlighting concerns regarding the inequities in access to cardiac care and the need for multidisciplinary care. We also recognize the problems of transparency in outcomes reporting and the promising application of telehealth. Finally, we highlight the training of providers, measures of productivity, diversity in the workforce, and the importance of patient-family centered organizations in advocating for patients. Although all of these issues remain relevant to many subspecialties in medicine, this article attempts to illustrate the unique needs of this population and highlight ways in which to work together to address important opportunities for change in the cardiac care community and beyond. This article provides a framework for policy and advocacy efforts for the next decade.

Exploration and Ethical Analysis of Open-label Pediatric Vaccine Trials in a Pandemic.

Young children will ultimately need to be vaccinated to stop the spread of coronavirus disease 2019 (COVID-19). Initial studies of vaccine were performed in adults. Randomized controlled trials are the gold standard. In the COVID-19 pandemic, many questions need to be answered about the ethics and feasibility of these trials. Given the harms of the COVID-19 pandemic and the now-known efficacy of the vaccines in adults and teens, the question of whether clinical equipoise exists for a placebo-controlled trial of vaccines in younger children remains. Parents may be reluctant to enroll children in these trials because they want their child to receive the vaccine or because they are worried about vaccines or clinical trials in general. One option for gathering data on tolerability and efficacy in children would be to use a nonrandomized trial to enroll parents willing to vaccinate their children and those who are hesitant. We discuss the advantages and disadvantages of such an open-label trial that could provide guidance for future pandemics. (Clin Ther.

Evolution and Current Results of a Unified Strategy for Sinus Venosus Surgery.

BACKGROUND: Given recent reports of percutaneous closure of sinus venosus atrial septal defects, we reviewed our experience with surgical repair. Owing to the high incidence of arrhythmias with the two-patch technique, since 2001 we have used either one-patch repairs or the Warden procedure. METHODS: A retrospective review was performed of pediatric patients undergoing sinus venosus atrial septal defect repair at our institution from January 1, 1990, to July 1, 2018. Standard demographic data such as echocardiographic and cross-sectional imaging along with operative details and clinical echocardiographic outcomes were collected. RESULTS: The cohort included 144 patients with a median age of 4.3 years (interquartile range, 8.5). Inferior SVASD was present in 24 patients (17%). A single autologous untreated pericardial patch was used for 114 patients (79%), a two-patch technique for 20 patients (14%, last performed in 2000), and a Warden procedure in 10 patients (7%). Median length of stay was 4 days (interquartile range, 2). On echocardiogram follow-up, no patient had pulmonary vein stenosis. One patient who had the Warden procedure required a balloon dilation of the superior caval vein 2 years postoperatively and a stent 3 years later. Two-patch patients were substantially less likely to be in normal sinus rhythm (41%) on postoperative electrocardiograms compared with the other two techniques (81% one-patch and 89% Warden, P = .02). CONCLUSIONS: The great majority of patients with sinus venosus atrial septal defects can be successfully repaired with a single patch of autologous pericardium. We transitioned to using either a single pericardial patch or the Warden procedure, resulting in a higher frequency of normal sinus rhythm on postoperative electrocardiograms.

Variability in clinical decision-making for ventricular assist device implantation in pediatrics.

BACKGROUND: Minimal data exist on clinical decision-making in VAD implantation in pediatrics. This study aims to identify areas of consensus/variability among pediatric VAD physicians in determining eligibility and factors that guide decision-making. METHODS: An 88-item survey with clinical vignettes was sent to 132 pediatric HT cardiologists and surgeons at 37 centers. Summary statistics are presented for the variables assessed. RESULTS: Total respondents were 65 (72% cardiologists, 28% surgeons) whose centers implanted 1-5 (34%), 6-10 (40%), or >10 (26%) VADs in the past year. Consensus varied by patients' age, diagnosis, and Pedimacs profile. Highest agreement to offer VAD (97%) was a mechanically ventilated teenager with dilated cardiomyopathy. Patients stable on inotropes were less likely offered VAD (11%-25%). SV infant with Pedimacs profile 2 had the most varied responses: 37% offered VAD; estimated survival ranged from 15% to 90%. Variables considered for VAD eligibility included mild developmental delays (100% offered VAD), moderate-severe behavioral concerns (46%), cancer in remission >2 years (100%), active malignancy with good prognosis (68%) or uncertain prognosis (36%), and BMI >35 (74%) or <15 (69%). Most respondents (91%) would consider destination therapy VADs in pediatrics, though not currently feasible at 1/3 of centers. Factors with greatest influence on decision-making included HT candidacy, families' goals of care, and risks of complications. CONCLUSIONS: Significant variation exists among pediatric VAD physicians when determining VAD eligibility and estimating survival, which can lead to differences in access to emerging technologies across institutions. Further work is needed to understand and mitigate these differences.

Pediatric Cardiology Specialist's Opinions Toward the Acceptability of Comfort Care for Congenital Heart Disease.

In order to evaluate physicians' willingness to seek legal action to mandate surgery when parents refuse surgery for various congenital heart lesions, we surveyed pediatric cardiologists and cardiovascular surgeons at 4 children's hospitals. We asked whether physicians would support parental refusal of surgery for specific heart defects and, if not, whether they would seek legal action to mandate surgery. We then analyzed associations between physicians' willingness to mandate surgery and national operative mortality rates for each lesion. We surveyed 126 cardiologists and 9 cardiac surgeons at four tertiary referral centers. Overall response rate was 77%. Greater than 70% of physicians would seek legal action and mandate surgery for the following lesions: ventricular septal defect, coarctation of the aorta, complete atrioventricular canal, transposition of the great arteries, tetralogy of Fallot, and unobstructed total anomalous pulmonary venous return. Surgery for all of these lesions has reported mortality rates of < 5%. Physicians were less likely to seek legal action when parents refused surgery for Shone complex, any single ventricle lesion, or any congenital heart disease accompanied by Trisomy 13 or Trisomy 18. Among experts in pediatric cardiology, there is widespread agreement about the appropriate response to parental refusal of surgery for most congenital heart lesions, and these lesions tended to be heart defects with lower surgical mortality rates. Lesions for which there was greater consensus among experts were those with the best outcomes. There was less consensus for lesions with higher mortality rates. Such surveys, revealing disagreement among expert professionals, can provide an operational definition of the current professional "gray zone" in which parental preferences should determine treatment.

Should Decision Making Be Shared in High-Risk Pediatric Heart Donation?

This article considers complexities of shared decision making in pediatric heart transplantation and suggests that decisions about pediatric heart transplantation should be shared between a clinical team and parents. This article also considers goals of shared decision making involving Public Health Service increased-risk donors and recommends policy changes to strengthen decision sharing.