Delivery of care for adult patients with congenital heart disease in Europe: results from the Euro Heart Survey.

AIMS: The increasing number of adults with congenital heart disease (CHD) has prompted the development of recommendations for the management of these patients and for the organization of their healthcare. The aim of this report is to describe the delivery of care in Europe for adults with congenital cardiac anomalies. METHODS AND RESULTS: As part of the Euro Heart Survey on Adult Congenital Heart Disease, we obtained data from 71 voluntarily participating centres that detailed their care practices for these patients. Forty-eight of these centres were specialist centres and 23 were non-specialist centres. We found that only 19% of the specialist centres complied with defined standards for optimal care structure. The criteria that appeared to be most difficult for all centres to achieve were performing 50 congenital heart operations or more per year and involving nurse specialists in the care of these patients. CONCLUSION: This survey indicated that the provision of care in Europe for adults with congenital heart defects is suboptimal. To fully realize the benefits of cardiac surgery performed in infants and children, continuous effort must be applied by healthcare professionals in order to implement the recommendations on the organization of care for these patients.     

Perspectives on advance care planning and palliative care among adults with congenital heart disease

Background: Patients with adult congenital heart disease (ACHD) report that ad‐ vance care planning (ACP) is important, and that they want information about prog‐ nosis. However, recognizing importance and being willing to participate are different constructs, and how and when to begin ACP and palliative care discussions remains ill‐defined.
Methods: We conducted a cross‐sectional survey of 150 consecutive outpatients to assess willingness to participate in ACP, with whom, and important barriers and facili‐ tators to these discussions.
Results: The majority of participants (69%) reported being willing to participate in ACP; 79% to have a meeting to discuss goals and care preferences; and 91% to speak to a clinician who specializes in palliative care. Being married and anticipating a shorter lifespan were associated with increased reported willingness to participate in ACP. The health care provider with whom most participants preferred to have these discussions was their ACHD clinician. Participants identified important barriers and facilitators to these discussions.
Conclusion: Patients with ACHD report being willing to participate in ACP and pallia‐ tive care discussions. Patients prefer to have these discussions with their ACHD clini‐ cians, thus ACHD clinicians need to be prepared to address these issues as part of routine care.     

Obesity trends in children,adolescents, and young adults with congenital heart disease

Objectives: To determine the prevalence, age of onset, and risk factors for overweight and obesity in children with congenital heart disease (CHD).
Study Design: Children with CHD who were seen at our institution from 1996 to 2017 were studied. Patients were full-time residents of the United States and were receiving all cardiac care at our institution. Patients were categorized by age and CHD diagnosis. The date of last normal weight for age and the date of first recorded weight in the range of overweight and obese were documented.
Results: Nine hundred sixty-eight patients with CHD were included. The prevalence of overweight and obesity was 31.5% and 16.4%, respectively. For patients who became overweight or obese, the last recorded normal weight was between 6 and 10 years of age. Electrophysiologic disease and older age were risk factors for obesity.
Conclusions: Children with CHD have an increasing risk of becoming overweight and obese in early childhood. This study provides important information and identifies critical period to implement preventative measures and counsel families about the risk of obesity in CHD.     

Risky business: insuring adults with congenital heart disease.

Accurate prognostication in congenital heart disease is vital for purposes of obtaining insurance, yet can be problematic for patients, physicians and insurers. This article discusses the scope of the problem, and describes the process of evaluating life insurance. Mortality data as well as predictors of adverse outcomes for individual congenital heart lesions are reviewed. Practical tips for patients and their physicians are given to aid in successful application for insurance. To expand the possibility of future patients obtaining insurance coverage, the ongoing reporting and constant updating of very long-term survival data in congenital heart disease is emphasized.     

Recommendations to organize care for adults with congenital heart disease in the Czech Republic

This paper was formed by the Expert committee for congenital heart disease (CHD) in adults – a division of Czech Society of Cardiology (ČKS). It was designed as an appendix to National cardiovascular programme ČKS created in 2013 and was based on Recommendations for organization of care for adults with congenital heart disease and for training in the subspecialty of ‘Grown-up Congenital Heart Disease’ in Europe: a position paper of the Working Group on Grown-up Congenital Heart Disease of the European Society of Cardiology created in 2014 [1].Aims of this paper are: To optimize medical care in all its aspects for adults with CHD in the Czech Republic, to facilitate easy transition between paediatric and adult medical care, to enable research in the field in order to create evidence based care, to support training of regional cardiologists and other specialists who are involved in monitoring adult patients with CHD, to help with communication with national institutions, to provide information for other medical workers and patients, to consolidate resources.     

Improving the quality of transition and transfer of care in young adults with congenital heart disease

The transition and transfer from pediatric to adult care is becoming increasingly important as improvements in the diagnosis and management of congenital heart disease allow patients to live longer. Transition is a complex and continuous process that requires careful planning. Inadequate transition has adverse effects on patients, their families and healthcare delivery systems. Currently, significant gaps exist in patient care as adolescents transfer to adult care and there are little data to drive the informed management of transition and transfer of care in adolescent congenital heart disease patients. Appropriate congenital heart disease care has been shown to decrease mortality in the adult population. This paper reviews the transition and transfer of care processes and outlines current congenital heart disease specific guidelines in the United States and compares these recommendations to Canadian and European guidelines. It then reviews perceived and real barriers to successful transition and identifies predictors of success during transfer to adult congenital heart disease care. Lastly, it explores how disease‐specific markers of outcomes and quality indicators are being utilized to guide transition and transfer of care in other chronic childhood illnesses, and identifies existing knowledge gaps and structural impediments to improving the management of transition and transfer among congenital heart disease patients.     

A model for geographic and sociodemographic access to care disparities for adults with congenital heart disease

Background: Follow‐up at a regional adult congenital heart disease (ACHD) center is recommended for all ACHD patients at least once per the 2018 ACC/AHA guidelines. Other specialties have demonstrated poorer follow‐up and outcomes correlating with increased distance from health care providers, but driving time to regional ACHD centers has not been examined in the US population.
Objective: To identify and characterize potential disparities in access to ACHD care in the US based on drive time to ACHD centers and compounding sociodemographic factors.
Methods: Mid‐ to high‐volume ACHD centers with ≥500 outpatient ACHD visits and ≥20 ACHD surgeries annually were included based on self‐reported, public data. Geographic Information System mapping was used to delineate drive times to ACHD centers. Sociodemographic data from the 2012‐2016 American Community Survey (US Census) and the Environmental Systems Research Institute were analyzed based on drive time to nearest ACHD center. Previously established CHD prevalence estimates were used to estimate the similarly located US ACHD population.
Results: Nearly half of the continental US population (45.1%) lives >1 hour drive to an ACHD center. Overall, 39.7% live 1‐4 hours away, 3.4% live 4‐6 hours away, and 2.0% live >6 hours away. Hispanics were disproportionately likely to live a >6 hour drive to a center (p < .001). Compared to people with <1 hour drive, those living >6 hours away have higher proportions of uninsured adults (29% vs. 18%; p < .001), households below the federal poverty level (19% vs. 13%; p < .001), and adults with less than college education (18% vs. 12%; p < .001).
Conclusions: We estimate that ~45% of the continental US population lives >1 hour to an ACHD center, with 5.4% living >4 hours away. Compounding barriers exist for Hispanic, uninsured, lower socioeconomic status, and less‐educated patients. These results may help drive future policy changes to improve access to ACHD care.     

Intensive care of the adult patient with congenital heart disease

Prevalence of congenital heart disease in the adult population has increased out of proportion to that of the pediatric population as survival has improved, and adult congenital heart disease patients make up a growing percentage of pediatric and adult cardiac intensive care unit admissions. These patients often develop complex multiorgan system disease as a result of long-standing altered cardiac physiology, and many require reoperation during adulthood. Practitioners who care for these patients in the cardiac intensive care unit must have a strong working knowledge of the pathophysiology of complex congenital heart disease, and a full team of specialists must be available to assist in the care of these patients. This chapter will review some of the common multiorgan system effects of long-standing congenital heart disease (eg, renal and hepatic dysfunction, coagulation abnormalities, arrhythmias) as well as some of the unique cardiopulmonary physiology of this patient population.     

An alternate technique to pacing in complex congenital heart disease: assessment of the left thoracotomy approach

BACKGROUND: Pacing in children with congenital heart disease often requires alternate approaches to standard transvenous pacing. The surgical approach used to implant the pacemaker leads has been shown to impact lead survival. There is a paucity of pediatric literature describing the experience using a left thoracotomy approach. OBJECTIVES: To report on short- and mid-term experiences with pacemaker implant via the left thoracotomy approach in children with complex congenital heart disease. METHODS and RESULTS: Data were abstracted retrospectively from patients' hospital charts. To date, the left thoracotomy technique has been used in 11 patients with complex heart disease, with a median of three prior cardiac operations. The median patient age was five years (range of two months to 23 years of age). The pacing indications were acquired postoperative atrioventricular block (n=5), sinus node dysfunction (n=5) and long QT syndrome (n=1). There were no intraoperative complications or long-term complications from this approach. The pacing thresholds at implant and follow-up were acceptable in all patients. One patient died in follow-up for reasons unrelated to the pacemaker or arrhythmia. CONCLUSIONS: The placement of epicardial pacemaker leads via the left thoracotomy approach is a safe and effective alternative to transvenous pacing in pediatric patients with complex congenital heart disease.     

Transition and transfer from pediatric to adult care of young adults with complex congenital heart disease

Many children with complex congenital heart disease are now surviving childhood with the potential to live meaningful and productive adult lives. The process of transitioning or preparing patients and families for transfer from pediatric to adult care is challenging and rarely implemented properly. An inadequate transition process results in delayed and inappropriate care, improper timing of transfer, and undue emotional and financial stress on the patients, their families, and the health care system. At worst, patients are lost to appropriate follow-up. This article discusses the general principles of transition and transfer for young adults with chronic illness, highlights the needs of young adults with congenital heart disease, discusses the barriers to transition, and proposes goals and key elements of a formal transition program.     

Self-estimated physical functioning poorly predicts actual exercise capacity in adolescents and adults with congenital heart disease

Aims: The aim of this study is to compare self-reported health-relatedquality-of-life (HRQoL) with the objective of exercise performancein patients with congenital heart disease (CHD) according todiagnosis. Methods and results: 564 patients (255 females, 14–73 years) with various CHD(62 shunt, 66 left heart obstruction, 33 PS/PR, 47 Ebstein,96 Fallot, 98 TGA after atrial switch, 38 other TGA, 31 Fontan,32 palliated/native cyanotic, 61 others) and a group of 53 healthycontrols (18 females, 14–57 years) completed a QoL questionnaire(SF-36) and performed a symptom-limited cardiopulmonary exercisetest. Despite several limitations at exercise (P = 1.30 x 10^–33),patients only reported reductions in HRQoL concerning physicalfunctioning (P = 4.41 x 10^–15) and general health (P =6.17 x 10^–5) and not psychosocial aspects. This couldbe confirmed in all diagnostic subgroups. Correlation to peakoxygen uptake was found in physical functioning (r = 0.435,P = 1.72 x 10^–27) and general health (r = 0.275, P = 3.79x 10^–11). However, there was severe overestimation ofphysical functioning in most patients when compared with actualexercise test results. Conclusion: Patients with CHD rate their HRQoL impaired only in physicalfunctioning and general health and not in any psychosocial aspect.Self-estimated physical functioning poorly predicts actual exercisecapacity.     

Neurocognitive profiles in adolescents and young adults with congenital heart disease

Introduction and Objectives

The objectives of this study were to assess the neuropsychological performance (NP) of adolescents and young adults with congenital heart disease (CHD), comparing them with a group of healthy controls, to determine whether there are different neurocognitive phenotypes in CHD, and to identify their relation to sociodemographic, neonatal, clinical and psychological adjustment variables.

Depression in adults with congenital heart disease-public health challenge in a rapidly expanding new patient population

There is a growing population of adults with congenital heart disease (CHD) due to improved survival beyond childhood. It has been suggested that adults with CHD may be at increased risk for mental health problems, particularly depression. The reported incidence of depression in CHD varies from 9% to 30%. This review examines the evidence for a higher depression rate in CHD vs general population. Possible explanations are offered from a variety of disease models, ranging from brain injury to the psychoanalytical approach. Risk factors for an abnormal emotional adjustment and depression include early exposure to stress from illness and medical interventions in infancy, separation from the parents during hospitalizations and brain organic syndromes. Later in life, patients often have to cope with physical limitations. Recent improvements in care may be protective. Current patients may benefit from an earlier age at first surgical intervention, fewer reoperations and inclusion to the mainstream schooling, among other factors. At this point, there is little systematic knowledge about evidence-based therapeutic interventions for depression in adults with CHD. Health care providers of patients with CHD should be aware of mental health challenges and may take a more proactive approach to identifying patients at risk for depression.     

Psychosocial functioning of the adult with congenital heart disease: a 20-33 years follow-up.

AIMS: Since knowledge about the psychosocial function of adult patients with congenital heart disease is limited, we compared biographical characteristics, and emotional and social functioning of these patients with that of the reference groups. METHODS AND RESULTS: Patients with congenital heart disease (N=362, aged 20-46 years), belonging to five diagnostic groups, were subjected to extensive medical and psychological examination, 20-33 years after their first open heart surgery. All the patients were seen by the same psychologist, who examined their psychosocial functioning using a structured interview and questionnaires. The majority (78%)was living independently and showed favourable outcome regarding the marital status. Among married/cohabitant patients, 25-39-year-olds showed normal offspring rates. None of the 20-24-year-old patients had any children. The offspring rate dropped after the age of 40. The proportion of adult patients with a history of special education was high (27%). Accordingly, patients showed lower educational and occupational levels compared to reference groups. As regard to the emotional and social functioning (leisure-time activities), the sample showed favourable results. CONCLUSIONS: Overall, this sample of patients with congenital heart disease seemed capable of leading normal lives and seemed motivated to make good use of their abilities.     

Fetal echocardiography for planning perinatal and delivery room care of neonates with congenital heart disease

Fetal echocardiography facilitates the prenatal diagnosis of infants with congenital heart disease (CHD) and through sequential examinations, allows assessment of fetal hemodynamics and cardiovascular status from the time of diagnosis to delivery. Fetal cardiologists have created diagnostic protocols aimed at risk stratifying severity and potential postnatal compromise in fetuses with CHD, and identifying those who may require special intervention at birth or within the first days of life. In this article, we review fetal cardiovascular physiology, the progression of CHD in utero and fetal echocardiographic findings used for risk stratification of newborns with CHD, as well as some of the basic principles of planning for the neonatal resuscitation and initial transitional care of these complex newborns.     

Opportunities for training to advance the care for adults with congenital heart disease with advanced circulatory failure

Heart failure is an emerging issue with important implications in adult patients with congenital heart disease. Practitioners with expertise in both adult congenital heart disease and heart failure are needed to manage this growing and often complex population. In the United States, the optimal training pathway to enable practitioners to best care for these patients is ill‐defined. This article explores possibilities and issues that interested trainees may encounter during their training experience.