Adult Congenital Heart Disease Care Research Articles

Caring for the Critically Ill Adult Congenital Heart Disease Patient.

This review aims to discuss the unique challenges that adult congenital heart disease (ACHD) patients present in the intensive care unit. Recent studies suggest that ACHD patients make up an increasing number of ICU admissions, and that their care greatly improves in centers with specialized ACHD care. Common reasons for admission include arrhythmia, hemorrhage, heart failure, and pulmonary disease. It is critical that the modern intensivist understand not only the congenital anatomy and subsequent repairs an ACHD patient has undergone, but also how that anatomy can predispose the patient to critical illness. Additionally, intensivists should rely on a multidisciplinary team, which includes an ACHD specialist, in the care of these patients.

Health Equity and Policy Considerations for Pediatric and Adult Congenital Heart Disease Care among Minoritized Populations in the United States.

Achieving health equity in populations with congenital heart disease (CHD) requires recognizing existing disparities throughout the lifespan that negatively and disproportionately impact specific groups of individuals. These disparities occur at individual, institutional, or system levels and often result in increased morbidity and mortality for marginalized or racially minoritized populations (population subgroups (e.g., ethnic, racial, social, religious) with differential power compared to those deemed to hold the majority power in the population). Creating actionable strategies and solutions to address these health disparities in patients with CHD requires critically examining multilevel factors and health policies that continue to drive health inequities, including varying social determinants of health (SDOH), systemic inequities, and structural racism. In this comprehensive review article, we focus on health equity solutions and health policy considerations for minoritized and marginalized populations with CHD throughout their lifespan in the United States. We review unique challenges that these populations may face and strategies for mitigating disparities in lifelong CHD care. We assess ways to deliver culturally competent CHD care and to help lower-health-literacy populations navigate CHD care. Finally, we review system-level health policies that impact reimbursement and research funding, as well as institutional policies that impact leadership diversity and representation in the workforce.

Adult Congenital Heart Disease Care in Canada: Has Quality of Care Improved in the Last Decade?

BackgroundPatients with adult congenital heart disease (ACHD) are at increased risk of comorbidity and death compared with the age-matched population. Specialized care is shown to improve survival. The purpose of this study was to analyze current measures of quality of care in Canada compared with those published by our group in 2012. MethodsA survey focusing on structure and process measures of care quality in 2020 was sent to 15 ACHD centres registered with the Canadian Adult Congenital Heart Network. For each domain of quality, comparisons were made with those published in 2012. ResultsIn Canada, 36,708 patients with ACHD received specialized care between 2019 and 2020. Ninety-five cardiologists were affiliated with ACHD centres. The median number of patients per ACHD clinic was 2000 (interquartile range [IQR]: 1050, 2875). Compared with the 2012 results, this represents a 68% increase in patients with ACHD but only a 19% increase in ACHD cardiologists. Compared with 2012, all procedural volumes increased with cardiac surgeries, increasing by 12% and percutaneous intervention by 22%. Wait time for nonurgent consults and interventions all exceeded national recommendations by an average of 7 months and had increased compared with 2012 by an additional 2 months. Variability in resources were noted across provincial regions. ConclusionsOver the past 10 years, ACHD care gaps have persisted, and personnel and infrastructure have not kept pace with estimates of ACHD population growth. Strategies are needed to improve and reduce disparity in ACHD care relative to training, staffing, and access to improved care for Canadians with ACHD.

Adult congenital heart disease care in a municipal public health system.

Specialty care is associated with improved outcomes for adults with adult CHD and must be extended to the underserved. A retrospective cohort study was performed to describe the provision of care to adult CHD patients in America's largest municipal public health system including patient demographics, diagnostic and therapeutic procedures, and adherence to guideline-recommended surveillance. We identified 229 adult CHD patients aged >18 years through electronic medical records. The most common diagnoses were atrial septal defect, ventricular septal defect, patent ductus arteriosus, and valvular pulmonary stenosis. In total, 65% had moderate or greater anatomic complexity. A large number of patients were uninsured (45%), non-white (96%), and non-English speaking (44%). One hundred forty-six patients (64%) presented with unrepaired primary defects. Fifty eight patients underwent primary repair during the study period; 48 of those repairs were surgical and 10 were transcatheter. Collaboration with an affiliated Comprehensive Care Center was utilised for 28% of patients. A high proportion of patients received adult CHD speciality visits (78%), echocardiograms (66%), and electrocardiograms (56%) at the guideline-recommended frequency throughout the study period. There was no significant difference in the rate of adherence to guideline-recommended surveillance based on insurance status, race/ethnicity, or primary language status. The proportion of patients who had guideline-recommended adult CHD visits, echocardiograms, and electrocardiograms was significantly lower for those with more advanced physiological stages. These results can inform the provision of adult CHD care in other public health system settings.

CE Workshop 06: The Cumulative Burden of Congenital Heart Disease Across the Lifespan: Implications for Neuropsychologists in Pediatrics Through Geriatrics

Abstract & Learning Objectives:The heart and the brain are inextricably linked across development by overlapping genetic programs and transacting physiologies that exist long before birth and endure throughout the lifespan. Congenital heart disease (CHD) refers to a diverse array of conditions in which structural heart development is atypical. Of the roughly 1 million babies born with CHD each year, some 40,000 are born with a “critical” form of CHD that will require intensive surgical intervention within the first year of life. As recently as the 1980s, children born with some forms of critical CHD did not survive; palliation was their only option. This has changed dramatically over the past 30-40 years. Driven by momentous breakthroughs in medical science and technology, approximately 80-95% of children born with CHD today will reach adulthood.But increased survival is only a part of the CHD story. Indeed, like extreme prematurity, leukemia, and many other previously fatal medical conditions with which neuropsychologists are familiar, increases in longevity among CHD survivors have come with increasing recognition of the many challenging transitions and cumulative medical, neurobehavioral, and psychosocial burdens inherent to “living with CHD.” CHD begins to alter expected brain development in utero with evidence of structural, volumetric, and metabolic differences documented as early as the second or third prenatal trimester. Brain dysmaturation, in turn, increases one’s risk for further acquired brain injury and gives rise to a range of neurobehavioral deficits and psychosocial difficulties that consistently rank among the most salient threats to quality of life among children, adolescents, and adults with CHD.More recently, as survival into adulthood has become increasingly common for individuals with CHD, we have also begun to more fully appreciate the cascading impact and cumulative neuropsychological burden of CHD across the lifespan, which impact a range of long-term outcomes such as educational and occupational attainment, living independently, and risk for dementia.In short, CHD can no longer reasonably be considered a child or pediatric condition, but rather a lifespan condition with the potential to adversely impact neurobehavioral and psychosocial outcomes in different ways and at different times across infancy, childhood, adolescence, and adulthood. Over a series of talks presented by a panel of recognized neuropsychologists and experts in CHD, this symposium aims to review the neuropsychology of CHD across the lifespan and to present an integrative lifespan developmental neuropsychological model of CHD that eschews prevailing “child” vs. “adult” distinctions. Each presentation will address a salient developmental epoch (prenatal-early childhood, school-age/adolescence, and adulthood/geriatric timeframes) and will include a comprehensive review of the extant literature pertaining to relevant neuroanatomical and neurodevelopmental/neuropsychological considerations for individuals with CHD during each epoch. Transitions, of which there are myriad for individuals living with CHD (e.g., from acute inpatient care to stepdown unit care; from inpatient to outpatient settings; from early intervention to the school system; from childhood to adolescence; from adolescence to young adulthood; from pediatric to adult CHD care), will feature prominently throughout the symposium, as will recommendations for competent, developmentally-informed clinical neuropsychological management and intervention planning throughout the lifespan. Upon conclusion of this course, learners will be able to:1.Describe the mechanisms by which congenital heart disease (CHD) impacts brain development and functioning across the lifespan (from infancy to older adulthood).2.Discuss neurodevelopmental/neuropsychological sequelae of CHD for children, adolescents, and adults.3.Explain the role of clinical neuropsychologists in evaluating, supporting, and optimizing the neuropsychological trajectories of individuals with CHD across the lifespan.

The Impact of the COVID-19 Pandemic Restrictions on the Provision of Adult Congenital Heart Disease Care Across Canada: A National Survey

The Impact of the COVID-19 Pandemic Restrictions on the Provision of Adult Congenital Heart Disease Care Across Canada: A National Survey

Multistakeholder Recommendations for Supporting Patients and Families Transitioning From Paediatric to Adult Congenital Heart Disease Care.

Transitioning from paediatric to adult congenital heart disease (CHD) care is a high-risk time for being lost to follow-up. Existing CHD transition programmes have not included patients, caregivers, and health care providers as partners in their development. This study aimed to develop recommendations for a CHD transition programme driven by lived and clinical experiences. We used a multilevel participatory process that engaged adult and paediatric people living with CHD, their caregivers, and CHD health care providers as members of the research team. We also consulted members of these stakeholder groups through a series of 3 virtual workshops that culminated in the generation of recommendations for the essential components of a CHD transition programme. The Transition Essentials recommendations inform what information, education, or support is required, who should provide it, and when and how it should be provided. Information, education, and support for self-management and knowledge are required for people living with CHD. Caregivers require information, education, and support to build capacity in people living with CHD and navigate their new role in their loved ones' life. The health care team should provide this information, education, and support with peer support options when people living with CHD are 15-22 years of age. This information, education, and support should be individualized, navigate limitations, build over time, have multimodal options, and be available virtually or in person. Engaging those with lived and clinical expertise to develop recommendations for the essential components of a CHD transition programme provides important insights missing from previous studies.

Optimizing efficiency of a custom clinical decision support tool improves adult congenital heart disease care

Study objectiveImprove the efficiency of an inpatient clinical decision support tool (CDS) for patients with adult congenital heart disease (ACHD). DesignThe efficiency of a CDS was evaluated across two time periods and compared. SettingAn academic, tertiary care center. ParticipantsACHD patients roomed in an inpatient setting. InterventionPlan-Do-Study-Act (PDSA) methods were applied starting in 2021 and included refinement of diagnostic codes and the addition of department encounter codes. Main outcome measuresTrue positive and false positive CDS alerts. ResultsBaseline data from 2017 had a median (IQR) of 38 (17) and 2019 baseline data had 65 (19) total alerts per month. Combining both baseline data years, the median true positive CDS alerts was 47.3 %. There were 71 (6) total alerts per month for the 2021–2022 time period and with ongoing PDSA cycles and optimization in the CDS the true positive alerts improved substantially resulting in a shifting of the median to 78.9 % within 9 months. ConclusionCDS can efficiently notify providers when an ACHD patient is encountered. The use of ICD 10 codes alone to identify ACHD patients has limited accuracy with a high proportion of false positives. Ongoing revision of the CDS system methods is important to improving efficiency and minimizing provider alert fatigue.

Referral Order Placement Decreases Time to Transfer to Adult Congenital Heart Disease Care.

Pediatric patients with moderate and great complexity congenital heart disease (CHD) may benefit from coordinated transfer to adult congenital heart disease (ACHD) centers to reduce the risk of complications; however, there are a variety of transfer practices. We examined the impact of referral order placement at the last pediatric cardiology visit on time to transfer to an ACHD center. We analyzed data collected from pediatric patients with moderate and great complexity CHD who were eligible to transfer to our tertiary center's accredited ACHD center. We examined transfer outcomes and time-to-transfer between those with a referral order placed at the last pediatric cardiology visit and those without using Cox proportional hazards modeling. The sample (n = 65) was 44.6% female and mean age at study start was 19.5years (± 2.2). Referral orders were placed for 32.3% of patients at the last pediatric cardiology visit. Those who had a referral order placed at the last visit had significantly higher number of successful transfers to the ACHD center compared to those who did not (95% vs 25%, p < 0.001). In a Cox regression model, placement of a referral order at the last pediatric cardiology visit was associated significantly with a sooner time to transfer (HR 6.0; 95% CI 2.2-16.2, p > 0.001), adjusting for age, sex, complexity, living location, and pediatric cardiology visit location. Placement of a referral order at the last pediatric cardiology visit may improve transfer occurrence and time to transfer to accredited ACHD centers.

The development of adult congenital heart disease care in the United States

The development of adult congenital heart disease care in the United States

Efficacy of congenital heart disease transition clinics in improving patient education

Abstract Background Less than one third of adolescents with congenital heart disease (CHD) successfully complete a structured transition to adult care. Patient education is at the centre of the transition process, however, data on the impact of transition visits are limited. Purpose To assess the efficacy of our transition clinic in educating adolescents with CHD about their cardiac condition in preparation for adult CHD care. Methods All patients seen in our transition clinic between April 2015 and January 2022 were included. Integrated care pathways designed by the Pan London Transition Consortium guided us in the assessment and education during each of the 3 pre-planned transition visits (“ready, steady and go” model). Data collected included demographic and clinical characteristics, and qualitative assessment of the following categories: understanding, naming and describing their cardiac condition, adherence to and involvement in medication management, self-reported anxiety and awareness of the importance of dental hygiene. Results A total of 611 patients were included in the study (age 15.1±2.3 years at the first visit, 48.1% female). 51.1% of patients attended 2 and 19.3% 3 transition consultations. Most adolescents (52.5%) had CHD of moderate complexity, whereas 28.5% had simple defects and 19% severe complexity. Learning disability (LD) was present in 19% of cases, of which 35.3% were genetic syndromes (21.6% Down Syndrome, 8.6% DiGeorge Syndrome, 2.6% Noonan Syndrome, 1.9% Sotos Syndrome, 0.9% Asperger Syndrome, 0.9% Cornelia De Lange Syndrome). The overall comprehension of their condition increased significantly in patients without LD at the second (good or excellent understanding: from 20.9% to 60.2%, p&amp;lt;0.0001; naming: from 19.8% to 50.2%, p&amp;lt;0.0001; describing: from 13.9% to 40.2%, p&amp;lt;0.0001) and third visit (understanding: 66.7%, p=0.043; naming: 60.4%, p=0.004, describing: 56.3%, p=0.002; Table 1). Patients with LD did not improve their knowledge over time in understanding, naming, and describing their condition between the first and second and second and third visits (all p&amp;gt;0.05). Adherence to and the involvement in medication management, the self-reported anxiety and awareness of the importance of dental care did not change over time (Figure 1). Conclusions A structured transition program is an important tool in educating and supporting the delivery of care for CHD adolescents and their families prior their transition into the adult care. Our transition programme had a positive impact on comprehension in the majority of children. Adolescents with learning difficulties are a group who may benefit from more intensive or individually tailored education during transition to ensure that they are supported to understand their health needs and empowered to make their own choices where possible. Funding Acknowledgement Type of funding sources: None.

THE IMPACT OF THE COVID-19 PANDEMIC RESTRICTIONS ON THE PROVISION OF ACHD CARE ACROSS CANADA

Patients with adult congenital heart disease (ACHD) are at increased risk for poor outcomes when compared to the age matched non-ACHD population and require specialist care to optimize outcomes including well-being and survival. The COVID-19 pandemic significantly impacted healthcare provision across Canada with reduction on in person evaluations. The effect of the COVID-19 restrictions on ACHD care including clinic evaluation, diagnostic and procedures in Canada has not been well characterized.

Psychological Outcomes and Interventions for Individuals With Congenital Heart Disease: A Scientific Statement From the American Heart Association

Although resilience and high quality of life are demonstrated by many individuals with congenital heart disease, a range of significant psychological challenges exists across the life span for this growing patient population. Psychiatric disorders represent the most common comorbidity among people with congenital heart disease. Clinicians are becoming increasingly aware of the magnitude of this problem and its interplay with patients' physical health, and many seek guidance and resources to improve emotional, behavioral' and social outcomes. This American Heart Association scientific statement summarizes the psychological outcomes of patients with congenital heart disease across the life span and reviews age-appropriate mental health interventions, including psychotherapy and pharmacotherapy. Data from studies on psychotherapeutic, educational' and pharmacological interventions for this population are scarce but promising. Models for the integration of mental health professionals within both pediatric and adult congenital heart disease care teams exist and have shown benefit. Despite strong advocacy by patients, families' and health care professionals, however, initiatives have been slow to move forward in the clinical setting. It is the goal of this scientific statement to serve as a catalyst to spur efforts for large-scale research studies examining psychological experiences, outcomes, and interventions tailored to this population and for integrating mental health professionals within congenital heart disease interdisciplinary teams to implement a care model that offers patients the best possible quality of life.

Rationale, design and methodology of APPROACH-IS II: International study of patient-reported outcomes and frailty phenotyping in adults with congenital heart disease

BackgroundIn recent years, patient-reported outcomes (PROs) have received increasing prominence in cardiovascular research and clinical care. An understanding of the variability and global experience of PROs in adults with congenital heart disease (CHD), however, is still lacking. Moreover, information on epidemiological characteristics and the frailty phenotype of older adults with CHD is minimal. The APPROACH-IS II study was established to address these knowledge gaps. This paper presents the design and methodology of APPROACH-IS II. Methods/designAPPROACH-IS II is a cross-sectional global multicentric study that includes Part 1 (assessing PROs) and Part 2 (investigating the frailty phenotype of older adults). With 53 participating centers, located in 32 countries across six continents, the aim is to enroll 8000 patients with CHD. In Part 1, self-report surveys are used to collect data on PROs (e.g., quality of life, perceived health, depressive symptoms, autonomy support), and explanatory variables (e.g., social support, stigma, illness identity, empowerment). In Part 2, the cognitive functioning and frailty phenotype of older adults are measured using validated assessments. DiscussionAPPROACH-IS II will generate a rich dataset representing the international experience of individuals in adult CHD care. The results of this project will provide a global view of PROs and the frailty phenotype of adults with CHD and will thereby address important knowledge gaps. Undoubtedly, the project will contribute to the overarching aim of improving optimal living and care provision for adults with CHD.

Abstract 12850: The Effect of Regionalization on End-of-Life Hospitalizations for Adult Congenital Cardiology Patients

Introduction: Adult congenital heart disease (ACHD) patients may have shortened life expectancy due to complications of interventions and accrued comorbidities. Specialized ACHD care has become standard of care. The effect of regionalization on ACHD patient death is not known. Methods: The Office of Statewide Health Planning and Development database identified ACHD patients ≥ 18 years who died in California hospitals from 1995 - 2018. Specialized ACHD centers were self-designated, so high and low ACHD surgical volume centers were also compared. Results: Of 3,724 patients, when comparing by volume, more deaths occurred at low (85.2%) than high volume centers (14.8%); when comparing by specialization, more deaths occurred at non-ACHD (78.8%) than ACHD centers (21.2%). Moderate (9.4%) and high complexity (4.4%) lesions were rare. Common causes of death were sepsis (14.6%), myocardial infarction (9.3%), stroke (7.5%), and heart failure (7.1%). High volume centers were more likely the location of death than low volume centers for patients aged 18-64, with private insurance, and who lived &gt;20 miles away (p &lt;0.0001 for all). These held true for ACHD vs non-ACHD centers (p &lt;0.0001 for all). Low volume centers were more likely the location of death than high volume centers for patients from zip codes with relative poverty (p 0.0243). This held true for non-ACHD vs ACHD centers (p 0.0182). Over 24 years, the proportion of patients dying at ACHD centers increased (Figure 1), while the proportion of patients dying at high volume centers decreased. Conclusion: ACHD patients who die at high surgical volume and ACHD self-designated centers are more likely to be young, have private insurance, be referred to centers far from home, and have higher socioeconomic status. These likely reflect disparities in access to specialized ACHD care. Despite regionalization with an increase in specialized ACHD centers over time, the majority of ACHD patients continue to die at non-specialized centers.

Distance to Care, Rural Dwelling Status, and Patterns of Care Utilization in Adult Congenital Heart Disease.

Many patients with adult congenital heart disease (ACHD) do not receive guideline-directed care. While distance to an ACHD center has been identified as a potential barrier to care, the impact of distance on care location is not well understood. The Oregon All Payer All Claims database was queried to identify subjects 18-65years who had a health encounter from 2010 to 2015 with an International Classification of Diseases-9 code consistent with ACHD. Residence area was classified using metropolitan statistical areas and driving distance was queried from Google Maps. Utilization rates and percentages were calculated and odds ratios were estimated using negative binomial and logistic regression. Of 10,199 identified individuals, 52.4% lived < 1h from the ACHD center, 37.5% 1-4h, and 10.1% > 4h. Increased distance from the ACHD center was associated with a lower rate of ACHD-specific follow-up [< 1h: 13.0% vs. > 4h: 5.0%, adjusted OR 0.32 (0.22, 0.48)], but with more inpatient, emergency room, and outpatient visits overall. Those who more lived more than 4h from the ACHD center had less inpatient visits at urban hospitals (55.5% vs. 93.9% in those < 1h) and the ACHD center (6.2% vs. 18.2%) and more inpatient admissions at rural or critical access hospitals (25.5% vs. 1.9%). Distance from the ACHD center was associated with a decreased probability of ACHD follow-up but higher health service use overall. Further work is needed to identify strategies to improve access to specialized ACHD care for all individuals with ACHD.

Barriers to care in urban and rural dwelling adults with congenital heart disease

Adults with congenital heart disease (CHD) face a unique set of medical, psychological, and social challenges, and access to specialised adult congenital heart disease care has been associated with improved outcomes. Rural adults with CHD may represent a uniquely disadvantaged group given additional challenges when accessing specialised care. The aim of this study was to investigate the challenges faced by adults with CHD in accessing outpatient cardiac care, with a specific focus on understanding differences between urban- and rural-dwelling patients. This cross-sectional, survey-based study took place in the adult congenital heart disease clinic at an urban academic medical center. Additional medical information was abstracted in a retrospective manner from the electronic health record. In addition to descriptive statistics, t-tests and Chi-square tests were performed to investigate differences between urban and rural dwelling patients. A total of 100 patients participated in the study (mean age 40±13 years, 60% female, 18% rural dwelling). Across the total sample, the median driving distance to clinic was 20 miles (interquartile range 12-77); it was 15 miles for urban dwellers and 77 miles for rural dwelling patients (p < 0.001). The most commonly identified barriers to cardiac clinic visits were financial losses related to taking time off from work (39%), distance of clinic from home (33%), and weather (33%). Compared to urban dwelling patients, on average those who were rural dwelling had a lower level of education (p = 0.04), more difficulty paying insurance premiums (p < 0.001) and copays (p = 0.005), and were more likely to identify the distance from clinic (p = 0.05) and having to go into the city (p = 0.02) as barriers to clinic appointments. The financial impact and distance to clinic were the most commonly identified barriers to outpatient cardiac care in this cohort of adults with CHD. These barriers, along with difficulty paying insurance premiums, are more common in rural dwelling patients. Initiatives such as telemedicine visits or providing financial subsidies for travel and treatment could help to expand specialty adult congenital heart disease care and better serve this growing patient population.

Population and Resource Utilization Among Patients With Adult Congenital Heart Disease: A Snapshot View of a Moderate-Size Canadian Regional Centre

BackgroundHealth care resource utilization for patients with adult congenital heart disease (ACHD) has not been well characterized outside of large Canadian specialized regional centres. We sought to describe the ACHD population and resource utilization patterns seen in a medium regional Canadian centre providing specialized ACHD care. MethodsA cross-sectional retrospective study was done from a sample of patients seen in 2018 at the ACHD clinic in Manitoba, Canada. Demographic data were collected along with cardiac anatomy and repair type. Health care resource utilization, clinic visits, hospital admissions, unexpected hospital presentations, and cardiac interventions were measured over a 5-year period. ResultsA random sample of 262 patients was selected from our specialized ACHD clinic. Mean age was 33.5 (±13.7) years; 48% of the population was female, and >50% resided within the major city limits. A total of 21% of the population had simple anatomy, 44% had moderate anatomy, and 35% had complex anatomy. The most commonly used imaging modality was echocardiography, followed by cardiac magnetic resonance imaging, with more frequent imaging done in patients with complex anatomy. Unexpected hospital encounters occurred at a rate of 16 per 100 person-years. Total inpatient hospital days occurred at a rate of 33 per 100 person-years, and visits to the congenital clinic occurred at a rate of 90 per 100 person-years. ConclusionsHealth care resource utilization appears to be highest in older adults and those with more complex ACHD anatomy. As the overall cohort of adults with ACHD continues to age, resource needs are likely to increase.

It's part of who I am: The impact of congenital heart disease on adult identity and life experience

As clinical care and research in adult congenital heart disease (ACHD) increasingly include a focus on quality of life, hearing and reflecting on our patients’ experiences must be a priority. The objective of this study was to describe how living with this chronic illness has affected the adult lives of patients living with ACHD. We conducted a qualitative study of 25 participants in which we asked about the impact of ACHD on their quality of life. We used thematic analysis to analyze the interview data and identified themes reflecting patients’ reported experiences. Study participants were a mean age of 38 years (range 21–63 years) and reported good functional status. ACHD was characterized as simple (24%), moderate (32%), or complex (44%). Three major themes arose: 1) impact of ACHD on identity, including perception of “normal,” self-esteem, and motivation; 2) sources of emotional distress; and 3) influence on adult decision-making, including careers and relationships. Living with ACHD impacts important aspects related to self-perceived quality of life. Addressing these concerns is important to providing comprehensive, patient-centered ACHD care.

Changes in care patterns associated with a transition program in adolescents with congenital heart disease: A single center study

BackgroundTransition programs educate patients on the importance of continued congenital heart disease specialized care. ObjectiveThe aim of this study was to evaluate changes in the rate of unplanned cardiac hospitalizations after the implementation of a formal transition program in adolescent and young adult congenital heart disease patients. MethodsIn 2016, a formal transition program began at our institution, whereby all patients ≥ 16 years/old with congenital heart disease seen in the pediatric cardiology clinic met with an adult congenital heart disease nurse to discuss transition-related topics. A chart review was performed to determine the difference in unplanned cardiac hospitalizations in control patients seen in the pediatric cardiology clinic before initiation of the transition program (years 2013–2015) compared with transition patients seen after at least 1 transition visit (years 2016–2018). ResultsA total of 303 control CHD patients (control group) were compared with 350 transition CHD patients (transition group). The rate of unplanned cardiac hospitalizations was significantly lower for the transition group after a transition visit compared to control patients (0.004 hosp/pt/yr vs. 0.019 hosp/pt/yr, p = 0.008). Being in the transition group was independently associated with a lower rate of unplanned cardiac hospitalizations. Those in the transition group had a higher rate of transfer to an adult congenital cardiologist (47% vs 38%, p = 0.034). ConclusionBeing in the transition group resulted in a lower rate of unplanned cardiac hospitalizations and an increased frequency of transfer to an adult congenital cardiology center. This study reinforces the importance of structured transition programs to educate and ensure care continuity in patients transferring to adult congenital heart disease care.