Transition Preparation for Young Adolescents with Congenital Heart Disease: A Clinical Trial.

OBJECTIVE: To evaluate the impact of a novel nurse-led transition intervention program designed for young adolescents (age 13-14 years) with congenital heart disease (CHD). We hypothesized that the intervention would result in improved self-management skills and CHD knowledge. STUDY DESIGN: Single-center cluster randomized controlled trial of a nurse-led transition intervention vs usual care. The intervention group received a 1-hour individualized session with a cardiology nurse, focusing on CHD education and self-management. The primary end point was change in TRANSITION-Q (transition readiness) score between baseline and 6 months. The secondary end point was change in MyHeart score (CHD knowledge). RESULTS: We randomized 60 participants to intervention (n = 30) or usual care (n = 30). TRANSITION-Q score (range 0-100) increased from 49 ± 10 at baseline to 54 ± 9.0 at 6 months (intervention) vs 47 ± 14 to 44 ± 14 (usual care). Adjusted for baseline score, TRANSITION-Q scores at 1 and 6 months were greater in the intervention group (mean difference 5.9, 95% CI 1.3-10.5, P = .01). MyHeart score (range 0-100) increased from 48 ± 24 at baseline to 71 ± 16 at 6 months (intervention) vs 54 ± 24 to 57 ± 22 (usual care). Adjusted for baseline score, MyHeart scores at 1 and 6 months were greater in the intervention group (mean difference 19, 95% CI 12-26, P < .0001). Participants aged 14 years had a greater increase in TRANSITION-Q score at 6 months compared with 13-year-old participants (P < .05). CONCLUSIONS: A nurse-led program improved transition readiness and CHD knowledge among young adolescents. This simple intervention can be readily adopted in other healthcare settings. TRIAL REGISTRATION: ClinicalTrials.gov: NCT02374892.

Gothenburg Empowerment Scale (GES): psychometric properties and measurement invariance in adults with congenital heart disease from Belgium, Norway and South Korea.

BACKGROUND: Patient empowerment is associated with improvements in different patient-reported and clinical outcomes. However, despite being widely researched, high quality and theoretically substantiated disease-generic measures of patient empowerment are lacking. The few good instruments that are available have not reported important psychometric properties, including measurement invariance. The aim of this study was to assess the psychometric properties of the 15-item Gothenburg Empowerment Scale (GES), with a particular focus on measurement invariance of the GES across individuals from three countries. METHODS: Adults with congenital heart disease from Belgium, Norway and South Korea completed the GES and other patient-reported outcomes as part of an international, cross-sectional, descriptive study called APPROACH-IS II. The scale's content (missing data) and factorial validity (confirmatory factor analyses), measurement invariance (multi-group confirmatory factor analyses), responsiveness (floor and ceiling effects) and reliability (internal consistency) were assessed. RESULTS: Content validity, responsiveness and reliability were confirmed. Nonetheless, metric but not scalar measurement invariance was supported when including the three countries, possibly because the scale performed differently in the sample from South Korea. A second set of analyses supported partial scalar invariance for a sample that was limited to Norway and Belgium. CONCLUSION: Our study offers preliminary evidence that GES is a valid and reliable measure of patient empowerment in adults with congenital heart disease. However, cross-country comparisons must be made with caution, given the scale did not perform equivalently across the three countries.

Coronavirus disease 2019 in adults with congenital heart disease: a position paper from the ESC working group of adult congenital heart disease, and the International Society for Adult Congenital Heart Disease.

We are witnessing an unparalleled pandemic caused by the novel Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) associated with coronavirus disease 2019 (COVID-19). Current data show that SARS-CoV-2 results in mild flu-like symptoms in the majority of healthy and young patients affected. Nevertheless, the severity of COVID-19 respiratory syndrome and the risk of adverse or catastrophic outcomes are increased in patients with pre-existing cardiovascular disease. Patients with adult congenital heart disease (ACHD)-by definition-have underlying cardiovascular disease. Many patients with ACHD are also afflicted with residual haemodynamic lesions such as valve dysfunction, diminished ventricular function, arrhythmias or cyanosis, have extracardiac comorbidities, and face additional challenges regarding pregnancy. Currently, there are emerging data of the effect of COVID-19 on ACHD patients, but many aspects, especially risk stratification and treatment considerations, remain unclear. In this article, we aim to discuss the broad impact of COVID-19 on ACHD patients, focusing specifically on pathophysiology, risk stratification for work, self-isolation, hospitalization, impact on pregnancy, psychosocial health, and longer-term implications for the provision of ACHD care.

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

INTRODUCTION: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Health anxiety among adults with congenital heart disease.

PURPOSE OF REVIEW: Adults with congenital heart disease (CHD) may experience anxiety specifically related to their medical condition. This review introduces the concepts of health anxiety and heart-focused anxiety, summarizes what is currently known about heart-focused anxiety among adults with CHD and offers suggestions to help adult CHD providers address heart-focused anxiety in their patients. RECENT FINDINGS: Although minimal research has been conducted specific to this outcome, health anxiety may occur at any point across the lifespan of individuals with CHD. A recent study found that children and adolescents with CHD reported greater health anxiety than community peers. Health anxiety was commonly reported among adults with CHD presenting for psychological assessment. It was linked with older age, trait anxiety, perceived parental overprotection and greater CHD complexity in one study. SUMMARY: Adults with CHD face many potential health-related stressors, including cardiac symptoms, treatments and interventions throughout the lifespan (including surgeries and other invasive procedures), the impact of CHD on daily lives and longer-term health expectations. Providers should be aware that heart-focused anxiety among patients is understandable and perhaps common. Patient-centred education and psychological intervention should be integrated within a comprehensive approach to long-term disease management.

Phenotypes of adults with congenital heart disease around the globe: a cluster analysis.

OBJECTIVE: To derive cluster analysis-based groupings for adults with congenital heart disease (ACHD) when it comes to perceived health, psychological functioning, health behaviours and quality of life (QoL). METHODS: This study was part of a larger worldwide multicentre study called APPROACH-IS; a cross sectional study which recruited 4028 patients (2013-2015) from 15 participating countries. A hierarchical cluster analysis was performed using Ward's method in order to group patients with similar psychological characteristics, which were defined by taking into consideration the scores of the following tests: Sense Of Coherence, Health Behavior Scale (physical exercise score), Hospital Anxiety Depression Scale, Illness Perception Questionnaire, Satisfaction with Life Scale and the Visual Analogue Scale scores of the EQ-5D perceived health scale and a linear analogue scale (0-100) measuring QoL. RESULTS: 3768 patients with complete data were divided into 3 clusters. The first and second clusters represented 89.6% of patients in the analysis who reported a good health perception, QoL, psychological functioning and the greatest amount of exercise. Patients in the third cluster reported substantially lower scores in all PROs. This cluster was characterised by a significantly higher proportion of females, a higher average age the lowest education level, more complex forms of congenital heart disease and more medical comorbidities. CONCLUSIONS: This study suggests that certain demographic and clinical characteristics may be linked to less favourable health perception, quality of life, psychological functioning, and health behaviours in ACHD. This information may be used to improve psychosocial screening and the timely provision of psychosocial care.

Smoking among adult congenital heart disease survivors in the United States: Prevalence and relationship with illness perceptions.

The relationship between smoking and illness perceptions among congenital heart disease (CHD) survivors is unknown. The primary aims of the present study were to compare the smoking prevalence among CHD survivors to a nationally representative U.S. sample and examine the relationship between smoking and illness perceptions. CHD survivors (N = 744) from six U.S. sites participated in the study. The smoking prevalence among CHD survivors (9.3%) was lower than the general population (15.3%). However, 23.3% of CHD survivors with severe functional limitations smoked. Smoking prevalence differed by U.S. region, with a greater proportion of those attending CHD care in the Midwest reporting smoking (11.8%). The illness perception dimensions of Concern and Emotional Response were independently associated with smoking. Differences in illness perceptions enhance our understanding of smoking among CHD survivors and may guide interventions promoting positive health behaviors. The protocol for the study from which the present analyses were conducted was recorded at ClinicalTrials.gov: NCT02150603.

Recommendations for advance care planning in adults with congenital heart disease: a position paper from the ESC Working Group of Adult Congenital Heart Disease, the Association of Cardiovascular Nursing and Allied Professions (ACNAP), the European Association for Palliative Care (EAPC), and the International Society for Adult Congenital Heart Disease (ISACHD).

Survival prospects in adults with congenital heart disease (CHD), although improved in recent decades, still remain below expectations for the general population. Patients and their loved ones benefit from preparation for both unexpected and predictable deaths, sometimes preceded by a prolonged period of declining health. Hence, advance care planning (ACP) is an integral part of comprehensive care for adults with CHD. This position paper summarizes evidence regarding benefits of and patients' preferences for ACP and provides practical advice regarding the implementation of ACP processes within clinical adult CHD practice. We suggest that ACP be delivered as a structured process across different stages, with content dependent upon the anticipated disease progression. We acknowledge potential barriers to initiate ACP discussions and emphasize the importance of a sensitive and situation-specific communication style. Conclusions presented in this article reflect agreed expert opinions and include both patient and provider perspectives.

A Typology of Transition Readiness for Adolescents with Congenital Heart Disease in Preparation for Transfer from Pediatric to Adult Care.

PURPOSE: To understand the effectiveness of a nurse-led transition intervention by analyzing qualitative data generated in the context of a clinical trial. DESIGN & METHODS: Qualitative study of a two-session transition intervention conducted by registered nurses at two sites. Adolescents aged 16-17 years with moderate or complex congenital heart disease (CHD) had been randomized to a two-session transition intervention or usual care. Session 1 emphasized patient education including creation of a health passport and goal setting. Session 2, two months later, emphasized self-management. Qualitative data extracted from intervention logs, field notes and audio recordings of the sessions were analyzed for content and themes. RESULTS: Data from 111 transition intervention sessions with 57 adolescents were analyzed. Creating a health passport, goal setting, and role-plays were the elements of the intervention most valued by participants. A typology of transition readiness was identified: 1) the independent adolescent (5%), already managing their own care; 2) the ready adolescent who was prepared for transition after completing the intervention (46%); 3) the follow-up needed adolescent who was still in need of extra coaching (26%), and 4) the at-risk adolescent who warranted immediate follow-up (14%). Baseline knowledge and transition surveys scores validated the typology. CONCLUSIONS: A two-session nursing intervention met the transition needs of approximately half of adolescents with CHD. However, additional transition-focused care was needed by 40% of participants (groups 3 and 4). PRACTICE IMPLICATIONS: These findings will guide pediatric nurses and other healthcare professionals to optimize an individualized approach for ensuring transition readiness for adolescents with CHD.

Evaluation and Management of the Child and Adult With Fontan Circulation: A Scientific Statement From the American Heart Association.

It has been 50 years since Francis Fontan pioneered the operation that today bears his name. Initially designed for patients with tricuspid atresia, this procedure is now offered for a vast array of congenital cardiac lesions when a circulation with 2 ventricles cannot be achieved. As a result of technical advances and improvements in patient selection and perioperative management, survival has steadily increased, and it is estimated that patients operated on today may hope for a 30-year survival of >80%. Up to 70 000 patients may be alive worldwide today with Fontan circulation, and this population is expected to double in the next 20 years. In the absence of a subpulmonary ventricle, Fontan circulation is characterized by chronically elevated systemic venous pressures and decreased cardiac output. The addition of this acquired abnormal circulation to innate abnormalities associated with single-ventricle congenital heart disease exposes these patients to a variety of complications. Circulatory failure, ventricular dysfunction, atrioventricular valve regurgitation, arrhythmia, protein-losing enteropathy, and plastic bronchitis are potential complications of the Fontan circulation. Abnormalities in body composition, bone structure, and growth have been detected. Liver fibrosis and renal dysfunction are common and may progress over time. Cognitive, neuropsychological, and behavioral deficits are highly prevalent. As a testimony to the success of the current strategy of care, the proportion of adults with Fontan circulation is increasing. Healthcare providers are ill-prepared to tackle these challenges, as well as specific needs such as contraception and pregnancy in female patients. The role of therapies such as cardiovascular drugs to prevent and treat complications, heart transplantation, and mechanical circulatory support remains undetermined. There is a clear need for consensus on how best to follow up patients with Fontan circulation and to treat their complications. This American Heart Association statement summarizes the current state of knowledge on the Fontan circulation and its consequences. A proposed surveillance testing toolkit provides recommendations for a range of acceptable approaches to follow-up care for the patient with Fontan circulation. Gaps in knowledge and areas for future focus of investigation are highlighted, with the objective of laying the groundwork for creating a normal quality and duration of life for these unique individuals.

Advance care planning and palliative care in ACHD: the healthcare providers' perspective.

BACKGROUND: Advance care planning and palliative care are gaining recognition as critical care components for adults with CHD, yet these often do not occur. Study objectives were to evaluate ACHD providers' 1) comfort managing patients' physical symptoms and psychosocial needs and 2) perspectives on the decision/timing of advance care planning initiation and palliative care referral. METHODS: Cross-sectional study of ACHD providers. Six hypothetical patients were described in case format, followed by questions regarding provider comfort managing symptoms, initiating advance care planning, and palliative care referral. RESULTS: Fifty providers (72% physicians) completed surveys. Participants reported low levels of personal palliative care knowledge, without variation by gender, years in practice, or prior palliative care training. Providers appeared more comfortable managing physical symptoms and discussing prognosis than addressing psychosocial needs. Providers recognised advance directives as important, although the percentage who would initiate advance care planning ranged from 18 to 67% and referral to palliative care from 14 to 32%. Barriers and facilitators to discussing advance care planning with patients were identified. Over 20% indicated that advance care planning and end-of-life discussions are best initiated with the development of at least one life-threatening complication/hospitalisation. CONCLUSIONS: Providers noted high value in advance directives yet were themselves less likely to initiate advance care planning or refer to palliative care. This raises the critical questions of when, how, and by whom discussion of these important matters should be initiated and how best to support ACHD providers in these endeavours.

The Fontan outcomes network: first steps towards building a lifespan registry for individuals with Fontan circulation in the United States.

The Fontan Outcomes Network was created to improve outcomes for children and adults with single ventricle CHD living with Fontan circulation. The network mission is to optimise longevity and quality of life by improving physical health, neurodevelopmental outcomes, resilience, and emotional health for these individuals and their families. This manuscript describes the systematic design of this new learning health network, including the initial steps in development of a national, lifespan registry, and pilot testing of data collection forms at 10 congenital heart centres.

Anxiety and Depression in Adults with Congenital Heart Disease: When to Suspect and How to Refer.

PURPOSE OF REVIEW: Adults with congenital heart disease face many potential psychosocial challenges. This review focuses on their experiences of clinically significant anxiety and depression and provides recommendations for appropriately timed referrals to mental health professionals. RECENT FINDINGS: Adults with congenital heart disease have a higher probability of experiencing symptoms consistent with mood and anxiety disorders, yet risk factors are not well understood. Although patients as a group are often interested in psychological treatment, there is currently under-recognition and under-treatment of mental health concerns. As provider and patient awareness of the biopsychosocial implications of living with congenital heart disease continues to grow, the focus of comprehensive care is broadening to include attention to psychosocial well-being. Care teams are encouraged to create a culture that fosters open and ongoing dialog about emotional well-being, including depression and anxiety, and includes reliable processes for mental health referrals.

Learning strategies among adult CHD fellows.

OBJECTIVE: Subspecialisation is increasingly a fundamental part of the contemporary practice of medicine. However, little is known about how medical trainees learn in the modern era, and particularly in growing and relatively new subspecialties, such as adult CHD. The purpose of this study was to assess institutional-led and self-directed learning strategies of adult CHD fellows. METHODS: This international, cross-sectional online survey was conducted by the International Society for Adult Congenital Heart Disease and consisted primarily of categorical questions and Likert rating scales. All current or recent (i.e., those within 2 years of training) fellows who reported training in adult CHD (within adult/paediatric cardiology training or within subspecialty fellowships) were eligible. RESULTS: A total of 75 fellows participated in the survey: mean age: 34 ± 5; 35 (47%) female. Most adult CHD subspecialty fellows considered case-based teaching (58%) as "very helpful", while topic-based teaching was considered "helpful" (67%); p = 0.003 (favouring case-based). When facing a non-urgent clinical dilemma, fellows reported that they were more likely to search for information online (58%) than consult a faculty member (29%) or textbook (3%). Many (69%) fellows use their smartphones at least once daily to search for information during regular clinical work. CONCLUSIONS: Fellows receiving adult CHD training reported a preference for case-based learning and frequent use of online material and smartphones. These findings may be incorporated into the design and enhancement of fellowships and development of online training resources.

Psychological distress in adults with congenital heart disease: focus beyond depression.

BACKGROUND: Adults with congenital heart disease face psychological challenges although an understanding of depression vs. anxiety symptoms is unclear. We analyzed the prevalence of elevated symptoms of anxiety and depression and explored associations with demographic and medical factors as well as quality of life. METHODS: Adults with congenital heart disease enrolled from an outpatient clinic completed the Hospital Anxiety and Depression Scale and two measures of quality of life: the Linear Analogue Scale and the Satisfaction with Life Scale. Medical data were obtained by chart review. RESULTS: Of 130 patients (median age = 32 years; 55% female), 55 (42%) had elevated anxiety symptoms and 16 (12%) had elevated depression symptoms on subscales of the Hospital Anxiety and Depression Scale. Most patients with elevated depression symptoms also had elevated anxiety symptoms (15/16; 94%). Of 56 patients with at least one elevated subscale, 37 (66%) were not receiving mental health treatment. Compared to patients with 0 or 1 elevated subscales, patients with elevations in both (n=15) were less likely to be studying or working (47% vs. 81%; p=0.016) and reported lower scores on the Linear Analogue Scale (60 vs. 81, p<0.001) and the Satisfaction with Life Scale (14 vs. 28, p<0.001). CONCLUSIONS: Among adults with congenital heart disease, elevated anxiety symptoms are common and typically accompany elevated depressive symptoms. The combination is associated with unemployment and lower quality of life. Improved strategies to provide psychosocial care and support appropriate engagement in employment are required.

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association.

Life expectancy and quality of life for those born with congenital heart disease (CHD) have greatly improved over the past 3 decades. While representing a great advance for these patients, who have been able to move from childhood to successful adult lives in increasing numbers, this development has resulted in an epidemiological shift and a generation of patients who are at risk of developing chronic multisystem disease in adulthood. Noncardiac complications significantly contribute to the morbidity and mortality of adults with CHD. Reduced survival has been documented in patients with CHD with renal dysfunction, restrictive lung disease, anemia, and cirrhosis. Furthermore, as this population ages, atherosclerotic cardiovascular disease and its risk factors are becoming increasingly prevalent. Disorders of psychosocial and cognitive development are key factors affecting the quality of life of these individuals. It is incumbent on physicians who care for patients with CHD to be mindful of the effects that disease of organs other than the heart may have on the well-being of adults with CHD. Further research is needed to understand how these noncardiac complications may affect the long-term outcome in these patients and what modifiable factors can be targeted for preventive intervention.

Adapted Motivational Interviewing to Promote Exercise in Adolescents With Congenital Heart Disease: A Pilot Trial.

PURPOSE: To assess a motivational interviewing (MI) intervention to improve moderate-to-vigorous physical activity (MVPA) in adolescents with congenital heart disease. METHODS: Intervention participants received one-on-one telephone-based adapted MI sessions over 3 months. Outcomes were acceptability, change mechanisms (stage of change and self-efficacy), and limited efficacy (physical activity, fitness, and quality of life). RESULTS: Thirty-six participants were randomized. Intervention participants completed 4.2 ± 1.2/6 MI sessions, with no improvements in the high self-efficacy or stage of change observed. Participants accumulated 47.24 ± 16.36 minutes of MVPA/day, and had comparable outcomes to peers without heart disease (except for functional capacity). There was no significant difference in change in any outcome by group. CONCLUSIONS: The intervention was acceptable, but effectiveness could not be determined due to the nature and size of sample. CLINICAL RELEVANCE: Pediatric cardiac rehabilitation remains the sole effective intervention to increase MVPA in this population.

Regional variation in quality of life in patients with a Fontan circulation: A multinational perspective.

BACKGROUND: Impaired quality of life (QOL) is associated with congenital heart disease (CHD) and country of residence; however, few studies have compared QOL in patients with differing complexities of CHD across regional populations. The current study examined regional variation in QOL outcomes in a large multinational sample of patients with a Fontan relative to patients with atrial septal defects (ASDs) and ventricular septal defects (VSDs). METHODS: From the Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease-International Study (APPROACH-IS), 405 patients (163 Fontan and 242 ASD/VSD) across Asia, Europe, and North America provided consent for access to their medical records and completed a survey evaluating QOL (0 to 100 linear analog scale). Primary CHD diagnosis, disease complexity, surgical history, and documented history of mood and anxiety disorders were recorded. Differences in QOL, medical complications, and mood and anxiety disorders between Fontan and ASD/VSD patients, and across geographic regions, were examined using analysis of covariance. Hierarchical regression analyses were conducted to identify variables associated with the QOL ratings. RESULTS: Patients with a Fontan reported significantly lower QOL, and greater medical complications and mood and anxiety disorders relative to patients with ASD/VSD. Inpatient cardiac admissions, mood disorders, and anxiety disorders were associated with lower QOL among patients with a Fontan, and mood disorders were associated with lower QOL among patients with ASD/VSD. Regional differences for QOL were not observed in patients with a Fontan; however, significant differences were identified in patients with ASD/VSD. CONCLUSIONS: Regional variation of QOL is commonplace in adults with CHD; however, it appears affected by greater disease burden. Among patients with a Fontan, regional variation of QOL is lost. Specific attempts to screen for QOL and mood and anxiety disorders among CHD patients may improve the care of patients with the greatest disease burden.

Readiness for Transition to Adult Health Care for Young Adolescents with Congenital Heart Disease.

This study evaluates transition readiness, medical condition knowledge, self-efficacy, and illness uncertainty in young adolescents (ages 12 to 15 years) with congenital heart disease (CHD), and medical, patient, and parental factors associated with transition readiness. We enrolled 82 patients with moderate or complex CHD (n = 36, 44% male; mean age 13.6 ± 1.3 years), and their parents. Patients completed standardized self-report measures: Transition Readiness Assessment Questionnaire (TRAQ), MyHeart scale, General Self-Efficacy scale, and Children's Uncertainty in Illness Scale. Parents completed the MyHeart scale and demographic information. Many young adolescents had not discussed transfer with a health care provider (n = 20, 24%) or parent (n = 34, 41%). Transition readiness was higher among patients who were older, more knowledgeable about their condition, had a history of primary cardiac repair and greater self-efficacy, and was lower for boys and patients on cardiac medications. Transition readiness was unrelated to CHD diagnosis and patients' illness uncertainty. Patients' self-advocacy skills were superior to their chronic disease self-management skills. Increased parental medical condition knowledge was positively correlated with patient knowledge, and patient-parent discussion of transfer was associated with increased patient's self-management skills. Transition is not uniformly discussed with young adolescent CHD patients. Parental involvement is correlated with increased transition readiness and patient disease self-management skills. Young adolescent transition programs should focus on education around improving patient medical condition knowledge, promote chronic disease self-management skills development, and include parental involvement.