HomeCirculationVol. 123, No. 13Best Practices in Managing Transition to Adulthood for Adolescents With Congenital Heart Disease: The Transition Process and Medical and Psychosocial Issues Free AccessResearch ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessResearch ArticlePDF/EPUBBest Practices in Managing Transition to Adulthood for Adolescents With Congenital Heart Disease: The Transition Process and Medical and Psychosocial IssuesA Scientific Statement From the American Heart Association Craig Sable, MD, FAHA, Elyse Foster, MD, FAHA, Karen Uzark, PhD, PNP, FAHA, Katherine Bjornsen, BSN, ARNP, Mary M. Canobbio, RN, MN, FAHA, Heidi M. Connolly, MD, Thomas P. Graham, MD, FAHA, Michelle Z. Gurvitz, MD, MS, Adrienne Kovacs, PhD, CPsych, Alison K. Meadows, MD, PhD, Graham J. Reid, PhD, CPsych, John G. Reiss, PhD, Kenneth N. Rosenbaum, MD, Paul J. Sagerman, MD, MS, Arwa Saidi, MB, BCh, Rhonda Schonberg, MS, Sangeeta Shah, MD, Elizabeth Tong, MS, RN, CPNP, FAHA and Roberta G. Williams, MD, FAHAon behalf of the American Heart Association Congenital Heart Defects Committee of the Council on Cardiovascular Disease in the Young, Council on Cardiovascular Nursing, Council on Clinical Cardiology, and Council on Peripheral Vascular Disease Craig SableCraig Sable Search for more papers by this author , Elyse FosterElyse Foster Search for more papers by this author , Karen UzarkKaren Uzark Search for more papers by this author , Katherine BjornsenKatherine Bjornsen Search for more papers by this author , Mary M. CanobbioMary M. Canobbio Search for more papers by this author , Heidi M. ConnollyHeidi M. Connolly Search for more papers by this author , Thomas P. GrahamThomas P. Graham Search for more papers by this author , Michelle Z. GurvitzMichelle Z. Gurvitz Search for more papers by this author , Adrienne KovacsAdrienne Kovacs Search for more papers by this author , Alison K. MeadowsAlison K. Meadows Search for more papers by this author , Graham J. ReidGraham J. Reid Search for more papers by this author , John G. ReissJohn G. Reiss Search for more papers by this author , Kenneth N. RosenbaumKenneth N. Rosenbaum Search for more papers by this author , Paul J. SagermanPaul J. Sagerman Search for more papers by this author , Arwa SaidiArwa Saidi Search for more papers by this author , Rhonda SchonbergRhonda Schonberg Search for more papers by this author , Sangeeta ShahSangeeta Shah Search for more papers by this author , Elizabeth TongElizabeth Tong Search for more papers by this author and Roberta G. WilliamsRoberta G. Williams Search for more papers by this author and on behalf of the American Heart Association Congenital Heart Defects Committee of the Council on Cardiovascular Disease in the Young, Council on Cardiovascular Nursing, Council on Clinical Cardiology, and Council on Peripheral Vascular Disease Originally published28 Feb 2011https://doi.org/10.1161/CIR.0b013e3182107c56Circulation. 2011;123:1454–1485Other version(s) of this articleYou are viewing the most recent version of this article. Previous versions: January 1, 2011: Previous Version 1 Many children born with complex childhood illnesses that historically caused early death are now surviving into adulthood with the expectation of leading meaningful and productive lives. They will ultimately need to transition their care from pediatric to adult-centered care. Unfortunately, in the absence of structured programs to guide this transition, there is often delayed or inappropriate care, improper timing of the transfer of care, and undue emotional and financial stress on the patients, their families, and the healthcare system. At its worst, and as frequently happens now, patients are lost to appropriate follow-up. In fact, the number of adults with congenital heart disease (CHD) in the United States is rising exponentially and now exceeds 1 000 000.1–7 At least half of these patients may have complex CHD. Fewer than 30% of adults with CHD are seen by appropriate specialized providers. Fewer than 15% of these patients, who are seen in specialty adult CHD (ACHD) clinics, have CHD that is classified as severe.8 Thus, adolescents with CHD constitute a growing population of individuals for whom a well-planned and well-executed “transition process” is essential.The goals of a formal transition program are to prepare young adults for transfer of care. It should provide uninterrupted health care that is patient centered, age and developmentally appropriate, flexible, and comprehensive. It should include age-appropriate education about medical conditions and promote skills in communication, decision making, self-care, and self-advocacy.9–13 It should foster greater personal and medical independence and a greater sense of control over health, healthcare decisions, and psychosocial environment. The ultimate goal of a transition program is to optimize the quality of life (QOL), life expectancy, and future productivity of young patients.14We acknowledge that the development of ideal transition programs is a laudable goal that may not be achievable in the current healthcare environment. With the recognition that adolescents and young adults receive their care from a diverse group of providers, this document is intended for pediatric and adult cardiologists who are likely to care for this group of patients. We have addressed diverse topics that pertain to the care of adolescents and young adults with CHD, beginning with issues directly related to transition. The American Heart Association (AHA) classification of recommendations and levels of evidence for practice guidelines were used when applicable. The classification and levels of evidence are shown in Table 1.Table 1. Applying Classification of Recommendation and Level of EvidenceTable 1. Applying Classification of Recommendation and Level of Evidence* Data available from clinical trials or registries about the usefulness/efficacy in different subpopulations, such as sex, age, history of diabetes, history of prior myocardial infarction, history of heart failure, and prior aspirin use. A recommendation with Level of Evidence B or C does not imply that the recommendation is weak. Many important clinical questions addressed in the guidelines do not lend themselves to clinical trials. Although randomized trials are unavailable, there may be a very clear clinical consensus that a particular test or therapy is useful or effective.† For comparative effectiveness recommendations (Class I and IIa; Level of Evidence A and B only), studies that support the use of comparator verbs should involve direct comparisons of the treatments or strategies being evaluated.Concepts of Transition CareIn the United States, ≈14% of children are identified as having a special healthcare need.15 More than 1 in every 5 households has at least 1 child with some special needs. This number represents a slight increase from data reported in 2001, perhaps because of increasing diagnostic capability, increased medical access, and an increasing survival rate for many congenital or chronic conditions that historically were associated with high childhood mortality. Advances in medical and surgical care for children with congenital cardiovascular disease have dramatically improved their life expectancy, resulting in 85% to 90% of these children surviving to adulthood.16Transition is defined by the Merriam-Webster dictionary as “a movement, development, or evolution from one form, stage, or style to another.” Medical transition is the process of moving from a pediatric medical system to an adult one. Transfer refers to the actual point in time at which responsibility for patient care is “handed off” to the adult provider. Developmental transitions occur at specific milestones throughout the life spectrum.The American Academy of Pediatrics states, “The goal of transition in health care for young adults with special health care needs is to maximize lifelong functioning and potential through the provision of high-quality, developmentally appropriate health care services that continue uninterrupted as the individual moves from adolescence to adulthood.”17 However, for children with congenital or long-term healthcare needs, transition to an adult lifestyle or environment is often made more difficult by the inability to obtain quality health care as they leave their pediatric providers. The American Academy of Pediatrics' Medical Home Initiative states that 90% of children with special needs reach their 21st birthday, yet 45% of them lack access to a physician who is familiar with their condition(s). In addition, 30% of all young adults 18 to 24 years of age lack a payment source for their health care.18 The Maternal Child Health Bureau, a division of the Health Resources and Services Administration, US Department of Health and Human Services, in response to Healthy People 2010 national health objectives and the President's New Freedom Initiative, includes “transition to adult health care, work, and independence” in 6 core systems changes that are deemed necessary to improve the overall health of children with special needs.19To date, transition outcomes overall have been shown to be suboptimal, particularly among ethnic minorities. Better outcomes are seen for older teens and those receiving care within a “medical home.”20 Several steps have been identified that are paramount to the successful transition of a child with special needs from a family-centered pediatric system to a patient-centered adult long-term care system, including the following: Comprehensive care that is coordinated and managed through a medical home (usually a primary care provider but in some instances a tertiary care center or a subspecialty practice)Access to healthcare financingEducation of adult providers in managing chronic conditions previously limited to the pediatric populationOngoing, coordinated communication between patients, families, and pediatric and adult healthcare providers to facilitate transition and transferReid et al21 reported in 2004 that only 48% of adolescents with CHD underwent successful transition. Their data suggested that ongoing care during adolescence with continued discussion of the importance of transition was very important to successful transition. The standard of transition as defined by the Society of Adolescent Medicine is “a purposeful, planned process that addresses the medical, psychosocial and educational/vocational needs of adolescents and young adults with chronic physical and medical conditions as they move from child-centered to adult-oriented health care systems.”9 The aim of the present statement is to provide guidelines for clinicians to promote early and high-quality care and support for their patients with CHD for successful transition of care.Timing of TransitionThe transitional process depends on the patient's medical and developmental status and should be individualized.22 The developmental status of the patient should be considered relative to normal developmental milestones (Table 2),4 with special considerations for how chronic illness (Table 3)23 and congenital heart defects (Table 4) impact these milestones.4 The American College of Cardiology task force and the recent “ACC/AHA Guidelines on the Management of Adults With Congenital Heart Disease” recommended that the transition process start at 12 years of age16 to prepare the patient for transfer to adult care.Table 2. Typical Life Span Developmental Tasks After Childhood for Individuals Without a Chronic Illness,4 by Age GroupDomainsMid Adolescence (14–16 y)Late Adolescence (16–19 y)Young Adulthood (19–35 y)Middle Adulthood (Mid 30s–Mid 40s)Maturity (Mid 40s–Mid 60s)Old Age (Mid 60s+)PhysicalCompleting pubertyMaintaining physical healthMaintaining physical healthAccepting physiological changesAccepting physiological changes and onset of common illnesses of adulthoodAdjusting to decreased physical health and/or illnessPositive body imagePositive body imageSocial and family relationsDatingDatingSeparation from family of originChildrearingAssisting teenage children in developing independenceGrandparentingEstablishing peer relationshipsEstablishing peer relationshipsSelecting a mate/partnerAdjusting to aging parentsCoping with loss of peersIncreasing independence from familyIncreasing independence from familyStarting a familyFinding a social groupEmotionalCompetence in emotional self-regulationCompetence in emotional self-regulationMaintaining emotional/ mental healthMaintaining emotional/ mental healthMaintaining emotional/ mental healthMaintaining emotional/ mental healthEducation and vocationCompleting high schoolHigher education and/or entering work forceEstablishing and/or maintaining an occupationMaintaining standard of livingSatisfaction with occupational productivityRetirementHealth behaviorsAppropriate exercise and dietAppropriate exercise and dietAppropriate exercise and dietAppropriate exercise and dietAppropriate exercise and dietAppropriate exercise and dietAvoiding health-risk behaviorsAvoiding health-risk behaviorsAvoiding health-risk behaviorsMaintaining appropriate weightMaintaining appropriate weightMaintaining appropriate weightMaintaining appropriate weightMaintaining appropriate weightMaintaining appropriate weightObtaining screening for health problems as recommended for age and sexObtaining screening for health problems as recommended for age and sexObtaining screening for health problems as recommended for age and sexPersonality and identitySex-role developmentIncreasing independenceBalancing independence and interdependence with family and friendsBalancing independence and interdependence with family and friendsBalancing independence and interdependence with family and friendsAcceptance of one's life historyAcceptance of deathTable 3. Transition Timeline for Adolescents With Special Healthcare Needs: Chronic Illnesses/Physical Disabilities23Table 3. Transition Timeline for Adolescents With Special Healthcare Needs: Chronic Illnesses/Physical Disabilities23SSI indicates Supplemental Security Income.Table 4. Life Span Developmental Tasks and Issues for the Adolescent and Adult With Congenital Heart Defects,4 by Age GroupDomainsMid Adolescence (14–16 y)Late Adolescence (16–19 y)Young Adulthood (19–35 y)Middle Adulthood (Mid 30s–Mid 40s)*PhysicalCoping with body image and limitations in physical functioningCoping with body image and limitations in physical functioningGradual or acute decreases in physical functioning; burden/complications with onset of common illnesses of adulthoodGradual or acute decreases in physical functioning; burden/complications with onset of common illnesses of adulthoodSocial and family relationsPeer acceptance of physical appearance/limitations; coping with stigmatization; lack of social support for CHD issuesDecisions about dating; increasing independence from family; lack of social support for CHD issuesDecisions regarding life partner and reproduction; coping with loss of normative family life cycle; finding a social group/networkAddressing the impact of premature death on partner, any children, and extended familyEmotionalManaging anxiety-provoking medical procedures; maintaining emotional adjustment during period of critical transitionsManaging anxiety-provoking medical procedures; maintaining emotional adjustment during period of critical transitionsManaging anxiety-provoking medical procedures; avoiding arrhythmia-related anxiety/ phobic reactions; avoiding despair, depression, or anxiety; maintaining emotional/mental healthManaging anxiety-provoking medical procedures; avoiding arrhythmia-related anxiety/ phobic reactions; avoiding despair, depression, or anxiety; maintaining emotional/mental healthEducation and vocationCoping with possible intellectual and/or learning disabilitiesSelecting educational and vocational goals appropriate to present/future abilitiesStigmatization/discrimination in obtaining employment; maintaining employment during medical crisesMaintaining/changing employment and/or career goals with decreases in physical functioningMedicalTaking some responsibility for medical care; learning appropriate health behaviorsIncreasing responsibility for medical care; transition to adult care; knowledge of diagnosis, prognosis, and associated health behaviorsPrimary responsibility for medical care; knowledge of prognosis; reoperation(s); CHD complications; coping with medical procedures and hospitalization; coping with procedure-related painPrimary responsibility for medical care; knowledge of prognosis; reoperation(s); CHD complications; coping with medical procedures and hospitalization; coping with procedure-related painHealth behaviorsAvoiding initiation of risky health behaviors; maintaining appropriate weight and getting exercise; maintaining oral hygiene and preventing endocarditisRegular medical follow-up; avoiding risky health behaviors; maintaining appropriate weight; getting appropriate exercise; maintaining oral hygiene and preventing endocarditisRegular medical follow-up; avoiding risky health behaviors; maintaining appropriate weight; getting appropriate exercise; maintaining oral hygiene and preventing endocarditisRegular medical follow-up; avoiding risky health behaviors; maintaining appropriate weight; getting appropriate exercise; maintaining oral hygiene and preventing endocarditisPersonality and identityIntegration of CHD into self; acceptance of being different and uniqueLack of control over health outcomes; increasing independenceBalancing independence and interdependence with family and friendsResolving loss of typical life achievements; facing prospect of premature deathCHD indicates congenital heart disease.*Life expectancy varies with lesion severity and increases with improved medical care.“Envisioning a future” is an important first stage in the transition process and occurs at the time of diagnosis of a chronic condition.24 This emphasizes starting the discussion early and includes prompting parents to consider future expectations for their child's education, employment, and independent living.24 Just as patients are prepared to take increased responsibility for their health care, parents also benefit from education and support as they gradually share and then hand over the primary responsibility for healthcare management. From initial diagnosis, discussion with parents should reference transitional topics to help parents prepare for this shift in responsibility. Preparing the patient for self-care is an ongoing process that should begin in early childhood and continue into adulthood25 to allow the development of the necessary self-care skills.26 Knowledge of the developmental stages in adolescence and the impact of chronic illness is essential for a successful transition.27 During childhood, the patient should become increasingly involved in direct discussions about his or her diagnosis, medications, and exercise limitations. In the teenage years, discussions about heart-healthy behaviors and the risks of smoking, alcohol, and drugs should be introduced and reviewed regularly. Vocational and employment advice should continue into early adulthood,25 and birth control, pregnancy, genetics, and long-term prognosis should also be emphasized.Table 3 describes practical steps that can be undertaken in the designated age group, with the assumption of appropriate developmental status. In patients with developmental delay, this timeline will have to be altered as appropriate. Self-care education should not culminate with transfer to adult care but is an ongoing educational process to address life changes. Even patients who continue to see the same healthcare providers for CHD care should undergo a process of transition similar to that provided by family practitioners and internal medicine/pediatric specialists who do not transfer patient care.28Before transfer to ACHD care, ensure that any ongoing medical or surgical issues are addressed. A portable and accessible summary of medical history allows for effective collaboration among healthcare providers.17 A transfer-of-care letter with relevant medical and psychosocial information and a care plan should be provided to the regional ACHD center in a timely fashion, with a copy for the patient to keep in case the ACHD provider changes.The simultaneous transfer of care of the young adult to adult general care and subspecialty care during a period of significant life changes can be overwhelming. This simultaneous transfer can be avoided by transitioning the adolescent with chronic health needs to a family practice or an internal medicine/pediatric provider or to a medical care home for primary care. This provider can then follow the patient into adulthood while the young adult is transferred to ACHD care. Consider referring patients with complex medical conditions to a regional “medical home,”29 because that facilitates better medical transition.20Social/Family Dynamics: Impact of Chronic Illness as Related to CHDPatientsCHD is a chronic illness, and as such, the need for long-tem medical monitoring, medication adjustments, repeated hospitalizations, and illness-related absences from school can affect the adolescent's self-image and self-esteem, as well as delay completion of normal developmental tasks.27,30 During the transition process, it is important for providers to understand how the individual adolescent and his or her family interpret the diagnosis and prognosis. Research consistently has shown that psychological adjustment is not proportionally related to the severity of the cardiac defect.31–36 Healthcare providers must also take care not to place their own judgment value on an adolescent's subjective experience and coping process. Cultural and family dynamics and past experiences both within and outside of the healthcare environment are other important factors to consider when preparing adolescents and their families for the transition process.Early research reported conflicting results about the emotional and behavioral problems in adolescents with CHD. Although some studies reported anxiety, diminished self-esteem, depression, increased dependency, and poor emotional and social adjustment among adolescents with CHD,37–40 others reported no significant psychopathology.41,42 This discrepancy has been attributed to differences in methodology, types of defects, and small sample size.43 A recent meta-analysis of 11 studies of the psychological problems of children and adolescents with CHD revealed that older children and adolescents had an increased risk of internalizing problems (eg, depression, anxiety) and externalizing behavior problems (eg, aggression, hyperactivity) compared with healthy control subjects.35 One study examined patients at a single institution before 1980 and a decade later to ascertain whether newer treatment modalities and improved surgical outcomes provided more favorable psychosocial outcomes. No differences were found; improvements in psychological outcomes did not track with medical outcomes.44 On balance, the percentage of adolescents and adults with CHD who have emotional and behavioral problems is slightly greater than in the general population.45Qualitative research using semistructured interviews has recently provided insight into the social experiences of adolescents with CHD. Studies suggest that although adolescents with CHD may try to live a normal life, these teens describe feeling “different” and set apart from their peers because of their heart disease.46 Adolescents speak of common dilemmas and concerns, including whether or not to disclose their heart disease to others; the fear of rejection and other effects of disclosure on social relationships; and the fear of forming close relationships. Adolescents may have recognized the need to take a more active role in managing their health care, but they were unsure how to begin.46 In the report by Tong et al,46 adolescents were concerned about planning for the future given their uncertain longevity. In another study, both adolescents and young adults tended to have unrealistic expectations of their life expectancy, and qualitative data suggested a lack of awareness and understanding of their future health risks.47 Compared with their healthy peers, adolescents with CHD are less likely to smoke, drink alcohol, or use illicit drugs and are less likely to be sexually active.48,49 Birks et al50 found that although the participants in their study coped well, concerns related to being unable to physically keep up with their peers or consume alcohol as freely as their peers (because of medication side effects) were especially challenging and isolating. Not engaging in common risky behaviors during adolescence is appropriate given the negative health implications; however, this creates additional challenges for social acceptance and integration for adolescents with CHD. Adolescents in another qualitative study developed strategies to deal with the physical limitations and decreased stamina imposed by the disease, but the discrimination and bullying they experienced from peers left them feeling particularly angry and excluded.51Evaluation of QOL in adolescents with CHD by use of the Pediatric Quality of Life Inventory revealed significantly lower overall perceived QOL, including physical, psychosocial, and school functioning, with the greatest difference in the domain of school functioning. Specific psychosocial issues included feeling angry and being worried about the future, and these were unrelated to the severity of the cardiac defect. In contrast, Culbert et al52 used the Child Health Questionnaire to examine health status or QOL in adolescents with transposition of the great arteries 11 to 15 years after their initial repair and concluded that health status as perceived by this specific cohort was excellent and showed no significant difference from the published normative data. In addition, a more positive outcome was found for subjects who had the arterial switch procedure versus the atrial (Senning/Mustard) repair.52 Although patients undergoing atrial switch may have had longer periods of cyanosis, they were born during the same time period as the arterial switch patients (1985–1989).Overall, the combined quantitative and qualitative data on the QOL of teenagers with CHD suggest that they face unique challenges in addition to those typically confronted during adolescence. Clinically, it is important to understand the reasons behind adolescent perceptions of their health status and QOL to provide appropriate education and support during the transition process.Parent/FamilyTransition planning for adolescents with CHD cannot occur without a tandem evaluation of the impact of the diagnosis on their parents. During their formative years, children and adolescents learn and take cues from their parents on how to manage and adapt to their illness. Although parents may be expected to take an active role in the transition process, some parents find this difficult. Because of the chronic nature of CHD, many parents have devoted substantial time and energy to monitoring their child's medical care to ensure optimal health and survival. Many studies suggest that parents of children with CHD are more likely to experience elevated symptoms of depression, anxiety, stress, and anger, as well as poorer QOL.53–57 Research has shown that parental anxiety about raising a child with CHD is more directly related to the diagnosis per se and not the severity of the defect.31,32,53,54,56,58 In addition, the quality of the parent-child relationship, and not disease severity, holds the key to successful transition, with perceived parental acceptance having a more positive effect on adolescent psychological well-being.33,59Parental anxiety can lead to overprotection and may also explain parents' reluctance to shift greater responsibility for illness management to their adolescent.27,60 Many adults with CHD recall parental overprotection during their teenage years.32,51,61,62 Parenting styles can either hinder or support the appropriate integration of illness into lifestyle.63 Furthermore, even after parents have relinquished control over their adult child's healthcare management, control might shift back to the parents in situations when there is dissatisfaction with medical communication or competency of care.62 McDonagh64 highlighted the importance of “negotiating” parental involvement throughout the transition process.Parent-adolescent perceptions of QOL may not always be in agreement. Casey et al65 found that parents underestimated their child's physical exercise tolerance in 80% of cases, and in their study on QOL in children with heart disease, Uzark et al36 found that parent-adolescent agreement about QOL was consistently lower for the adolescent group than for their younger counterparts, with the greatest difference occurring in the domain of social functioning. Other studies, however, have found that children and adolescents with CHD reported more behavioral/emotional problems and poorer QOL than their parents reported about them.66,67 To fully understand the impact of CHD on adolescents, therefore, it is important to gather information from both teens and their parents during the transition process. Optimal transition involves collaboration; in addition to patients and healthcare providers, parents and other family members must be integrated into the process.There are limited data on the impact of CHD on other family members. Janus and Goldberg68 looked at the impact on healthy siblings. High treatment intensity was associated with high accomm
