REVIEWS So hard to say goodbye: transition from paediatric to adult cardiology care Adrienne H. Kovacs and Brian W. McCrindle Abstract | Important medical advances have created a large and growing population of adults who were diagnosed with congenital or paediatric-onset cardiovascular conditions as children and now require specialized cardiac care as adults. Although some adult patients continue to be cared for by paediatric cardiology programmes, guidelines recommend that patients transfer from paediatric to adult care at 18–21 years of age. Unfortunately, lapses in care during the transfer period are common and associated with poor health outcomes. Comprehensive transition programmes are necessary to maintain continuity of care and effectively prepare adolescents and young adults for the differences between paediatric and adult cardiac care programmes. Transition, unlike transfer, is an extended process that begins during childhood or early adolescence and focuses on patient education and fostering self-management skills and appropriate interdependence. The perspectives of patients, families, and care providers should be recognized and incorporated into transition programmes, and should be used to adapt strategies to meet the needs of individual patients and families. This Review summarizes the current knowledge on the transition of young patients from paediatric to adult cardiology care from the perspectives of all stakeholders, and offers practical recommendations for the development of transition programmes. Kovacs, A. H. & McCrindle, B. W. Nat. Rev. Cardiol. 11, 51–62 (2014); published online 12 November 2013; doi:10.1038/nrcardio.2013.172

Introduction

Toronto Congenital Cardiac Centre for Adults, Peter Munk Cardiac Centre, University Health Network, 585 University Avenue, Toronto, ON M5G 2N2, Canada (A. H. Kovacs). Labatt Family Heart Centre, The Hospital for Sick Children, 555 University Avenue, Toronto, ON M5G 1X8, Canada (B. W. McCrindle). Correspondence to: B. W. McCrindle brian.mccrindle@ sickkids.ca

The majority of patients with childhood cardio­vascular conditions now survive to adulthood because of advances in clinical care.1 The number of adult survivors now exceeds the number of current paediatric patients.2 Adult survivors include patients with con­ditions such as congenital heart disease (CHD), arrhythmias, cardio­ myopathies, acquired paediatric conditions such as Kawasaki disease and rheumatic heart disease, or risk factors for atherosclerosis that present during child­ hood or adolescence. The spectrum of conditions is further broadened by the evolving medical and surgical algorithms through which these patients are managed, including cardiac transplantation. Paediatric con­ditions affect the adult lives of these patients, as few are cured and many are, at best, palliated. Long-term, continuous care by qualified providers is necessary for the major­ ity of patients to prevent and manage morbidities and to maximize functional health status and health-related quality of life. The transition can be difficult, as it occurs during a challenging developmental period for the patient and bridges different health-care systems. Failing to ensure continuity of care during the trans­ition from adolescence to adulthood causes interruptions or lapses in care, which are, unfortunately, prevalent.3–5 Hence, a successful and sustained transfer and transition process to appropriate adult-care providers is essential and b­enefits all stakeholders. Competing interests The authors declare no competing interests.

This Review summarizes what is currently known about the transition of young patients from paediatric to adult cardiology care from the perspectives of patients, parents, and care providers. Much of the existing research on transition has focused on patients with CHD, there­ fore practical recommendations offered in this Review could be used to transition patients with CHD as well as other paediatric-onset cardiac conditions.

Concepts of transfer and transition Transfer to adult care for many patients, families, and care providers is a major event. The transfer event itself is frequently characterized by the sudden realization of a last visit to their paediatric care providers, the transfer of medical records, and expectations that an appoint­ ment with an adult-care cardiologist will be made and kept and that care will be bridged and uninterrupted.6 Even for patients cared for in centres with affiliated adult CHD programmes, to which their future care can be directed, many patients fail to achieve a successful transfer or attend even an initial appointment with an adult-care provider.7 The absence of a process of trans­ ition or preparation individualized to the patient often contributes to this failure. Transition is an extended and comprehensive process; transfer is only one compo­ nent of this process. Transition takes into account the perspectives of the patient and his or her family, pae­ diatric and adult care providers, and the health-care system.8–10 Nonetheless, the focus of the process is on the patient, and involves education, preparation, and

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REVIEWS Key points ■■ Important medical advances have allowed most patients with congenital or paediatric-onset cardiovascular conditions to reach adulthood ■■ Although some countries have a mandated age at which patients transfer from paediatric to adult care, guidelines recommend a flexible age of transfer of 18–21 years ■■ Many patients experience lapses of care during the transfer period; alarmingly, lapses are associated with a reduction in the quality of medical outcomes ■■ Adolescents and young adults should be educated about the importance of lifelong specialized cardiac follow-up care before, during, and after the transfer event ■■ Transition is an extended process, distinct from transfer, that begins by early adolescence and is focused on patient education and self-management skills ■■ The perspectives of patients, families, and care providers are important considerations in the development, adaptation, and individualization of transition programmes

the development of self-management skills together with emotional support. The transition process should begin early and be individually adapted to the physical, developmental, and psychosocial maturational stages and milestones of the patient throughout the process. Transition to adult care should not be focused solely on achieving continuity of medical care for the patient’s cardiac condition. Patients might have additional neuro­ developmental, cognitive, behavioural, mental health, and psychosocial issues. 11 These conditions could be the consequence of concomitant genetic or syndromic abnormalities, such as DiGeorge syndrome (also known as chromosome 22q11 microdeletion syndrome) or Down syndrome (also known as trisomy 21). Comorbid conditions can also be the neurodevelopmental and psychological consequences of earlier cardiovascular physiology or complications from its treatment, such as a brain injury associated with neonatal circulatory arrest, cardiopulmonary bypass, or embolic or ischaemic stroke. Comorbid noncardiac medical conditions un­related to the patient’s cardiovascular condition can also be present, including other congenital anomalies. Moreover, patients might have cardiac or noncardiac complications asso­ ciated with paediatric cardiovascular abnormalities. Prominent examples include patients with a single ven­ tricle who have undergone Fontan palliation and could, therefore, have ventricular dysfunction, valvar dysfunc­ tion, arrhythmia, thrombosis, protein-losing entero­pathy, plastic bronchitis, hepatic cirrhosis, and short stature as a result of their cardiac condition. Chronic medical conditions, such as diabetes mellitus, obesity, asthma, and hypothyroidism can also be present. The transfer and transition of the patient’s cardiac care should not occur in isolation, and consideration should be given to these other factors and conditions. An interdisciplinary and multidimensional approach to the transition process is optimal for these patients with complex health issues.

Effective transfer to adult care The challenge The large and increasing population of adults with con­ genital or other paediatric-onset cardiac con­ditions who require specialized care is placing demand on health-care systems for the development and sustained growth of 52  |  JANUARY 2014  |  VOLUME 11

appropriate resources, including personnel. To deter­ mine where these adult patients are being cared for, 228 paediatric cardiology programmes in the USA and Europe were surveyed regarding their practices for the transfer and transition of children with CHD.12 Of the 69 responding centres, 51 (74%) reported that they trans­ fer their patients to adult-focused care at a median age of 18 years. Notably, 41 of the 51 programmes (80%) who transferred patients did so to a formalized adult CHD programme, and common factors that prompted transfer included the presence of adult comorbidities, pregnancy, and patient or family requests to transfer from paediatric cardiology. However, not all paediatric cardio­logists transfer care to specialized adult CHD pro­ grammes. Young adults (mean age 25 years) with CHD of m­o derate-to-severe complexity who had an index outpatient cardiology visit to a hospital in the USA at 14–18 years of age were identified and contacted through hospital and public records and social networking methods.13 Of the 153 patients who could be contacted and who agreed to be interviewed, 125 (82%) had been seen by an adult-care cardiologist within the previous 2 years; the majority (69%) were still being cared for by a paediatric cardiologist.13 The care of adolescents and adults with CHD of m­o derate-to-severe complexity requires specialized expertise and systems, and often incorporates inpatient care. The systems in place to provide care to patients with CHD in childhood are more specialized at CHD care than those that provide care to patients with adult‑onset cardiovascular disease, and patients with adult CHD are often treated in hospitals without adult CHD pro­ grammes. The California hospital discharge database was queried for patient demographics, CHD complex­ ity, emergency department admissions, and insurance patterns.14 In the younger cohort (aged 12–20 years), 12 hospitals accounted for 70% of hospitalizations, whereas in the older cohort (aged 21–44 years), 25 hospitals accounted for 45% of cases.14 Multivariate regression analysis revealed that one of the significant predictors of inpatient admission via the emergency department was age >18 years.14 These data support the notion that CHD care in the USA is more dispersed for adult patients than for paediatric patients. This dispersion could contribute to lapses in care.

Lapses in care Lapses in specialized cardiology care often begin in the paediatric setting, several years before the planned age of transfer of care. The proportion of young adults (aged 19–21 years) with CHD who had at least one follow-up visit at a Canadian adult CHD centre has been investi­ gated.7 In a cohort of 360 patients, only 47% had received care in a specialized adult CHD centre.7 Although the primary aim of this study was to determine the percent­ age of patients who successfully transferred from paedi­ atric to adult care at 18 years of age, 22% of the cohort had not received care in a paediatric hospital after 10 years of age.7 In multivariate analyses, one of the factors sig­ nificantly associated with successful transfer (defined as



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REVIEWS receiving care in an adult CHD centre) was older age at the time of the patient’s most-recent paedi­atric cardio­ logy appointment. A provincial database from Quebec, Canada was queried to determine the proportion of children and young adults with CHD (diagnosed before 6 years of age) with subsequent documented outpatient cardiology visits.15 The proportion of patients who had not received any outpatient cardiology care after 6 years of age was 28%; 47% had not received cardiology care after 13 years of age, and 61% had not received cardiology care after 18 years of age. Even among those patients with severe CHD lesions, 21% had not received cardiology outpatient care after their 18th birthday.7 Therefore, the importance of life-long cardiac care must be discussed early and in the paediatric care setting. Although lapses in care are often present during child­ hood or adolescence, they occur most frequently at the time of transfer. In a 2013 survey study of almost 1,000 adults with CHD across 12 adult CHD centres, 42% of patients self-reported a >3-year lapse in care, and 8% reported a gap in care of >10 years; gaps occurred most frequently at 20 years of age.16 Concerns about lapses in CHD care are not unique to North America. In a Belgian study, at a centre where the paediatric and adult CHD programmes were physically located in the same building (thus facilitating the trans­ fer of patients and access to medical records), only 58 of 794 (7%) adults with CHD were not receiving follow-up care by a cardiologist.17 By contrast, in a study of over 10,500 German patients, 8,028 (76%) were not receiving follow-up care at specialized adult CHD centre during a 5‑year period.18

Clinical impact of lapses in care Lapses in cardiac care are of great clinical importance, owing to their association with poorer medical out­ comes than among those who receive continuous care. Substantial rates of nonadherence to medi­cation regi­ mens and lack of attendance at clinical follow-up ses­ sions have been observed among adolescents and young adults transferring from paediatric to adult health care. 19–21 Poor adherence to medication regimens is common among adolescents who have undergone heart transplantation, and has been attributed to incompletely mature cognitive skills, particularly abstract thinking.22 Not surprisingly, in a retrospective analysis of 168,809 patients who underwent renal transplantation, patients aged 14–16 years had the greatest risk of kidney allograft failure.23 For patients who have had organ transplant­ ation, including cardiac transplantation, comprehensive programmes are required to reduce graft loss during the transition from paediatric to adult care. In a retrospective study of patients with coarctation of the aorta who were referred to a newly established adult CHD clinic, over half of the patients had lapses in care and 41% had clinically relevant hypertension, although few received appropriate antihypertensive medication.24 A front-page article in a Danish newspaper about adults with CHD who had been “lost for follow-up” prompted 147 adults to contact a centre for congenital cardiac

care.25 Those patients who responded to the newspaper article underwent clinical examination and echocardio­ graphy, and 52 (35%) were scheduled for further followup care because of important residual lesions or a high risk of late complications. Among 158 adults with CHD of moderate or severe complexity, 99 (63%) had a lapse in care of 2–50 years (median of 10 years).26 One-third of patients with a lapse in care recalled being told that follow-up care was not needed.26 In multivariate regression analysis, a lapse in care was significantly associated with receiving a new diagnosis of haemodynamic importance (P 5 years previously.34 Qualitative research provides insight into the expect­ ations and experiences of adolescents transferring from paediatric to adult cardiac care. Although adolescents with CHD are aware that they are leaving a familiar setting, they tend to view this change as normal and have a ‘wait and see’ approach to the adult CHD programme.35 However, many patients with CHD lack knowledge about their condition, the implications of transition on their cardiac care, and what to expect at their first adult cardio­logy outpatient clinic visit.27,35 When asked about their expectations for adult care, adolescents who under­ went paediatric heart transplantation anticipated a busier and more serious environment.36 Following transfer to 54  |  JANUARY 2014  |  VOLUME 11

adult care, patients who underwent cardiac transplant­ ation during childhood tended to compare paediatric and adult centres, with the latter being characterized as ‘less fun’. These patients also seemed to miss the personal relationships they had developed over time with their paediatric health-care providers.37 The process of transition does not necessarily end in the paediatric setting, and ideally continues after transfer to adult care. ‘Emerging adulthood’ is used to describe individuals aged 18–25 years who sense that they have left adolescence, but also that they have not yet reached adulthood.38 Accepting responsibility and making deci­ sions are two key features of reaching full adulthood.38 Emerging adults in the transition process can be emotion­ ally challenged by separation from paediatric health-care providers and the directly supportive environment that had assumed full responsibility for the management of their cardiac health. These patients can be unprepared for the change in communication style between the pae­ diatric and adult settings, and the increased expectations for knowledge, self-management, and self-advocacy. Some patients, therefore, experience a ‘cultural chiasm’.39 Anxiety, psychosocial, and cognitive issues can provide additional challenges.40 In the adult setting, patients might also receive more-direct information regarding expectations and their long-term prognosis than they did in the paediatric setting. During this time, patients might begin to perceive clearly the implications of their cardiac condition for social, educational, and vocational goals, and understand their ability to access appropriate health care.41 They might also be concerned about s­tigmatization and discrimination by employers and peers.42 Given these perspectives, transition needs for ado­ lescents and young adults must address the develop­ ment of knowledge and skills required for self-care and



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REVIEWS self-advocacy. Parents and health-care providers should be astute, and therefore elicit continuous feedback from the patient during this process, and provide ongoing support to foster independence. Psychosocial chal­ lenges should be appropriately assessed and addressed.43 Novel strategies that particularly appeal to adolescents and young adults should be applied. These include peer support groups and the use of media platforms for ed­ucation, communication, and self-management.29

Parent or guardian perspectives Preparation for transition can be influenced by the per­ spectives of the patient’s parents or guardians. Lack of knowledge about their child’s cardiac condition has been associated with a similar lack of knowledge on the part of the patient, and is associated with a low level of parental education.44 Although most parents of a child with CHD recognize that their child will need life-long cardio­logy care, many do not realize that during adulthood their child should receive their cardiology care from an adult CHD specialist.45 A qualitative study showed that 41% of parents were concerned about their child leaving their paediatric cardiologist, and only 45% indicated that their child would be ready to take complete responsibil­ ity for their health care at 18 years of age.27 Anxiety exists among parents of adolescents who underwent paediatric heart transplantation, and is often related to lower expec­ tations of the quality of care provided in the adult setting than in the paediatric setting.36 Parents can be active participants in, as well as advo­ cates for, their child’s transition, particularly if they are involved early in the process at an appropriate level of participation. However, parents and health-care pro­ viders can have different opinions on their relative responsibilities for preparing the patient during transi­ tion.27,46 Parents might have difficulties allowing their child to assume increasing responsibility for their own health care. Parental overinvolvement can prevent the patient from acquiring knowledge, independence, and self-advocac­y skills during the process of transition. Perceived parental overprotection can contribute to long-term, heart-related anxiety that is not necessar­ ily correlated with complexity of the patient’s cardiac condition.47 Parental education on the rationale behind transition strategies (for example, encouraging children to speak independently with health-care providers) can result in greater parental acceptance of these strategies.48 Care provider perspectives Paediatric cardiology Paediatric cardiologists and nurses have shepherded their patients and families through the trials and tribulations of presentation, diagnosis, and management into longterm follow-up care. Hence, they often have an emo­ tional attachment to their patients and a desire to shelter them from the uncertainties of their long-term progno­ sis; they can, therefore, convey unrealistic optimism.49 Paediatric-care providers often conceptualize functional health status and health-related quality of life differently than patients and their families. Health-care providers

seem to be more likely than patients and families to focus on the in-hospital psychosocial implications of CHD.50 Health-care providers can also have different views on their patient’s readiness for transition than patients and their families and might have limited confidence in the expertise of adult cardiologists to care for these complex patients.27,49 Academic and financial incentives for paediatric cardiologists to continue to care for their adult patients can also be present, or they might do this out of necessity when appropriate adult-care providers are not available.51 Indeed, their concerns are not unfounded as the number of adult CHD care programmes is insuf­ ficient to meet current demands.52,53 Perceived practice barriers can also impede transition, including time con­ straints during clinic appointments that preclude educa­ tion and discussions of transition, lack of structure and processes for managing transition, and limited knowledge of the differences in practice structure and approach of the adult-care providers relative to themselves. Paediatriccare providers might be focused on transfer as the ter­ mination of their responsibilities to the patients and, therefore, do not participate fully in the continuous process of transition. In conjunction, an important role exists for nursing and allied health-care professionals to address some of these barriers to the i­nitiation and ­negotiation of effective transition.54,55 Adult cardiology Demand exceeds supply for specialist cardiology care for adult patients with paediatric-onset heart disease, particularly CHD. Although consensus exists regard­ ing which patients require specialist care, and appropri­ ate care algorithms and recommended best practices for transition are present,10,56–58 manpower and access remain primary concerns.52 Identifying these patients and transferring them to an appropriate destination can be challenging. Adult patients with CHD of moderateto-severe complexity should be cared for by cardiolo­ gists specializing in adult CHD. The first concern of the adult-­cardiology-care provider is the timely and accu­ rate receipt of the patient’s full medical history, includ­ ing advance notification of any psychosocial issues and any immediate care requirements. However, adult-care cardio­l ogists should not be merely the receiver of a hand-off; they should be involved in transition before the actual transfer event takes place. For the trans­itioning adolescent and emerging adult, the adult-care environ­ ment and approach can seem overwhelmingly businesslike and intimidating. Adult-care cardiologists often expect these young patients to be knowledgeable and prepared to assume responsibility, and can express frus­ tration when they are not. During trans­ition, responsibil­ ity must be shared between all stakeholders in a manner that might not be necessary when dealing directly with adult patients. Adult-care providers might not have the skills or experience to adapt their communication style to the developmental stage of the transitioning patient, or to the presence of a concerned parent. They might not recognize or be prepared to fully co-ordinate the care of associated medical and psychosocial comorbidities.

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REVIEWS In a survey of internal medicine providers, improved training in congenital and childhood-onset medical conditions was needed for i­nternists, as were more adult subspecialists.59

The broader health-care system Many challenges exist in establishing an ideal transition programme in the current health-care environment.8 For patients with complex disease, insurance and finan­ cial difficulties are among the most-common reasons for gaps in cardiology care. 16 In the USA, between 2001 (when participants were 14–17 years of age) and 2007, a 7.7-fold increase occurred in the proportion of people aged 19–23 years without health insurance. 60 Furthermore, this reduction in health-care coverage was associated with declined health status.60 In a Californian study of patients aged 21–24 years who had graduated from a public programme for youth with special healthcare needs, 40% had a lapse in insurance coverage after turning 21 years of age.61 Not surprisingly, insurance cov­ erage impacts health-care utilization for young adults.21 Financial barriers are a common cause of non­adherence during the transition period.19 American health-care reform legislation will hopefully reduce insurance bar­ riers, thereby improving the quality of and access to medical care.

Best practices for transition Guidelines for transition to adult care In 2002, the American Academy of Pediatrics (AAP), the American Academy of Family Physicians (ACFP), and the American College of Physicians (ACP) pub­ lished a consensus statement in which they specified the importance of a thoughtful transition of patients with special health-care needs into adult care.8 A decade later, minimal progress in transition outcomes has been docu­ mented.62 In 2011, the AAP, ACFP, and ACP published a clinical report describing practice-based guidelines for transition to adult care.9 In the report, the goal of trans­ ition is to ensure that “high-quality, develop­mentally appropriate health care services are available in an u­ninterrupted manner as the person moves from ado­ lescence to adulthood”. A key component in their model is the medical home that provides preventive care, acute illness management, and chronic condition management; a co-management approach is advocated in which the roles and responsibilities of primary-care and specialtycare providers are clearly defined. Position statements have also been provided by the Society for Adolescent Medicine and the Canadian Paediatric Society.63,64 Both documents emphasize that transitional care must be tailored to the chronological age and d­e velopmental a­bilities of individual patients. Joint guidelines from the ACC and the AHA for the care of adults with CHD recommend the development and implementation of a formal transition process aimed to prepare teenagers and young adults with CHD to become increasingly responsible for their health care.57 In 2011, the AHA published a scientific statement on best practices in managing transition for adolescents with 56  |  JANUARY 2014  |  VOLUME 11

CHD.10 This document is a comprehensive summary of the medical surveillance, lifestyle, and psychosocial needs of adolescents and young adults with CHD, with a view that patients with moderate or complex CHD be managed by cardiologists specialized in adult CHD. Of note, as a reflection of the limited evidence base regard­ ing transition, most recommendations are graded Class I (benefits outweigh the risks and the intervention should be performed), but Level of Evidence C (based upon c­onsensus opinion, case studies, or standard of care).

Structure of a transition programme The development of an effective transition prgramme relies on confirmed institutional support. 65,66 Trans­ formational transition leadership is a key factor in fos­ tering a collaborative process and providing mentorship through modelling optimal behaviours.67 A transition co-ordinator or ‘champion’, who is both an educator and a point person for patients, families, and health-care providers, is often recommended and has proven to be effective.67,68 For example, a relatively simple interven­ tion consisting of a transition co-ordinator (who sent appointment reminders and assisted with appointment rebooking) together with an after-hours phone service for patients aged 15–25 years with type 1 diabetes reduced both blood glucose levels and hospital admis­ sions.63 Importantly, savings accrued by the reduction in acute-care bed days offset the cost of the programme.63 A structured, collaborative, and comprehensive approach is ideal for the transition process. Adolescents and their parents also hope for strong collaboration between paediatric and adult providers.36 The Toronto Transition Task Force is one such collaborative approach, in which paediatric and adult CHD health-care providers collaborate on strategies focused on patient and family education, preparation for transfer from paediatric to adult care at the mandated age of 18 years, and a coordinated transfer of care.70 Some useful resources and descriptions of well-developed programmes can help guide the transition process (Box 1). Meeting educational needs In a systematic review of the effectiveness of 10 transi­ tion care programmes, patient education was one of the most-common components of successful initiatives.71 For example, a two-session educational intervention (focused on the importance of medication adherence, clinical appointments, diet, and the transfer of medi­ cation responsibilities from caregivers) given to youths who had undergone liver transplantation improved adherence and liver function at follow-up assessments.72 Few American and European paediatric cardiology programmes that transfer patients to adult CHD care provide any structured preparation. 12 Furthermore, although many paediatric cardiologists report that they educate transitioning patients, a minority use formal tools such as surveys or checklists. 51 Although the process of transition is recommended to begin during early adolescence, younger children, depending on their developmental capabilities, can be taught the name of



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REVIEWS Box 1 | Annotated web bibliography of resources for patient transition AAP National Center for Medical Home Implementation (USA) ■■ http://www.medicalhomeinfo.org/how/care_delivery/transitions.aspx ■■ Provides general and practical information about the medical home approach to care ■■ Includes the AAP Clinical Report on transition as well as links to several key transition publications Graduate Certificate in Education and Health Care Transition (USA) ■■ http://education.ufl.edu/education-healthcare-transition/ ■■ This University of Florida programme was developed for graduate students and health professionals in all areas of practice ■■ Participants receive a certificate after completing four online courses (12 credit hours) over the course of 1 year Health Care Transition Research Consortium (USA) ■■ https://sites.google.com/site/healthcaretransition/ ■■ An organization focused on the advancement of evidence-based research on health-care transition and self-management ■■ Includes information about the annual spring meeting and annual fall conference Health Care Transition Initiative at the University of Florida (USA) ■■ http://hctransitions.ichp.ufl.edu/ ■■ Offers several products (for example, research reports, planning guides, informational booklets and videos, and online training programmes) targeting adolescents, young adults, families and health-care professionals ■■ Includes a link to a recommended website (Talking with Your Doctor and Other Healthcare Professionals) which offers valuable strategies and a video The Hospital for Sick Children Good 2 Go Transition Program (Canada) ■■ http://www.sickkids.ca/good2go/ ■■ Good 2 Go is a hospital-wide programme focused on education and staff support, resource development, and evaluation ■■ Offers transition interventions, tools, and resources with the goal of preparing youth with chronic health conditions to transfer from paediatric care to adult care (at the age of 18) with the requisite knowledge and skills National Health Care Transition Center (USA) ■■ http://www.gottransition.org/ ■■ Disseminates and facilitates the implementation of best practices in transition, particularly for young people with special health care needs ■■ Provides information on the ‘six core elements of health care transition’, which are currently being implemented and evaluated ■■ Offers information for youths, families, providers, and US states On TRAC (Transitioning Responsibly to Adult Care) (Canada) ■■ http://ontracbc.ca/ ■■ Province-wide multifaceted transition initiative developed in British Columbia, Canada ■■ Targets youth and young adults (aged 12–24 years) with chronic health conditions and/or disabilities ■■ Offers different ‘toolkits’ for youth/young adults, parents/families, and health‑care providers Royal Children’s Hospital Melbourne (Australia) ■■ http://www.rch.org.au/transition/ ■■ Includes separate sections for young people, parents and providers ■■ Offers downloadable fact sheets and checklists Scientific Statement from the American Heart Association (USA) ■■ http://circ.ahajournals.org/content/123/13/1454.full.pdf ■■ Provides a link to the Scientific Statement published in 2011: ‘Best practices in managing transition to adulthood for adolescents with congenital heart disease: the transition process and medical and psychosocial issues’ University of Florida Jacksonville Health and Transition Services (USA) ■■ http://hscj.ufl.edu/jaxhats/ ■■ Provides an example of a Medical Home (the JaxHATS clinic) for adolescents and young adults with chronic medical or developmental problems aged 16–26 years University of Washington Adolescent Health Transition Project (USA) ■■ http://depts.washington.edu/healthtr/ ■■ Offers information about the transition process and transition resources for patients, their families, schools, and health providers Abbreviation: AAP, American Academy of Pediatrics.

their cardiac condition and be directly asked about their cardiac symptoms (Box 2). Education can be provided in both outpatient and inpatient settings and in multiple formats, including verbal, written, and online content, as well as formal patient and family education events. The incorporation of education ‘checklists’ into the patients’ paper or electronic medical records would ensure that an educational curriculum is provided and tracked across the extended transition period. Participation in a struc­ tured education programme is associated with greater knowledge of their condition among adolescents and adults with CHD.73

Fostering self-management skills Providing information alone is not sufficient for patients transitioning from paediatric to adult cardiac care; pro­ viding active skills training is of equal importance. Selfmanagement refers to “active participation of the patients in the everyday care of the symptoms of their illness(es) and medical treatments, as well as maintaining general health and prevention of progression of medical condi­ tions”.74 Certain self-management skills should be opti­ mally achieved at the completion of the transition process by all patients with the appropriate cognitive and devel­ opmental abilities to do so (Box 3). Various strat­egies are available to foster self-managemen­t skills in adolescents with cardiac conditions. One relatively simple way to foster self-management is to speak with patients alone, in the absence of their parents, for a portion of every clinic visit beginning in adolescence.63,65 This approach gives young patients the opportunity to learn how to ask and answer questions from their care provider, develop self-advocacy skills, and become comfortable discussing sensitive issues. Preparing verbal three-sentenc­e sum­ maries in advance of medical appointments can also help patients to develop self-managemen­t skills; parents can help their children prepare to communicate these summaries to their health-care providers.75 These sum­ maries should outline three components: age, diagnosis, and brief medical history; current treatment plan; and questions or concerns to be discussed during the medical appointment.75 Assisting with the creation of a portable health summary, such as a health ‘passport’ that can be carried in their wallet, can also empower patients. The summary helps patients communicate their medical needs with new providers or in case of medical emer­ gency.8 Incorporating these kinds of exercises into an educational intervention improves patients’ knowledge of their condition and their care.76 Optimizing the timing of transfer Recommendations on the optimal timing of trans­ fer depend on whether one is referring to the entire trans­ition process or the transfer event. Educating and empowering patients should begin early in the transition process (Figure 2). Current recommendations suggest that education should ideally be started by 12 years of age.9,10 Envisioning a future is important for adolescents with chronic health conditions and for their parents.77 The transfer event is best made during a period of clinical

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REVIEWS Box 2 | Components of comprehensive patient education Following a comprehensive patient education programme, the patient should know: ■■ Name and description of his or her cardiac condition ■■ Names of any previous cardiac interventions (for example, surgeries, catheter interventions, device implantation, transplantation) and the date or age at which they occurred ■■ Name, dose, and purpose of all medications ■■ Endocarditis prophylaxis (if needed) ■■ Specific clinical symptoms that would warrant immediate medical attention ■■ Physical activity recommendations ■■ Unique considerations related to tobacco, alcohol, and substance use (if any) ■■ Unique considerations for educational or vocational decisions (if any) ■■ Unique considerations for contraception, pregnancy, and recurrence in offspring (if any) ■■ Long-term health expectations including the preparation of advance-care planning documents (if appropriate) ■■ Importance of life-long cardiac care

Box 3 | Self-management skills for transitioning patients Self-managing patients will: ■■ Be able to contact health providers ■■ Know when and how to access emergency health care ■■ Create and use a portable health summary ■■ Schedule and attend clinic appointments ■■ Adhere to a medication regimen ■■ Request refills of prescriptions ■■ Maintain health records ■■ Communicate with health-care providers independent from parents or guardians (for example, when discussing health history or current symptoms) ■■ Understand when and how to access mental health services

Transfer Clinical guidelines recommend a flexible age of transfer, ideally 18–21 years of age.

Childhood In childhood (even at birth) parents can be prepared for their children with cardiac conditions to become adults with cardiac conditions.

Birth

25 years

Adolescence Beginning by 12–13 years (as developmentally appropriate), young people should be helped to gradually develop the knowledge and selfmanagement skills to eventually assume responsibility for their helath-care management.

Emerging adulthood Transition rarely ends after transfer to adult-focused care. At 18–25 years of age, knowledge and self-management will continue to develop. A focus on responsibility and decision-making becomes prominent.

Figure 2 | Suggested timeline for transition preparation. The transition from paediatric to adult care should be a process that occurs over a number of years. Preparation for the transfer event can begin in childhood. The transfer event, which ideally occurs between 18 and 21 years of age, should be followed by support for the emerging adult patient.

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stability. If this strategy is not achievable, extra planning and preparation for transfer must ensure that the receiv­ ing adult-care providers and care facility have the capac­ ity and awareness to address and manage immediate and ongoing complex concerns, including psycho­ social issues. Checks and balances should be in place to track the transfer. In such circumstances, a period of co‑managemen­t could be required. Recommendations regarding the optimal timing of the transfer event are variable. Clinical guidelines from the AAP, ACFP, and ACP recommend a flexible age of transfer, ideally between 18 and 21 years of age.9 The timing of transfer of patients with other chronic medical conditions, such as cystic fibrosis and type 1 diabetes, typically falls within this age range.21,78 In a hospital-based transition protocol for adolescents with HIV infection, however, the optimal age for transfer from adolescent to adult care was advocated to be 25 years, owing to high failure rates when the age of transfer was 21 years.79 Of 69 paediatric cardiology programmes in the USA and Europe that participated in a survey, 51 (74%) reported that they transfer patients to adult-focused care at a median age of 18 years.12 In some countries, including the USA, no mandatory age of transfer exists; therefore, some patients continue to receive care within paediatric cardiology programmes for their entire lives. In other countries, however, the age of transfer is man­ dated, usually 16–18 years of age. The advantage of this approach is that patients, parents, and health-care pro­ viders know when to expect transfer of care. The dis­ advantage is that some patients might be ready earlier than this cut-off, whereas others might benefit from additional time in the paediatric setting. Some cardiolo­ gists suggest that “exclusion from paediatric cardiology at an arbitrary age may expose patients to delayed pres­ entation and adverse outcomes that are likely associated with p­rolonged lapses in care”.13 According to a survey of 51 programmes in the USA and Europe, transfer to adult care was governmentally mandated for 51% of programmes, and directed by hospital policy for 27%.12 Nonetheless, many paediatric cardiologists are opposed to setting policies for the age at which patients are t­ransferred from paediatric to adult care.51 Within environments that support a flexible approach to the timing of transfer, an assessment of transition readiness is often recommended to guide the timing of transfer for an individual patient.10 However, in a sys­ tematic review of transition readiness and transfer sat­ isfaction measures, no measures with well-established reliability and validity could be identified.80 Without a well-validated measure of readiness for transfer, r­eadiness will likely remain a matter of clinical judgment.

Optimizing the transfer event Regardless of the timing of transfer, a written trans­ ition plan, prepared in early adolescence, can help prepare patients and their families. 8 Unfortunately, however, such plans are uncommon. The 2007 Survey of Adult Transition and Health included 1,865 young adult respondents aged 19–23 years, of whom only 55%



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REVIEWS Box 4 | Markers of a successful transition Patient ■■ Receives comprehensive education (Box 2) ■■ Develops self-management skills (Box 3) ■■ Creates a portable medical summary ■■ Is prepared for transfer in advance (for example, has participated in the development of a written transition plan) ■■ Receives a transfer summary ■■ Is transferred to a qualified adult care team Parents or guardians ■■ Are provided with education regarding the rationale behind transition (and specific transition strategies) ■■ Are prepared in advance for the transfer of care ■■ Develop confidence in the adult care team Paediatric programme ■■ Initiates the transition process by the time the patient is 12 years of age ■■ Initiates the transfer process at a time appropriate to the patient’s age and developmental stage ■■ Has a designated transition champion ■■ Has strategies to track patients and evaluate transition outcomes (for example, a registry) ■■ Has an explicit policy on transfer of care that is known to all stakeholders ■■ Works within the medical home model ■■ Provides patient education and self-management skills training in multiple settings (both inpatient and outpatient) and modalities (for example, in person, in group settings, with written or online materials) ■■ Ensures care providers speak independently with patients for at least part of the clinic visit ■■ Provides a comprehensive transfer letter to adult providers Adult programme ■■ Has a designated transition champion ■■ Has strategies to track patients (for example, a registry) and evaluate transition outcomes ■■ Receives comprehensive transfer letters from paediatric providers ■■ Maintains ongoing consultation with paediatric providers as needed Health-care system ■■ Has an adequate number of trained and qualified adult providers ■■ Provides affordable health insurance

recalled that their health-care providers had discussed how their needs would change as they grew older.81 Planning for transfer of care should be a routine part of adolescent cardiac care and should not be introduced at the final paediatric appointment. An explicit policy in which the programme’s approach to transition is made known to patients, families and health-care pro­ viders should be in place. 9 For example, posted signs in clinic rooms in a hospital in Toronto (ON, Canada) read, “At SickKids we prepare our patients to graduate to adult care. Don’t wait until you are 18 to start plan­ ning to leave. Talk with your health-care provider”. An official trans­ition clinic, which focuses on education, self-management skills, and preparation for transfer, is a common element of successful transition programmes.71 The Copenhagen Transition Program is a formal out­ patient nursing clinic for adolescents with CHD that was established in 2004, and is a good example of a trans­ ition clinic. 69 This programme is focused on patient education—patients are mailed appointment letters, 1 h appointments are scheduled in clinics that are held 2 days per week, and the visit is documented in the h­ospital-wide medical records system. Adolescents are

seen individually by nurses, while their parents remain in the waiting room. Many patients and their families wish to meet their adult-care providers and visit the adult clinic before the transition event.37 Joint clinics run by both those who provide paediatric care and those who provide adult care are ideal, although the logistic feasibility of such clinics depends at least partially upon the number of patients transferring each year. For this reason, joint clinics might be more feasible for heart transplant­ation teams than for CHD teams. Other ways to introduce the adult-care team exist if joint clinics are not feasible. Formal transition events (transition evenings, for example) that are attended by adult-care providers and include a tour of the adult clinic could be offered.70 Transfer letters or brochures for the patients could be provided at the final paediatric clinic appointment or following receipt of the transfer documents at the adult programme.70 This material can include important contact information, an introduction to the adult-care team (including photographs), and a d­iscussion of the importance of life-long cardiac care. Medical records are also transferred from pae­diatric to adult programmes. Following the final paedi­atric clinic appointment, the transfer packet sent to the adult clinic should include all the information necessary to provide optimal continuity of care.70 The referral letter should include pertinent information including diag­ noses (including noncardiac conditions and complica­ tions), surgical and interventional procedures, past and anticipated complications, psychosocial and develop­ mental concerns, and medication history. Procedural reports, inpatient discharge summaries, and the mostrecent diagnostic test results should also be included. Paediatric-care providers are also encouraged to make recommendations about the timing of the first adult clinic visit and the testing that will be required at that visit. Additional information should accompany the transfer of patients with important cognitive deficits (for example, the patient’s communication abilities and whether general anaesthesia is required for diagnostic testing). The transfer letter should not be considered the sole communication event, as adult providers might seek further consultation with paediatric-care providers as they are the most familiar with a patient’s medical history.

Tracking transition and transfer outcomes Tracking and evaluation of outcomes is an important component of a well-structured transition and transfer process. Patients can experience loss to follow-up and lapses of care at any point in the transition process, and these lapses must be monitored and minimized. The identification and commitment of a designated trans­ition champion or partner among the health-care team can facilitate the tracking of milestones and the maintenance of timelines and checklists, ensure patient and family satisfaction, and troubleshoot challenges for all stakeholders (Box 4). Maintaining a registry that actively tracks transitioning youths (in the paediatric setting) and young adults (in the adult-care setting) is encouraged in order to recognize lapses in care as soon

NATURE REVIEWS | CARDIOLOGY

VOLUME 11  |  JANUARY 2014  |  59 © 2014 Macmillan Publishers Limited. All rights reserved

REVIEWS Box 5 | Future research directions ■■ Develop and validate a transition readiness measure suitable for both clinical and research use ■■ Understand the transitional needs of young patients with noncongenital paediatric-onset heart disease (for example, arrhythmias, cardiomyopathies) ■■ Understand the unique transitional needs of young patients with heart disease who also have relevant genetic or syndromic abnormalities (particularly those with cognitive or developmental disabilities that preclude the adoption of some or all self‑management skills) ■■ Improve our understanding of factors associated with lapses or continuity of cardiac care through longitudinal research ■■ Evaluate transition interventions using three outcomes: acceptance by all stakeholders, health outcomes, and cost-effectiveness

as they occur.9 Ongoing involvement and engagement of a stakeholder group including patients, families, and care providers can facilitate continual improvement in the quality of the transition programme. Identification of successful strategies and components should lead to evaluation and dissemination to other programmes. Many aspects of transition are not specific to patients with paediatric-onset cardiac disease, and forums for sharing best practices across specialties and institutions should be increased and expanded.

Conclusions The large and growing population of adolescent and adult survivors of congenital and paediatric-onset cardio­vascular conditions requires specialized adult care. To minimize lapses in care and maximize shortterm and long-term medical and psychosocial outcomes, a comprehensive process of transition from paediatric

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to adult care is required. Transition, with an emphasis on patient education and self-management skills, is an extended process that begins by early adolescence and recognizes factors important to families, care providers, and the health-care system (Box 5). Although several sets of guidelines and position papers are unanimous in highlighting the importance of the provision of thoughtful transitional care, few studies have directly evaluated the effectiveness of specific transition interventions. The evidence base to support recom­ mendations for optimal practices must be improved. As previously noted, almost all recommendations regarding transition are based on consensus opinion, case studies, or standards of care. Transition interventions should be developed and empirically evaluated to determine their rates of success (including measures of medical and psychosocial outcomes) and cost-effectiveness, which will establish whether they warrant widespread implementation. Research on the optimal timing of transfer is currently challenged by the absence of a wellvalidated measure of transition readiness for use with cardiac patients. Future research (Box 5) should aim to provide optimal t­ransitional care to all young people with heart disease. Review criteria The evidence review was conducted using PubMed for English language articles, and search criteria included “transition to adult care” and “transfer to adult care”, cross-referenced with “congenital heart disease” and “paediatric heart disease” for publication years 1990– 2013. Additional articles of interest were identified from related citations, and review of references cited in full-text articles retrieved. Relevant articles regarding aspects and perspectives of transition to adult care for noncardiac chronic conditions were also identified.

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