For the first time in history, there are now more adults than children living with congenital heart disease (CHD) in North America. Adults with CHD of moderate or great complexity, however, remain at significant risk of heart failure, arrhythmias, additional surgeries and interventional procedures, and premature mortality. This population thus requires specialized CHD care during childhood, adolescence, and adulthood. There are known challenges in retaining these patients in specialized care and ensuring that they have the knowledge and skills to assume responsibility for the management of a lifelong medical condition. For this reason, a focus on the transition from pediatric to adult care has become prominent for CHD providers and programs.
Despite widespread recognition of the importance of a purposeful and planned approach to transition, progress has unfortunately been slow. Thus outlining a series of mandatory components of a transition program to be adopted by every pediatric center is unlikely to be of benefit. Instead, to facilitate implementation, recommendations must be adaptable to the unique needs and resources of individual programs. This chapter begins with a review of the literature regarding two indicators of a successful transition, namely (1) continuity of optimal health care across the life span, and (2) patients having the “knowledge and self-management skills to assume maximal responsibility of their health care management and live as full and independent lives as possible.” The chapter concludes with guiding strategies for pediatric and adult programs that wish to develop or advance their transition services.
Continuity of Care
Individuals with CHD of moderate or great complexity require uninterrupted specialized cardiac care throughout their lives. Guidelines recommend a flexible age of transfer between pediatric and adult programs, ideally between the ages of 18 and 21 years. This flexibility allows for tailoring to the developmental, maturational, and physical considerations of each patient. Although this approach entails an assessment of an individual patient’s preparedness for transfer, it remains unknown how to practically use information obtained from transition readiness measures. Fortunately, qualitative research has revealed that many adolescents with CHD view leaving pediatric care as normal and maintain a wait-and-see approach to adult care.
Although the importance of continuity of care is apparent to CHD pediatric and adult providers, lapses of care are unfortunately common. An American multisite study of approximately 1000 adults with CHD revealed that more than 40% of patients had a lapse of 3 years or longer and that gaps were most common at the age of 20 years. Thus the time of transfer appears particularly vulnerable to lapses in care. However, two Canadian studies demonstrated that lapses frequently occur prior to planned transfer, in children and adolescents. A proactive approach is required, such that the message of the importance of lifelong care should be expressed to patients and families beginning early in the pediatric setting. There should also be mechanisms to track patients in pediatric and adult care settings to allow for the prompt identification of patients with gaps in care.
American and European pediatric cardiology programs were surveyed regarding practices for the transfer and transition of patients with CHD. Fifty-one of 69 responding centers (74%) indicated that they transfer patients to adult care; transfer was most commonly triggered by the presence of adult comorbidities, pregnancy, and requests by patients or families. Other research suggests that many young adults with CHD continue to be followed in the pediatric setting. It is known that there are too few adult CHD (ACHD) programs to take care of all adults with CHD. Data from the California hospital discharge database revealed that 12 hospitals accounted for 70% of hospitalizations for younger patients (12 to 20 years), but 25 hospitals accounted for only 45% of hospitalizations for older patients (21 to 44 years). Thus the care of adults with CHD appears much more dispersed than that provided in the pediatric setting.
A series of studies confirm the importance of uninterrupted CHD care and the risks of suboptimal care when lapses unfortunately occur. In an American study, almost two-thirds of adults with CHD had a lapse in care with a median duration of 10 years; lapses were associated with cardiovascular symptoms on presentation and the need for urgent cardiac intervention. In a sample of British adult patients with coarctation of the aorta, over half had experienced a lapse in care, and although 41% had significant hypertension, few were taking medications for this. Another British study indicated that approximately one-quarter of patients with repaired tetralogy of Fallot were not registered with ACHD clinics. Patients in this study who were not receiving specialized ACHD care had not undergone pulmonary valve replacement, although the researchers anticipated that one-third would have done so had they been retained in specialized care. In a Danish study of adults with CHD who presented for cardiology care following a lapse, approximately one-third were noted to have significant residual lesions or be at high risk of late complications. The following factors have been deemed protective against lapses in care: worse health status, patient attendance at pediatric clinic appointments without parents, the patient belief that specialized care was necessary, and referral to an ACHD center. These results highlight the importance of a proactive approach to transition in which the importance of lifelong care is emphasized and a planned approach to transfer of care takes place.
Maximal Responsibility of Health Care Management
In addition to continuity of care, the second major aim of transition is to support patients in obtaining the knowledge and self-management skills so that they can assume increasing responsibility for their health care management. As health care oversight gradually shifts from parents to patients, the ultimate goal is for patients to live as independent and rich lives as possible.
Ideally, a process of transition is initiated by the age of 12 years. A formalized approach to patient education and the acquisition of self-management skills is advised. A comprehensive educational curriculum includes information related to CHD and treatment (eg, the diagnosis; information related to previous cardiac interventions, medications, and clinical symptoms that warrant immediate attention; the importance of long-term specialized health care) as well as broader lifestyle implications (eg, physical activity, substance use, vocational and educational considerations, family planning, mental health, etc.). The use of checklists and documentation in the paper or electronic medical record can ensure that important educational content is reviewed. Although concerns about clinical time and resources are common, positive outcomes have been demonstrated following a brief 1-hour nurse-led transition-focused session.
It is not sufficient for adolescents and young adults to obtain knowledge to provide descriptions of their CHD and medical and lifestyle considerations. They should also learn behaviors that will help them assume responsibility for their health care management. Important self-management skills include being able to contact and communicate with health care providers, schedule medical appointments, maintain health records, and understand when and how to access emergency health care and mental health services. A collaborative approach is important so that parents, providers, and even administrative staff support the development and practice of self-management skills. For example, capable adolescent patients can be expected to speak with their provider on their own for a portion of every clinic visit. Appointment booking staff can encourage patients (rather than parents) to telephone to schedule or reschedule appointments.
Patients vary significantly in their developmental, maturational, and physical abilities, and these abilities must be considered when providing transition services. Some patients will be able to assume knowledge and self-management behaviors years earlier than others, and there are some patients who will never be able to assume responsibility for their health care management. Individuals with CHD are at elevated risk of neurodevelopmental deficits, the likelihood and severity of which increase with CHD complexity. We must begin to explore ways to most effectively meet the transitional needs of these patients and their caregivers.
Parents are major stakeholders in the process of transition because they are being asked to gradually relinquish control to their adolescent and young adult children. Many parents are unaware of the importance of specialized CHD care in the adult setting. Fewer than half think that that their children would be prepared to assume responsibility for their health care management at the age of 18 years. Although parental overinvolvement may hinder the education and development of self-management skills of adolescent patients, when engaged early and respectfully, parents have the potential to be strong advocates for their child’s transition.
Guidance for Providers and Programs
Slow progress in the area of transition is likely a reflection of low or inconsistent programmatic commitment to the goals of transition and practical matters related to limited resources and competing demands on providers’ time. The establishment and maintenance of a comprehensive transition program depends upon institutional support and leadership. Transition coordinators (also known as “champions”) are important, although the goals of transition cannot be accomplished by one individual within a program; the collective responsibility is shared by pediatric and adult health care teams. Further, the perspectives and roles of all key stakeholders (patients, parents/guardians, and providers) must be addressed ( Fig. 21.1 ). Although pediatric and adult programs share the goals and accountability of transition, there remain unique responsibilities, as summarized in Boxes 21.1 and 21.2 .
