The 2008 ACHD guidelines published by the American College of Cardiology (ACC) and the American Heart Associated (AHA) recognized the importance of transition with class I recommendations, stating a goal to “transition and ultimately transfer the patient into adult care settings (9).” Notably, certain countries or healthcare systems may mandate transfer of care at a certain age. Apart from these requirements, transfer is still recommended for several reasons, related to the physical and mental growth that occurs as patients reach adulthood.

The ACC, the American Board of Pediatrics, and the American Board of Internal Medicine estimated that adult cardiologists outnumber pediatric cardiologists by 23:1. A 2009 ACC-sponsored workforce analysis documented over 20,000 practicing adult cardiologists (or cardiology subspecialists) as opposed to only 2,000 practicing pediatric cardiologists. In addition, there are only approximately 150 pediatric cardiology fellowship positions available annually compared to approximately 2,000 adult cardiology fellowship positions (10). The pediatric-trained cardiology workforce is not equipped to provide care for the rapidly expanding ACHD population and will need assistance from adult cardiology providers. Moreover, many younger pediatric cardiologists may be uninterested in caring for a growing percentage of adult patients.

Pediatric providers are often unable (and sometimes unwilling) to care for problems that are typically not encountered until adulthood. Adult providers are often much more accustomed (and may be better equipped) to provide care for patients with these issues. In addition, while the diagnosis of CHD may be the focus of the medical record in an ACHD patient, health maintenance and adult-onset diseases must not be overlooked. Adults with CHD often exhibit a significant lifetime risk of acquired cardiovascular disease, systemic hypertension, and diabetes (11). However, they typically report a low frequency of health maintenance care and counseling regarding contraception, pregnancy, and obesity prevention (12). In addition, routine recommended cancer screening is often overlooked (13).

In addition to the physical healthcare benefits that these patients may experience with adult-oriented care, transfer in care offers mental and emotional benefits to the patient, possibly related to the process of maturing into adulthood (8,14). If the choice (if available) is made to not transfer a patient to an adult provider, the
patient should still go through the transition process, as it is vital to the patient’s education, growth, and autonomy (14).

Ideally, patients should be involved in a formal transition program that provides uninterrupted health care that is patient-centered, comprehensive, age and developmentally specific, and flexible. Transition and transfer should be viewed as another step in the process and as an inevitable occurrence (4,9,16). The transition policy should ideally be institution-wide and predictable so patients and providers understand that it is a planned and coordinated progression (4,7). Yet, the transition process (and age at transfer) should be flexible enough to adapt to a patient’s developmental status (4,7,8). The program should include age-appropriate education about one’s health status and aid in developing skills in communication, decision-making, self-care, and self-reliance. This undoubtedly takes years to complete and should be started in the early teenage years. The ultimate goal is to maximize the ACHD patient’s quality of life, survival, and future productivity.

The first step, sometimes referred to as “envisioning a future,” involves giving the child and his/her family opportunities to think about the child as an adult. This plants the seed for lifelong follow-up as well as the expectation of an independent future (8,16). This can be considered as “pretransition” or preparation for transition in that it should occur during visits throughout early childhood. This involves discussing the expectations of a future with the ability to live an independent life and can begin to emphasize the need for a healthy, active lifestyle as well as the likely need for lifelong cardiac follow-up (4,7).

Overall this process involves many components to help ensure an appropriate and successful transition and eventual transfer of care.

The 2001 AAP consensus statement notes the importance of providing uninterrupted care as adolescents become adults (19). One of the most important goals of a transition process is to ensure continuous care for the patient and his/her family. As mentioned previously, education regarding the need for continuous, lifelong, care can be initiated during early childhood. Patient survey data have shown that one of the biggest obstacles to continuous care often is an unawareness of the need for this follow-up (5,20,21,22).

As defined earlier, the transition process prepares patients to “take responsibility for their own health care.” Patient maturity and accountability for their health is at the center of the transition and transfer process (7). Throughout the process, there is a gradual shift in responsibility from the parents to the patient. Kieckhefer et al. describes this process as similar to a shifting business relationship. Early in the transition process, the child moves to being his or her own provider with the parents acting as managers (supportive and able to assume control, if needed). This gradually evolves to the child as a manager with the parent shifting into a role of supervisor and, then, of consultant. Finally, when proper educational and maturity levels are achieved, the child becomes the CEO of his or her health care (23). In this setting, the eventual transfer of care to an adult provider is viewed as a “graduation” from the pediatric system and the achievement of autonomy (16).

One of the most important steps of the transition process involves patient education. Multiple studies have found that adolescent and young adult patients often lack understanding of their cardiac condition (12,21,24). This includes lack of knowledge regarding their initial diagnosis, surgical repair history, current treatment and medication plan, and what might be the expected course for the future. Given this, it is recommended that the transition process should include education related to a patient’s specific heart malformation and history. Table 68.2 displays the specific educational topics that should be covered over the course of visits during the transitioning period (7). Ideally, the patient’s family should be included in many aspects of this educational process, but the ultimate goal is for the patient to obtain the knowledge required for him or her to take full control of all healthcare responsibilities.

Educational plans should be individualized to take into account the patient’s complexity as well as their developmental capabilities and maturity. Early in the transition process (ages 12 to 15), basic concepts regarding the normal heart should be introduced and only once this basic understanding is achieved should discussion then focus on their individual cardiac malformation. Research has shown that typically by the age of 15, patients begin to grasp more complex concepts (24).

A major additional component, related to education involves organizing the personnel and locations that should be involved in the transition and transfer of a patient. Given that many CHD patients have multiple noncardiac concerns, this process must be individualized. Transfer involves primary care providers, noncardiac specialty care providers, as well as different locations of ancillary services. Whereas in pediatrics, the patient may have been able to obtain all of their required care in a single pediatric tertiary center or a multidisciplinary clinic location, the adult care may be spread out at multiple locations (16). To enable preservation of continuity and to address this often fragmented system, establishment of a medical home is a key step in the transition and transfer process. This medical home is typically a primary care provider. Adult primary care providers, however, often have little experience with caring for the transitioned adult patient. They often lack education regarding the complex “pediatric” medical conditions and may not be familiar

with the resources available for young adults with chronic healthcare needs (25). Thus, the ideal primary care provider should have specific experience in caring for adult survivors of chronic childhood disease. If this cannot be arranged, any provider, including specialty providers, with the time and resources in place to coordinate multidisciplinary care may serve as the medical home (4,8).