Preparing Adolescents with Congenital Heart Disease for Transition to Adulthood

Just over 50 years ago, children with serious congenital heart disease (CHD) invariably suffered premature death or marked disability. With the invention of the heart-lung machine by Gibbons and its expanded utilization by Kurklin, Lillehei, and other early congenital heart surgeons, the outlook for these young patients improved significantly. Progress in the treatment of children with CHD has continued to evolve to the point that currently, there are few congenital heart defects than cannot be treated with open-heart surgery, catheter based therapy, or medication. These patients are now surviving into adulthood with the anticipation of leading healthy and productive lives. In fact, advances in the treatment of children with CHD have improved their life expectancy, such that nearly 90% of these children survive to adulthood.[1]

Because of the advances in surgical and other treatments for patients with CHD, the number of older patients having had definitive treatment for CHD has increased to the point that currently, there are nearly 1 million patients in the United States in this situation. Patients with CHD were traditionally treated by pediatricians or pediatric cardiologists. However, the care of these patients should optimally be transitioned from pediatric to adult-centered practitioners.[2] Most of these patients, unfortunately, do not receive optimal care, in part, because of the absence or underutilization of structured programs dedicated to the treatment of adults with CHD. In addition, patients are often lost to appropriate follow-up because they no longer see their pediatrician when they become an adolescent or adult. In fact, less than half of adults with CHD are seen by providers specializing in the care of these patients.

The goals of a formal transition program should prepare young adults for the transfer of care to an adult-oriented system. This transition in care should foster independence and a sense of control over their own care decisions, and thereby improve quality of life, life expectancy, and self-sufficiency.

As outlined in the Best Practice paper,[2] the successful transition of a child with CHD from a family-centered pediatric system to a patient-centered adult chronic care system should include coordinated comprehensive care, with access to adequate financing, education of physicians and other providers in the management of adult patients with CHD, and ongoing, coordinated communication between all parties involved. The statement and its content is critically important in that it provides guidelines for clinicians to promote early and high-quality care and support for their patients with CHD for successful transition of care. In the "best case" scenario, adults with CHD live normal, well-adjusted lives. Unfortunately this is often not the case, but rather these individuals may suffer emotional and behavioral problems, a diminished quality of life, and be subject to the consequences of misinformation.

Transition planning for adolescents and young adults with CHD cannot occur without a simultaneous evaluation of the impact of the diagnosis on their parents, other family members, and the patient's social environment. In addition, the primary care physician or other provider plays a critical role in the transition process. During adolescence, the coordination of care between primary and specialty providers is particularly important. Expertise in adolescent medicine is important, including discussion of the usual adolescent issues including sex, birth control, drugs, and alcohol. Although adolescents may not consider having children in the near future, information should be available with regard to the risk of pregnancy, birth control options, family planning, and the likelihood of having a child with CHD. More general issues relate to daily life considerations such as social interactions, job choice, exercise, and participation in athletics. Although it is often the case that young patients are "cured" of CHD, it is also often not the case. Continued close follow-up is often necessary, and in many cases, additional surgery may be required and life expectancy may be decreased. These issues will need to be considered when making some life choices. When required, health professionals should consider several factors when discussing end of life and other serious issues with a young person, including the person's developmental level and decision-making capability. Although most patients with CHD now can expect to life a near-normal life, these issues unfortunately still arise in some cases.

The principles that provide the framework whereby the transition experience for young adult patients with CHD are well described in the context of a model that incorporates three phases of the transition process: pre-transition, transition, and transfer. These principles are well outlined in the text of the paper.[2]

One issue that remains is the impact of future health care changes and financial constraints of the transition process. Although managing transition to adulthood for adolescents with CHD is important, it is probably perceived as less critical than other medical issues (e.g., an operation to correct transposition of the great vessels). Thus, emphasis on the transition process and other medical and psychosocial issues may be neglected or receive less emphasis in the process of health care reform. However, many of the concepts brought up in the Best Practice paper are not dependent on health care financing, but rather on good clinical practice. There is no reason to believe that the transition process cannot proceed in large part without significantly impacting overall healthcare cost. In fact, improved care may potentially reduce overall health care expenditure.

In summary, an increasing number of young people with CHD are surviving into adulthood with a near-normal life expectancy and excellent quality of life. Thus, there is an urgent need for programs designed to facilitate their transition from pediatric to adult health care environments. The current paper outlines the principle, process, and best practices of transition, its importance in the development of well-adjusted individuals with CHD, and the delivery of optimal care to this growing population of patients.