Approximately 90% of children born with congenital heart disease (CHD) are expected to reach adulthood.1 With advances in surgical and medical treatment, there are now more adults than children living with CHD,2 with a current estimated 1.3 million adults with CHD in the United States.3–5 This burgeoning population is accompanied by a set of specialized healthcare needs and quality-of-life concerns. Understanding what to expect for patients, parents, and their healthcare providers is essential for the well- being and adjustment of these children as they grow up into young adulthood and beyond.
The purpose of this chapter is to review the quality of life of survivors of CHD, their ongoing medical needs, and the setting and team needed to provide them with optimal care past the pediatric age group.
It is increasingly important to prepare the pediatric patient with CHD and their family for transition into the adult healthcare system. Transition is defined as the “purposeful and planned movement of adolescents and young adults with chronic physical and medical conditions from child-centered to adult-oriented healthcare systems.”6 Young adults are at risk for being lost to care during this time period; it is estimated that between one half and three quarters of adults with CHD do not have regular cardiology follow-up.7,8 For those who are lost to follow-up, there is a 3-fold increase in the need for an urgent intervention such as cardiac catheterization or surgery.8
Although several models have been proposed for transition,9 it is generally agreed that the goal is for the patient to enter the adult care environment and to establish accountability for their own medical care including communication, autonomy, self-care, and self-advocacy (Table 17-1).10–12 The healthcare team, the family, and the patient must work together to achieve successful transition. The process should begin in childhood at an appropriate age for the individual child's psychosocial maturation and neurodevelopment.13 Providers should begin early, age-appropriate education of the patient to encourage understanding of his or her disease process. As they develop into teenagers, patients should be given more autonomy, and a portion of the office visit should be conducted without the family present.10 This should be framed in such a way that the family sees their role as one of encouragement and empowerment, but does not feel excluded from the patient's care. It is imperative that the family be prepared early in the transition process to allow increasing autonomy for the pediatric patient who will one day be responsible for his or her own care. The probability of a successful transition increases dramatically when pediatric providers set the stage for the following 2 fundamental transition goals: patient education about their medical condition and parental encouragement to allow their child the independence to take an active role in their own care.14 A summary of the recommendations from the 32nd Bethesda Conference ...