Andersons pediatric cardiology 1791

FIG 67.30 Malignancy-free survival in children after heart transplantation (From the registry of the International Society of Heart and Lung Transplantation J Heart Lung Transplant 2016;35(10):1185–1195.) PTLD includes all clinical syndromes associated with lymphoproliferation after transplantation The spectrum ranges from a mononucleosis-like illness to life-threatening malignancies with clonal chromosomal abnormalities EBV plays a major role in their development, with the highest risk for development being a primary infection with the virus Given their naivete to EBV, primary infection, and thus PTLD, this problem is more common in children The risk is also related to patient age at time of transplant (infancy, childhood, adolescence), EBV serostatus, and the viral load.16,107 There are no controlled clinical trials comparing interventions or therapies for these disorders Treatments include reduction in immunosuppression, antiviral medication, immunoglobulin therapy, monoclonal antibodies such as rituximab, chemotherapy, tumor debulking, radiation therapy, and, rarely, bone marrow transplantation.108 Treatment for PTLD carries with it a higher risk of rejection related to the reduction of immunosuppression required to treat the disease.15,16 Growth Somatic growth retardation is common in children requiring cardiac transplantation This is especially true of those with congenital heart disease and those with long waitlist times Posttransplantation weight and body mass index Z-scores trend toward normalization, but height Z-scores exhibit less catchup.109 Cardiac growth occurs in line with somatic growth.110 Development Assessment of infant development has revealed that mean developmental scores for both mental and psychomotor indexes fall within normal limits, although they have tended to be at the lower end of normal.111 Reports beyond infancy demonstrate a range of variation in a spectrum of developmental parameters compared with a normal population, with overall cognitive scores at the lower limit of normal.18 Focused studies of patients born with hypoplastic left heart syndrome have all demonstrated lower scores on the scales for infant development and intelligence compared with the general population, but the scores are similar to those of patients undergoing the Norwood sequence of palliative procedures.112 Functional Status, Quality of Life, and Psychosocial Issues A functional status score is collected in the United States for all pediatric heart transplant recipients, and a recent analysis showed that the vast majority of children who survive at least 1 year posttransplant have an excellent functional status with few limitations to daily life.113 Although studies are limited, most children and families report a good quality of life following heart transplantation.114 A longitudinal study of children after transplantation of either the heart or the heart and lungs showed academic cognitive functioning to be in the normal range, without change as a function of time from transplantation.115 Performance at school, however, was significantly poorer than that of healthy children, and the prevalence of behavioral problems was significantly greater Similar results were reported in another longitudinal study that followed children for more than 10 years, with psychologic functioning outside the normal range in one-quarter of patients.116 Pre- and postoperative psychologic assessment, and support of the patient and his or her family is clearly an important role of any center involved in cardiac transplantation A recent study from Stanford evaluated quality of life in adults following pediatric heart transplantation and revealed a good quality of life with a reasonable level of success in educational and work-related achievements.117 Nonadherence Nonadherence remains a significant challenge for childhood recipients of heart transplants, especially during adolescence Adolescence itself is independently associated with worse survival.2 The reasons for this are multifactorial and include biologic factors, adherence to medical care, and the normal developmental challenges of adolescence that affect the ability to transition to self-monitoring and self-care The complex psychologic issues already described combined with normal adolescent development after cardiac transplantation render a large population of recipients at risk of nonadherence.19,118,119 Nonadherence has been linked to late rejection and to high rates of death in adolescents.19 High variability in trough levels of medications, with both high and low levels presumably reflecting variable adherence, is a marker for children at greater risk for recurrent rejection and hospitalization after transplantation.120 There are increasing strategies to recognize and manage nonadherence including web-based and/or medical apps targeted at the technology-savvy adolescent.121 A coordinated approach between pediatricians, the transplant team, the patient, and the family must be in place,119,122 particularly at the time of transition from pediatric to adult-based care Transition to Adult Care Transition is a planned process that addresses the medical, psychologic, and educational needs of adolescents and young adults with chronic physical and medical conditions as they move from child-centered to adult-oriented care Medium-term rejection and graft loss has been associated with an ineffective transition process Transition is not a single time point but rather a process that should be initiated with patients in their early teens and their families; it is an ongoing educational endeavor with the goal of providing uninterrupted health care Major educational topics include learning about their heart disease, medication doses and side effects, and signs and symptoms of infection and rejection Additional milestones and skills all focusing on promoting selfmanagement include learning to contact the pharmacy for medication refills, ... A coordinated approach between pediatricians, the transplant team, the patient, and the family must be in place,119,122 particularly at the time of transition from pediatric to adult-based care... family is clearly an important role of any center involved in cardiac transplantation A recent study from Stanford evaluated quality of life in adults following pediatric heart transplantation and revealed a good quality of life with a reasonable level of success in educational and work-related achievements.117... Functional Status, Quality of Life, and Psychosocial Issues A functional status score is collected in the United States for all pediatric heart transplant recipients, and a recent analysis showed that the vast majority of children who survive at least 1 year posttransplant have an excellent functional