Andersons pediatric cardiology 1777

FIG 67.8 Diagnosis distribution of recipients of heart transplantation by geographic location CHD, Congenital heart disease; DCM, dilated cardiomyopathy (From the registry of the International Society of Heart and Lung Transplantation J Heart Lung Transplant 2016;35(10):1185–1195.) Outcomes Mortality While Awaiting Transplantation Children waiting for heart transplantation have the highest mortality in solid organ transplantation.5,6 Death while waiting reflects a combination of availability of donor organs; the medical state of the recipient; recipient age, weight and diagnosis; and availability of durable mechanical circulatory support options.6,7 Although modifying the availability of organs is difficult, knowledge of the other factors that influence mortality during the period of waiting plays an important role in decision making regarding the appropriate timing of listing a patient for transplantation Analysis of the US Scientific Registry of Transplant Recipients database for the period between 1999 and 2006 demonstrates mortality for all listed patients while waiting of 17% at 1 year.6 Recipient characteristics associated with increased waitlist mortality include extracorporeal membrane oxygenation (ECMO) support, ventilator support, listing status 1A, congenital heart disease, renal replacement therapy, and nonwhite race A key finding is that waitlist mortality varies by as much as 10-fold based on recipient factors (5% to 39%) The mortality for infants during the period of waiting ranges from 25% to 30% and has consistently been higher than that reported for older patients Patients with a cardiomyopathy diagnosis have better waitlist outcomes than those with congenital heart disease Waitlist mortality even within the congenital heart disease group varies by underlying diagnosis and previous surgical palliation, especially for those with single-ventricle physiology.8 In the most recent era, there has been a 50% reduction in waitlist mortality due to the advent and more widespread use of durable ventricular assist devices (VADs) for mechanical support, with a fourfold higher likelihood of surviving to transplantation.7 Survival After Transplantation Data from the registry of the ISHLT2 show that survival after transplantation in the most recent era is 91% and 81% at 1 and 5 years, respectively, with a 10-year overall survival of 66% for the prior era (Fig 67.9) Looked at in a different way, the same data show a half-life for transplantation, defined as the time to 50% survival without death or retransplantation, of 20.7 years for those aged less than 1 year at transplantation, 18.2 years for those aged from 1 to 5 years, 14 years for those aged from 6 to 10 years, and 12.7 years for those older than 11 years— numbers that continue to improve almost annually (Fig 67.10) These differences are even more marked when conditional survival is examined, which excludes mortality related to the procedure itself, with a notable era effect (Figs 67.11 and 67.12) Infants and neonates are relatively protected from later complications, whereas adolescents, who have lower mortality over the short term, are at increased risk of death or the need for retransplantation during longterm follow-up FIG 67.9 Kaplan-Meier survival curve out to 25 years after heart transplantation during childhood stratified by era NA, Not applicable (From the registry of the International Society of Heart and Lung Transplantation J Heart Lung Transplant 2016;35[10]:1185–1195.)