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Little is known about the quality of life (QOL) of children with heart disease who undergo life-saving surgery. The aim of this multicenter study was to examine self- and parent-reported QOL outcomes in pediatric heart transplant recipients.Pediatric heart transplant recipients/families (n = 174) from 7 transplant programs completed the Pediatric Quality of Life Inventory Generic Core Scales and Cardiac Module. Scores for the heart transplant sample were compared with non-transplant patients who had undergone conventional cardiac surgery and with a healthy child sample. Within the cardiac surgery group, heart disease/surgery was further categorized by severity/complexity.Heart transplant recipients were a mean age of 10.6 ± 4.7 years at a mean time post-transplant of 6.0 ± 4.1 years. By both self-report and parent proxy report, mean scores for heart transplant recipients were significantly lower than those in healthy children for physical and psychosocial QOL, including emotional and social functioning (p0.001), with 31.3% self-reporting significantly impaired psychosocial QOL scores. By self-report, there were no significant differences in emotional and social mean scores between the transplant and cardiac surgery groups. Transplant recipients reported significantly fewer cardiac symptoms than children with cardiac surgery (p0.01). Their self-reported school functioning scores were not significantly different from children with moderate to severe disease.Although pediatric heart transplant recipients experience significant symptomatic improvement, they remain at-risk for impaired psychosocial QOL, similar to children with residual or palliated heart disease. Assessment is needed to identify children at-risk and improve psychosocial outcomes.

Purpose Repeat heart transplantation (re-HTx) is standard practice in many pediatric centers, with acceptable outcomes in appropriate patients (pts). There are little data available on outcomes of 3 r4 HTx following failure of a 2 144 graft. We sought to compare outcomes of 3 r4 HTx in pediatric and young adult pts to outcomes of comparable pts with 2 144 HTx. Methods and Materials All recipients of 3 r4 HTx in whom primary HTx was prior to age 21 were identified in the United Network of Organ Sharing (UNOS) database (1985-2011) and matched 3:1 with a control group of 2 144 HTx pts by age, era, and re-HTx indication. The primary outcome measure was 1 and 5 year (yr) survival; secondary outcomes included inter-group differences in rejection and cardiac allograft vasculopathy (CAV). Results At time of HTx, cases (n=27) and controls (n=79) were alike in sensitization (PRA > 10 in 29.6% vs 29.1%, p= 0.94), serum creatinine (median 1.2 vs 1.1, p=0.30), bilirubin (median 0.8 vs 0.6, p=0.79), hospitalization (33.3% vs 53.2%, p=0.08), inotropic support (25.9% vs 41.8%, p=0.14) and mechanical ventilation (7.4% vs 7.6%, p=1.0). Inter-HTx interval was shorter for cases than controls (median 5.0 yr vs 9.8 yr, p=0.001). There was no difference in survival between groups at 1 and 5 yrs (76% vs 80% at 1 yr and 62% vs 58% at 5 yrs, p=0.75). The prevalence of rejection within the first yr (33.3% vs 44.3%, p=0.32) and post-HTx CAV (14.8% vs 30.4%, p=0.11) were comparable between groups. Risk factors for non-survival in 3 r4 HTx pts were: mechanical ventilation at HTx (Hazard ratio (HR) 8.4, 95% confidence interval (CI) 1.5-46.5); ECMO support at HTx (HR 25.5, 95% CI 1.6-408); and elevated serum bilirubin at HTx (HR 2.3, 95% CI 1.2-4.2). Conclusions Third HTx remains rare in pediatric and young adult pts. Outcomes of 3 r4 HTx are similar to 2 144 HTx in matched pts, with no difference in short or long term survival and comparable rates of early rejection and CAV. Consideration of 3 r4 HTx appears reasonable in appropriately selected pts.

Predicting 1-Year Survival in Pediatric Heart Transplant Candidates with Congenital Heart Disease: An Analysis of the PHTS Database K.R. Schumacher, D. Naftel, M. Tresler, R. Kirk, C. Almond, T.P. Singh, R. Spicer, T.M. Hoffman, D. Hsu, C. Canter, M. Zamberlan, R.J. Gajarski. Congenital Heart Center, University of Michigan, Ann Arbor, MI; University of Alabama, Birmingham, AL; Freeman Hospital, Newcastle Upon Tyne, United Kingdom; Boston Children’s Hospital, Boston, MA; Cincinnati Children’s Hospital, Cincinnati, OH; Nationwide Children’s Hospital, Columbus, OH; The Children’s Hospital at Montefiore, New York, NY; St. Louis Children’s Hospital, St. Louis, MO.

stains in these samples were negative, and lymphocyte marker stains revealed mostly CD3 lymphocytes. Conclusions: Post-transplant pulmonary capillaritis can occur in very young children, and as hypothesized in adults, likely represents a form of humoral rejection that is distinct from typical acute cellular rejection. Immunohistochemical stains may be useful in differentiating a humoral basis for lung rejection. Rituximab may be an effective treatment for acute pulmonary capillaritis/ humoral rejection.