The evolution of pediatric heart retransplantation over three decades: An analysis from the PHTS.

BACKGROUND: Retransplantation is rare and associated with worse survival and more morbidity. The study aim is to describe an updated cohort of pediatric retransplants, determine if there has been an era effect on outcomes, and understand if identified trends are explained by changes in patient selection. METHODS: Pediatric Heart Transplant Society database analysis of retransplantation patients <18 years of age (Era 1: 1993-2001, Era 2: 2002-2010, Era 3: 2011-2018). Multivariate analysis identified risk factors for graft loss. Multiphase parametric hazard modeling was used to depict era and risk factor effect. RESULTS: Survival was lower (p < .0001) for retransplant (n = 222) compared to primary transplant (n = 6548) (median 9.3 vs 20.2 years). Median survival increased from Era 1 to 2 (4.8 vs 9.3 years; p < .0001) with no incremental change in Era 3. Era 2 and 3 retransplants had a longer inter-transplant interval (p < .0001), were less frequently for early graft failure (p = .0004) or acute rejection (p = .007), more frequently from a ventricular assist device (p = .0014), and less frequently from extracorporeal membrane oxygenation (p = .0024). Predictors of graft loss included Era 1 (HR 10.55, p = .001), congenital heart disease (HR 4.42, p = .01), inter-transplant interval <1 year (HR 5.34, p = .002), and mechanical support (ventricular assist device HR 7.47, p = .0042; extracorporeal membrane oxygenation HR 10.09, p < .0001). For each 1-year increase in inter-transplant interval, graft loss risk decreased by 1.15 (p = .0002). Retransplantation was associated with more rejection, infection, and allograft vasculopathy. CONCLUSIONS: Graft survival has improved in pediatric retransplants making it a viable option in select patients. Retransplantation should be avoided in the setting of early graft failure especially requiring mechanical support.

Double-Lumen Aortic Arch: Persistence of the Fifth Aortic Arch.

A persistent fifth aortic arch is exceptionally rare. We report a patient with a double-lumen aortic arch in association with a perimembranous ventricular septal defect and Cornelia de Lange syndrome. We also discuss the morphologic consequences of persistent fifth aortic, arch, which may not be limited to the double-lumen aorta but in fact may be more common.