Abstract

For children and adolescents with end-stage chronic parenchymal and vascular pulmonary disease, lung transplantation is often the last therapeutic option. Since the first pediatric lung transplant in 1986 [1], the management of pediatric patients has developed rapidly, and currently lung transplantation is an accepted treatment in carefully selected children and adolescents, offering a net survival benefit and an improved quality of life [2–4]. While the overall survival after pediatric lung transplant has improved in recent years, rates of chronic lung allograft dysfunction (CLAD) and late mortality have essentially remained unaltered [2, 4]. Thus, the latest improvements have resulted from a reduction in early mortality, mainly due to infectious complications [2, 4]. According to the International Society for Heart and Lung Transplantation (ISHLT) Thoracic Transplant (TTX) Registry, a total of 101 pediatric lung transplants were performed worldwide in 2017. These cases were distributed among 37 centers, but only 5 centers performed more than 4 procedures [5]. While most pediatric lung transplants are performed in high-volume adult centers achieving excellent results [6], recent publications have shown that also the centers’ pediatric experience greatly influences the outcome [7]. Especially for the management of patients with cystic fibrosis (CF) undergoing lung transplant, pediatric and CF-specific knowledge is crucial, predicting better long-term survival [7].

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