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Andersons pediatric cardiology 1792

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understanding when and how to contact the transplant team, and practicing the scheduling of their own appointments and arranging transportation for clinic visits The importance of and approaches to transition for childhood recipients of heart transplantation continue to evolve.123 Transplantation of the Heart and Lungs Transplantation of the heart together with the lungs has limited application in children, with 10 or less being reported to the registry of the ISHLT annually since 2007.124 The main indication remains pulmonary arterial hypertension in 61% and cystic fibrosis in 28% Most occur in children and adolescents with no reports of infant heart-lung transplants since 2008 and only two since 2000 (Video 67.2) Postoperative transplant-related morbidities are similar to those for isolated transplantation of the heart and the lungs, with the issues relating to transplantation of the lungs predominating and generally guiding the clinical course and plans for treatment Long-term outcomes are well below those for transplantation of the heart and have shown no improvement over the last decade, with a median survival of 4.8 years for pulmonary arterial hypertension, 3.8 years for cystic fibrosis, and 2.4 years for congenital heart disease.124 Future Directions Overall, the survival for children undergoing transplantation of the heart has improved remarkably during the past 20 years, and the functional status of most patients is excellent Despite this, there is a continued risk of late morbidity related to immunosuppression Mortality while waiting for transplantation, though improved, remains unacceptably high—especially for infants and those with congenital heart disease Improved management of heart failure and mechanical support options now more effectively bridge older children and adolescents to transplantation Key opportunities include the development of mechanical support options for infants and smaller children and those with congenital heart disease, newer immunosuppressant agents to mitigate antibodymediated rejection, and noninvasive long-term surveillance strategies

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