Abstract
Heart transplantation (HTx) in children with a univentricular physiology is a challenge. In this study, we aimed to investigate the early and late survival as well as the causes of death of HTx recipients at different stages of univentricular palliation. Between January 1987 and September 2015, 40 orthotropic cardiac transplants were performed in 38 children with univentricular hearts at our institution. Outcomes were reviewed using medical records and transplant databases. For the purposes of this analysis, patients were divided into 3 subgroups according to their stage of palliation: Stage 1 (n = 10, 26%), Stage 2 (n = 5, 13%) and Fontan (n = 23, 61%). The median age at HTx was 15.2 years (range 0-38). The median follow-up time after transplantation was 8.7 years (range 0-25.4). Indications for transplant were ventricular dysfunction in 25 (66%) patients, protein losing enteropathy in 10 patients transplanted in Fontan (26%) and refractory arrhythmias in 3 (8%) patients with an atriopulmonary connection. Total mortality was 42% (4.84/100 patient-years), and total early mortality was 21%. Overall survival at 1, 10 and 20 years was 73% (95% confidence interval 56-84%), 58% (95% confidence interval 40-72%) and 49% (95% confidenc...Continue Reading
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