De Rita Fabrizio, Crossland David, Griselli Massimo, Hasan Asif
Department of Congenital Cardiothoracic Surgery, Freeman Hospital, Newcastle Upon Tyne, UK.
Department of Congenital Cardiology, Freeman Hospital, Newcastle Upon Tyne, UK.
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2015;18(1):2-6. doi: 10.1053/j.pcsu.2015.01.004.
With and increasing number of early survivors after the palliation of the single ventricle physiology there is a burgeoning Fontan population worldwide that will pose unique challenges because of the inevitable sequelae related to the absence of the alleged "needless" sub-pulmonic ventricle. The increasing number and older-age single-ventricle patients highlights the results of successful contemporary surgical palliation in children, leading to the development of an adult single-ventricle population with unpredictable socio-economic and health service impacts. The wide variability in clinical status of patients with Fontan circulation reflects not only the broadened spectrum of morphological substrates involved, but also the evolving surgical techniques during the last four decades. This has come in the wake of a gradual understanding of an incredibly tricky physiology. The magnitude of the disease, the physio-pathological mechanisms, and the therapeutic options to optimize the "failing Fontan" status and to delay the irreversible deterioration of "Fontan failure" condition are discussed in this review.
随着单心室生理姑息治疗后早期存活者数量的增加，全球范围内接受Fontan手术的人群不断壮大，由于与所谓“不必要”的肺下室缺失相关的不可避免的后遗症，这将带来独特的挑战。单心室患者数量的增加和年龄的增长凸显了当代儿童心脏手术姑息治疗的成功结果，导致了一个对社会经济和医疗服务影响不可预测的成年单心室人群的出现。Fontan循环患者临床状况的广泛差异不仅反映了所涉及的形态学底物范围的扩大，也反映了过去四十年来不断发展的手术技术。这是在对极其复杂的生理学逐渐理解之后出现的。本文综述了该疾病的严重程度、生理病理机制以及优化“衰竭Fontan”状态和延缓“Fontan衰竭”病情不可逆恶化的治疗选择。
