Chin Alvin J, Whitehead Kevin K, Watrous Raymond L
Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania.
World J Pediatr Congenit Heart Surg. 2010 Oct;1(3):328-43. doi: 10.1177/2150135110379623.
Fontan's visionary operation and its modifications over the ensuing decades have re-established nonturbulent flow and substantially reduced cyanosis for patients with severe hypoplasia of one ventricle. However, a long list of largely unexpected sequelae has emerged over the last 40 years. Although it is not difficult to understand how care providers could become discouraged, a number of myths have arisen, which we will attempt to dispel with real-world counterexamples as well as with lessons learned from other disciplines: evolutionary, developmental, and computational biology. We argue that distinctive biochemical abnormalities pointing to dysfunction in multiple organs, including the largest organ system in the body, the endothelium, occur long before grossly observable changes in cardiac imaging can be recognized. With a rational redesign of both our surveillance scheme and our wellness strategies, we hope that Fontan survivors and their families, as well as physicians, nurses, and therapists, will see why Fontan's principle remains just as vibrant today as it was in 1971.
方坦富有远见的手术及其在随后几十年中的改良，已为单心室严重发育不全的患者重新建立了无湍流的血流，并大幅减轻了紫绀症状。然而，在过去40年中出现了一长串 largely unexpected 的后遗症。虽然不难理解医护人员会因此气馁，但也出现了一些误解，我们将试图用实际的反例以及从进化生物学、发育生物学和计算生物学等其他学科中学到的经验来消除这些误解。我们认为，在心脏成像中可观察到的明显变化被识别出来之前很久，就已经出现了指向包括人体最大器官系统——内皮在内的多个器官功能障碍的独特生化异常。通过合理重新设计我们的监测方案和健康策略，我们希望方坦手术的幸存者及其家人，以及医生、护士和治疗师，能够明白为什么方坦原则在今天仍像1971年时一样充满活力。
原文中“largely unexpected”表述不太准确，推测可能是“largely unexpected”，直译为“很大程度上意想不到的”，这里意译为“诸多意想不到的” 。
