Ohuchi Hideo
Department of Pediatric Cardiology and Adult Congenital Heart Disease, National Cerebral and Cardiovascular Center, Osaka, Japan.
J Cardiol. 2016 Sep;68(3):181-9. doi: 10.1016/j.jjcc.2016.04.001. Epub 2016 Apr 29.
Most of patients after the Fontan operation can reach their adulthood, however, the management strategy for this complex pathophysiology has not been yet established. In general, elevated central venous pressure (CVP) and low cardiac output (CO) due to impaired ventricular preload characterize the Fontan circulation and the ideal hemodynamics could be a combination of a lower CVP with a higher CO. Thus, preserved functional systemic ventricle with low pulmonary artery resistance is thought to be crucial for better long-term outcome. However, on the other hand, because of the unique hemodynamics, these patients have significantly higher incidence of complications, sequelae, and even mortality. The major complications are supraventricular arrhythmias, heart failure, and Fontan-related problems, including protein-losing enteropathy and pulmonary arteriovenous fistulae, both of which are refractory to the treatments, and most of these "Fontan inconveniences" increase as patients age. In addition, one of the recent emerging problems is Fontan-associated liver disease that includes liver cirrhosis and hepatocellular carcinoma. Furthermore, women with Fontan circulation also reach childbearing age and there have been increasing numbers of reports showing a high incidence of pregnancy-associated complications. All these problems may be a part of "Fontan inconveniences" because most of the current Fontan patients are still "young" i.e. in their twenties or thirties and it may be not surprising that more new Fontan-associated pathophysiology emerges as patients age. Recent evidence reminds us of the concept that adult Fontan pathophysiology is not just a cardiovascular disease, rather, a multiorgan disease with many interactions between cardiovascular and non-cardiovascular organs. Therefore, a multidisciplinary approach is mandatory to take care of and anticipate the better long-term outcome.
大多数接受Fontan手术的患者能够成年，然而，针对这种复杂病理生理学的管理策略尚未确立。一般来说，Fontan循环的特征是由于心室前负荷受损导致中心静脉压（CVP）升高和心输出量（CO）降低，而理想的血流动力学状态可能是较低的CVP与较高的CO相结合。因此，保留功能正常且肺动脉阻力低的体循环心室被认为对更好的长期预后至关重要。然而，另一方面，由于独特的血流动力学，这些患者并发症、后遗症甚至死亡率的发生率显著更高。主要并发症包括室上性心律失常、心力衰竭以及与Fontan相关的问题，如蛋白丢失性肠病和肺动静脉瘘，这两者对治疗均难起效，并且随着患者年龄增长，这些“Fontan不便之处”大多会增加。此外，近期出现的问题之一是Fontan相关肝病，包括肝硬化和肝细胞癌。此外，处于Fontan循环的女性也到了育龄期，越来越多的报告显示妊娠相关并发症的发生率很高。所有这些问题可能都是“Fontan不便之处”的一部分，因为目前大多数Fontan患者仍“年轻”，即二三十岁，随着患者年龄增长出现更多新的Fontan相关病理生理学情况可能并不奇怪。最近的证据让我们想起这样一个概念，即成人Fontan病理生理学不仅仅是一种心血管疾病，而是一种多器官疾病，心血管和非心血管器官之间存在许多相互作用。因此，必须采取多学科方法来照顾并预期获得更好的长期预后。
