Barracano Rosaria, Merola Assunta, Fusco Flavia, Scognamiglio Giancarlo, Sarubbi Berardo
Adult Congenital Heart Disease Unit, AO dei Colli, Monaldi Hospital, Naples, Italy.
Int J Cardiol Congenit Heart Dis. 2022 Jan 5;7:100322. doi: 10.1016/j.ijcchd.2022.100322. eCollection 2022 Mar.
Protein-losing enteropathy (PLE) represents a rare but severe and potentially life-threatening complication following Fontan operation in patients with a functional single ventricle. PLE is characterized by enteric protein loss, leading to devastating multiorgan involvement with increased morbidity and mortality. In spite of remarkable advances in the care of congenital heart disease in recent years, treatment of PLE is still one of the most challenging tasks due to limited understanding of the underlying mechanisms and lack of high-quality evidence from large scale, controlled studies to test the real efficacy of the several treatment strategies, which have been proposed. For this reason, we herewith aim to discuss the pathogenesis and diagnosis of PLE in Fontan patients as well as provide a comprehensive overview on potential advantages and disadvantages of the currently available therapeutic strategies, in order to propose a stepwise approach for the management of this unique condition.
蛋白丢失性肠病（PLE）是功能性单心室患者接受Fontan手术后一种罕见但严重且可能危及生命的并发症。PLE的特征是肠道蛋白质丢失，导致多器官严重受累，发病率和死亡率增加。尽管近年来先天性心脏病的治疗取得了显著进展，但由于对其潜在机制的了解有限，且缺乏来自大规模对照研究的高质量证据来检验已提出的几种治疗策略的实际疗效，PLE的治疗仍然是最具挑战性的任务之一。因此，我们在此旨在讨论Fontan患者PLE的发病机制和诊断，并全面概述当前可用治疗策略的潜在优缺点，以便为这种特殊情况的管理提出一种逐步方法。
