Jalal Zakaria, Gewillig Marc, Boudjemline Younes, Guérin Patrice, Pilati Mara, Butera Gianfranco, Malekzadeh-Milani Sophie, Avesani Martina, Thambo Jean-Benoit
University Hospital of Bordeaux - Department of Pediatric and Adult Congenital Cardiology, Pessac, France.
IHU LIRYC Electrophysiology and Heart Modeling Institute, Fondation Bordeaux Université, Pessac, France.
Front Pediatr. 2022 Aug 30;10:965989. doi: 10.3389/fped.2022.965989. eCollection 2022.
The Fontan operation represents the last of multiple steps that are offered a wide range of congenital cardiac lesions with a single ventricle (SV) physiology. Nowadays this surgical program consists of a total cavopulmonary connection (TCPC), by anastomosing systemic veins to the pulmonary arteries (PAs), excluding the right-sided circulation from the heart. As a result of imaging, surgical, percutaneous, and critical care improvements, survival in this population has steadily increased. However, the Fontan physiology chronically increases systemic venous pressure causing systemic venous congestion and decreased cardiac output, exposing patients to the failure of the Fontan circulation (FC), which is associated with a wide variety of clinical complications such as liver disease, cyanosis, thromboembolism, protein-losing enteropathy (PLE), plastic bronchitis (PB), and renal dysfunction, ultimately resulting in an increased risk of exercise intolerance, arrhythmias, and premature death. The pathophysiology of the failing Fontan is complex and multifactorial; i.e., caused by the single ventricle dysfunction (diastolic/systolic failure, arrhythmias, AV valve regurgitation, etc.) or caused by the specific circulation (conduits, pulmonary vessels, etc.). The treatment is still challenging and may include multiple options and tools. Among the possible options, today, interventional catheterization is a reliable option, through which different procedures can target various failing elements of the FC. In this review, we aim to provide an overview of indications, techniques, and results of transcatheter options to treat cavopulmonary stenosis, collaterals, impaired lymphatic drainage, and the management of the fenestration, as well as to explore the recent advancements and clinical applications of transcatheter cavopulmonary connections, percutaneous valvular treatments, and to discuss the future perspectives of percutaneous therapies in the Fontan population.
Fontan手术是针对多种具有单心室（SV）生理特征的先天性心脏病变所采取的一系列治疗步骤中的最后一步。如今，该手术方案包括完全腔肺连接（TCPC），即将体循环静脉与肺动脉（PA）吻合，使心脏右侧循环与心脏分离。由于影像学、外科手术、经皮介入及重症监护技术的改进，这一群体的生存率稳步提高。然而，Fontan生理状态会使体循环静脉压力长期升高，导致体循环静脉淤血和心输出量降低，使患者面临Fontan循环（FC）衰竭的风险，这与多种临床并发症相关，如肝病、发绀、血栓栓塞、蛋白丢失性肠病（PLE）、塑料支气管炎（PB）和肾功能障碍，最终导致运动耐量下降、心律失常和过早死亡的风险增加。Fontan循环衰竭的病理生理学复杂且具有多因素性，即由单心室功能障碍（舒张期/收缩期衰竭、心律失常、房室瓣反流等）或特定循环（管道、肺血管等）引起。治疗仍然具有挑战性，可能包括多种选择和手段。在众多可能的选择中，如今介入导管术是一种可靠的选择，通过它可以采用不同的操作来针对FC的各种衰竭因素。在本综述中，我们旨在概述治疗腔肺狭窄、侧支循环、淋巴引流受损以及开窗处理的经导管治疗方法的适应证、技术和结果，探讨经导管腔肺连接、经皮瓣膜治疗的最新进展和临床应用，并讨论Fontan人群经皮治疗的未来前景。
