Nationwide Children's Hospital, Ohio State University, Columbus, Ohio, USA.
University Health Network, Toronto General Hospital, Toronto, Ontario, Canada.
Can J Cardiol. 2022 Jul;38(7):1036-1047. doi: 10.1016/j.cjca.2022.02.023. Epub 2022 Mar 1.
Atrial arrhythmias are highly prevalent in the aging Fontan population and contribute importantly to morbidity and mortality. Although the most common arrhythmia is scar-based intra-atrial re-entrant tachycardia, various other arrhythmias may occur, including focal atrial tachycardia, atrioventricular node-dependent tachycardias, and atrial fibrillation. The type and prevalence of atrial arrhythmia is determined, in part, by the underlying congenital defect and variant of Fontan surgery. Although the cumulative incidence of atrial tachyarrhythmias has decreased substantially from the atriopulmonary anastomosis to the more recent total cavopulmonary-connection Fontan, the burden of atrial arrhythmias remains substantial. Management is often multifaceted and can include anticoagulation, anti-arrhythmic drug therapy, pacing, and cardioversion. Catheter ablation plays a key role in control of arrhythmia. Risks and benefits must be carefully weighed. Among the important considerations are the clinical burden of arrhythmia, ventricular function, hemodynamic stability in tachycardia, suspected arrhythmia mechanisms, risks associated with anaesthesia, venous access, approaches to reaching the pulmonary venous atrium, and accompanying comorbidities. Careful review of surgical notes, electrocardiographic tracings, and advanced imaging is paramount, with particular attention to anatomic abnormalities such as venous obstructions and displaced conduction systems. Despite numerous challenges, ablation of atrial arrhythmias is effective in improving clinical status. Nevertheless, onset of new arrhythmias is common during long-term follow-up. Advanced technologies, such as high-density mapping catheters and remote magnetic guided ablation, carry the potential to further improve outcomes. Fontan patients with atrial arrhythmias should be referred to centres with dedicated expertise in congenital heart disease including catheter ablation, anaesthesia support, and advanced imaging.
心房心律失常在老年 Fontan 人群中非常普遍，对发病率和死亡率有重要影响。虽然最常见的心律失常是基于瘢痕的心房内折返性心动过速，但也可能发生各种其他心律失常，包括局灶性心房性心动过速、房室结依赖性心动过速和心房颤动。心房心律失常的类型和发生率部分取决于潜在的先天性缺陷和 Fontan 手术的变异。尽管从心-肺吻合术到最近的全腔静脉肺动脉连接 Fontan，房性心动过速的累积发生率已大幅下降，但心房性心律失常的负担仍然很大。治疗通常是多方面的，包括抗凝、抗心律失常药物治疗、起搏和电复律。导管消融在控制心律失常方面起着关键作用。必须仔细权衡风险和益处。重要的考虑因素包括心律失常的临床负担、心室功能、心动过速时的血液动力学稳定性、可疑心律失常机制、麻醉相关风险、静脉通路、到达肺静脉心房的方法以及伴随的合并症。仔细审查手术记录、心电图描记和高级影像学至关重要，特别要注意静脉阻塞和传导系统移位等解剖异常。尽管存在许多挑战，但消融治疗心房心律失常可有效改善临床状况。然而，在长期随访中，新的心律失常很常见。高级技术，如高密度标测导管和远程磁引导消融，有可能进一步改善结果。有房性心律失常的 Fontan 患者应转诊至专门从事先天性心脏病的中心，包括导管消融、麻醉支持和高级影像学。
