Internal Medicine and Pediatrics, Emory University, Atlanta, Georgia, USA.
Pediatric Cardiology, Children's Healthcare of Atlanta Inc, Atlanta, Georgia, USA.
Heart. 2020 Jan;106(2):105-110. doi: 10.1136/heartjnl-2019-314937. Epub 2019 Oct 29.
Adults with congenital heart disease who underwent the Fontan operation to palliate single ventricle heart defects-by direct connection of caval venous return to the pulmonary circulation-have improved survival due to advancements in surgical and interventional techniques. However, cardiac and non-cardiac comorbidities often coexist, complicating management, and contributing to premature morbidity and mortality. Cardiac issues include heart failure symptoms related to systolic and diastolic dysfunction, atrial and ventricular arrhythmias and systemic atrioventricular valve regurgitation. Structural issues may be related to obstruction of the Fontan pathway, or to branch pulmonary artery stenosis, both of which exacerbate symptoms. Non-cardiac complications in adults involve hepatic congestion, fibrosis and cirrhosis, hepatocellular carcinoma, chronic kidney disease, stroke, venous stasis, lymphatic issues and involvement of other organ systems. 'Fontan failure' refers to circulatory dysfunction, either cardiac, non-cardiac, or both, diagnosed after exclusion or treatment of reversible contributors such as structural issues and arrhythmias. Counselling about reproductive health, mental health, perioperative management and overall wellness are paramount for patients' well-being. Fontan patients are typically managed in highly specialised adult congenital heart centres, but may present to cardiologists or other practitioners with cardiac and non-cardiac emergencies or urgencies, sometimes after being out of care. In this review, we discuss the management of the adult Fontan patient, including surveillance, cardiac and non-cardiac complications, reproductive health, and advanced therapies.
患有单心室心脏缺陷的先天性心脏病成人通过腔静脉回流直接连接到肺循环进行法乐四联症手术姑息治疗，由于手术和介入技术的进步，生存率得到了提高。然而，心脏和非心脏合并症常常并存，使管理变得复杂，并导致过早发病和死亡。心脏问题包括与收缩和舒张功能障碍、房性和室性心律失常以及全身房室瓣反流相关的心衰症状。结构性问题可能与法乐四联症通路阻塞或分支肺动脉狭窄有关，这两者都会加重症状。成人的非心脏并发症包括肝充血、纤维化和肝硬化、肝细胞癌、慢性肾脏病、中风、静脉淤滞、淋巴问题以及其他器官系统的受累。“法乐四联症衰竭”是指在排除或治疗结构性问题和心律失常等可逆转因素后，诊断出的循环功能障碍，无论是心脏性、非心脏性还是两者兼有。对生殖健康、心理健康、围手术期管理和整体健康的咨询对于患者的福祉至关重要。法乐四联症患者通常在高度专业化的成人先天性心脏病中心进行管理，但可能会因心脏和非心脏急症或紧急情况就诊于心脏病专家或其他医生，有时是在离开护理之后。在这篇综述中，我们讨论了法乐四联症成年患者的管理，包括监测、心脏和非心脏并发症、生殖健康以及先进的治疗方法。
