Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, USA.
Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, USA.
Pediatr Cardiol. 2022 Aug;43(6):1175-1192. doi: 10.1007/s00246-022-02930-z. Epub 2022 May 23.
Today, it is anticipated most individuals diagnosed with single-ventricle malformation will survive surgical reconstruction through a successful Fontan operation. As greater numbers of patients survive, so has the recognition that individuals with Fontan circulation face a variety of challenges. The goal of a normal quality and duration of life will not be reached by all. The hurdles fall into a variety of domains. From a cardiovascular perspective, the Fontan circulation is fundamentally flawed by its inherent nature of creating a state of chronically elevated venous pressure and congestion, accompanied by a relatively low cardiac output. Ventricular dysfunction, atrioventricular valve regurgitation, and arrhythmia may directly impact cardiac performance and can progress with time. Problems are not limited to the cardiovascular system. Fontan circulatory physiology impacts a multitude of biological processes and health parameters outside the heart. The lymphatic circulation is under strain manifesting as variable degrees of protein-rich lymph loss and immune system dysregulation. Organ system dysfunction develops through altered perfusion profiles. Liver fibrosis is ubiquitous, and a process of systemic fibrogenesis in response to circulatory stressors may affect other organs as well. Somatic growth and development can be delayed. Behavioral and mental health problems are common, presenting as clinically important levels of anxiety and depression. Most striking is the high variability in prevalence and magnitude of these complications within the population, indicating the likelihood of additional factors enhancing or mitigating their emergence. We propose that optimal care for the individual with single ventricle and a Fontan circulation is ideally offered in a comprehensive multidisciplinary manner, with attention to elements that are beyond cardiac management alone. In this report, we share the concepts, our experiences, and perspectives on development of a clinic model-the "Fontan rehabilitation, wellness and resilience development" or FORWARD program. We provide insights into the mechanics of our multidisciplinary model of care and the benefits offered serving our growing population of individuals with a Fontan circulation and their families.
如今，预计大多数被诊断为单心室畸形的患者将通过成功的 Fontan 手术重建而存活。随着越来越多的患者存活下来，人们已经认识到 Fontan 循环的患者面临着各种挑战。并非所有患者都能达到正常的生活质量和寿命。这些障碍涉及多个领域。从心血管的角度来看，Fontan 循环由于其固有的本质，即造成慢性静脉压升高和充血状态，并伴有相对较低的心输出量，因此存在缺陷。心室功能障碍、房室瓣反流和心律失常可能直接影响心脏功能，并随时间推移而进展。问题不仅限于心血管系统。Fontan 循环生理学影响心脏以外的多种生物过程和健康参数。淋巴循环承受压力，表现为不同程度的富含蛋白质的淋巴丢失和免疫系统失调。器官系统功能障碍通过改变灌注模式发展。肝纤维化是普遍存在的，对循环应激的全身性纤维化反应也可能影响其他器官。躯体生长和发育可能会延迟。行为和心理健康问题很常见，表现为临床重要程度的焦虑和抑郁。最引人注目的是，这些并发症在人群中的患病率和严重程度存在高度变异性，表明可能有其他因素增强或减轻其发生。我们提出，对患有单心室和 Fontan 循环的个体的最佳护理是通过综合多学科的方式提供的，要关注心脏管理之外的元素。在本报告中，我们分享了概念、经验和关于建立临床模式的观点——“Fontan 康复、健康和复原力发展”或 FORWARD 计划。我们深入了解了我们多学科护理模式的机制，以及为我们不断增长的 Fontan 循环患者及其家庭提供的服务所带来的益处。
