University Hospitals Leuven, Congenital and Structural Cardiology, Catholic University of Leuven, Leuven, Belgium.
The Helen B. Taussig Heart Center, Johns Hopkins University School of Medicine, Baltimore, Maryland.
JAMA Cardiol. 2020 May 1;5(5):590-597. doi: 10.1001/jamacardio.2019.5459.
The Fontan circulation, accomplished by direct surgical connection of the vena cavae to the pulmonary arteries, can be an effective palliation for patients with a single ventricle. However, failure of the Fontan circulation can result from mechanical obstruction, cardiac arrhythmias, increasing pulmonary vascular resistance, or deteriorating ventricular performance. Although systolic ventricular failure can usually be identified by a combination of clinical signs, symptoms, and imaging findings, diastolic ventricular dysfunction is likely an underrecognized cause of Fontan failure. Methods for detection of impaired diastolic function in a single ventricle are evolving, and established techniques appropriate in the biventricular heart lack validation in single ventricle. Association of biomarkers, cardiac magnetic resonance, and echocardiographic findings with invasively acquired pressure-volume loop data in humans may offer a way forward to accurate, noninvasive diagnosis. Today, therapy for severe diastolic ventricular dysfunction in the Fontan circulation is often disappointing and may require consideration of a ventricular assist device or even cardiac transplant. Progress toward improved outcomes of the Fontan palliation likely depends on successful innovation in primary prevention strategies and the development of more effective pharmacotherapy.
腔静脉到肺动脉的直接外科连接完成的法洛四联症循环，可以为单心室患者提供有效的姑息治疗。然而，法洛四联症循环的衰竭可能是由于机械阻塞、心律失常、肺血管阻力增加或心室功能恶化引起的。尽管收缩性心室衰竭通常可以通过临床症状、体征和影像学发现的组合来识别，但舒张性心室功能障碍可能是法洛四联症衰竭的一个未被认识到的原因。检测单心室舒张功能障碍的方法正在不断发展，而在双心室心脏中适用的既定技术尚未在单心室中得到验证。生物标志物、心脏磁共振和超声心动图检查与人体压力-容积环数据的侵入性获取之间的关联，可能为准确的非侵入性诊断提供了一种方法。如今，法洛四联症循环中严重舒张性心室功能障碍的治疗往往令人失望，可能需要考虑使用心室辅助装置甚至心脏移植。法洛四联症姑息治疗的进展可能取决于初级预防策略的成功创新和更有效的药物治疗的发展。
