Belahnech Yassin, Martí Aguasca Gerard, Dos Subirà Laura
Adult Congenital Heart Disease Unit, Cardiology Department, Vall d'Hebron University Hospital, Vall d'Hebron Research Institute, Universitat Autònoma de Barcelona, 08035 Barcelona, Spain.
Centro de Investigación Biomédica en Red en Enfermedades Cardiovasculares (CIBERCV), Instituto de Salud Carlos III, 28029 Madrid, Spain.
J Clin Med. 2024 Aug 7;13(16):4633. doi: 10.3390/jcm13164633.
Over the past five decades, the Fontan procedure has been developed to improve the life expectancy of patients with congenital heart defects characterized by a functionally single ventricle. The Fontan circulation aims at redirecting systemic venous return to the pulmonary circulation in the absence of an impelling subpulmonary ventricle, which makes this physiology quite fragile and leads to several long-term complications. Despite the importance of hemodynamic assessment through cardiac catheterization in the management and follow-up of these patients, a thorough understanding of the ultimate functioning of this type of circulation is lacking, and the interpretation of the hemodynamic data is often complex. In recent years, new tools such as combined catheterization with cardiopulmonary exercise testing have been incorporated to improve the understanding of the hemodynamic profile of these patients. Furthermore, extensive percutaneous treatment options have been developed, addressing issues ranging from obstructive problems in Fontan pathway and acquired shunts through compensatory collaterals to the percutaneous treatment of lymphatic circulation disorders and transcatheter edge-to-edge repair of atrioventricular valves. The aim of this review is to detail the various tools used in cardiac catheterization for patients with Fontan circulation, analyze different percutaneous treatment strategies, and discuss the latest advancements in this field.
在过去的五十年里，Fontan手术不断发展，以提高患有功能性单心室先天性心脏缺陷患者的预期寿命。Fontan循环旨在在没有推动性肺下心室的情况下，将体静脉回流重新导向肺循环，这使得这种生理状态相当脆弱，并导致多种长期并发症。尽管通过心导管检查进行血流动力学评估在这些患者的管理和随访中很重要，但对这类循环的最终功能仍缺乏全面了解，且血流动力学数据的解读往往很复杂。近年来，诸如联合心导管检查与心肺运动试验等新工具已被采用，以增进对这些患者血流动力学特征的理解。此外，还开发了广泛的经皮治疗方案，涉及从Fontan通路的梗阻问题、通过代偿性侧支形成的获得性分流，到淋巴循环障碍的经皮治疗以及房室瓣的经导管边缘对边缘修复等各种问题。本综述的目的是详细介绍用于Fontan循环患者心导管检查的各种工具，分析不同的经皮治疗策略，并讨论该领域的最新进展。
