Shams Tamana, Salvador Nunes Luis, Schmidt Sabine, Gast Enora, Sempoux Christine, Moradpour Darius, Di Bernardo Stefano, Sekarski Nicole, Rutz Tobias, Ladouceur Magalie, Bouchardy-Clément Judith, Coukos Alexander, Fraga Montserrat
Service de gastroentérologie et d'hépatologie, Centre hospitalier universitaire vaudois, 1011 Lausanne.
Service de radiodiagnostic et de radiologie interventionnelle, Centre hospitalier universitaire vaudois, 1011 Lausanne.
Rev Med Suisse. 2024 Aug 28;20(884):1469-1475. doi: 10.53738/REVMED.2024.20.884.1469.
Fontan surgery is vital for infants born with a single-ventricle heart. This intervention establishes a new blood flow circuit bypassing the single ventricle, thereby the separating pulmonary and systemic circulation to preserve single ventricular function. However, it carries risks of hepatic complications, collectively termed Fontan-associated liver disease (FALD), characterized by progressive hepatic congestion and fibrosis potentially leading to an equivalent of cirrhosis. Diagnosis and staging of FALD are complex, requiring multidisciplinary management. In advanced FALD, consideration is given to heart transplantation alone or combined heart-liver transplantation, underscoring the importance of an integrated approach to optimize care for these increasingly more common patients.
对于患有单心室心脏的婴儿来说,Fontan手术至关重要。这种干预措施建立了一个绕过单心室的新血流回路,从而将肺循环和体循环分开,以维持单心室功能。然而,它存在肝脏并发症的风险,统称为Fontan相关肝病(FALD),其特征是进行性肝充血和纤维化,可能导致相当于肝硬化的情况。FALD的诊断和分期很复杂,需要多学科管理。在晚期FALD中,会考虑单独进行心脏移植或联合心脏-肝脏移植,这突出了采用综合方法为这些越来越常见的患者优化护理的重要性。