Literature review for Fontan-associated liver disease: An update
Journal
Formosan Journal of Surgery
Journal Volume
56
Journal Issue
2
Date Issued
2023-03-01
Author(s)
Hsiao, Chung Chen
Abstract
Dear Editor,
The Fontan operation is a palliative surgical procedure performed in patients with a functional or anatomic single ventricle (univentricular heart). Following the Fontan procedure, patients face substantial risks of morbidity and mortality and require lifelong follow-up with cardiologists who are experienced in caring for patients with complex congenital heart disease. Fontan-associated liver disease (FALD) is a potential complication in patients who have undergone the procedure and is mainly caused by liver congestion. Increased T2 signal intensity and decreased T1 signal intensity in magnetic resonance imaging are characteristic of FALD.[1] Liver tumors, especially hepatocellular carcinoma (HCC), can develop. It is challenging to differentiate HCC from benign nodules by using typical imaging characteristics because of the abnormality of the hepatic vein drainage; artery-phase hyperenhanced nodules have been reported measuring ≥1 cm in size and exhibiting washout in the portal venous phase/delayed phase.[2] Warning signs of highly suspicious HCC in imaging include large tumor size, an interval change in size or echogenicity, mass-like appearance, and nodules causing contour abnormality on the liver surface.[3] Reported risk factors significantly associated with HCC were washed out in the portal venous phase, a long time having elapsed since the Fontan procedure, large nodule size (>1 cm), and elevated α-fetoprotein.[2] Research has recommended liver biopsy for suspicious liver tumors in the absence of any contraindication. During surgical resection for HCC, the inferior vena cava, is often clamped to reduce blood loss, but this maneuver has the effect of elevating the central venous pressure level.[4] Alternatively, the reverse Trendelenburg position could be applied to reduce blood loss without inferior vena cava clamping. If laparoscopic resection is being considered, maintaining pneumoperitoneum pressure at approximately 6 to 10 mm Hg with adequate fluid infusion is recommended.[4] To achieve early diagnosis and early treatment, active surveillance is needed for patients after undergoing the Fontan procedure. Cirrhosis and HCC surveillance has been recommended for patients with FALD who have survived 10 years after the Fontan operation; surveillance should consist of imaging and consideration of other noninvasive markers, such as a modification of the Model for End-stage Liver Disease score excluding the international normalized ratio of prothrombin time, fibrosis score, α-fetoprotein, and elastography. Where transplantation is not an option, these young patients face poor quality of life and overall survival.[5] However, liver transplant alone, even in a “perfect” Fontan, is not advisable because of the difficulty in managing elevated right-side heart pressure, particularly during the anhepatic and reperfusion phases of the procedure.[5] Combined heart and liver transplants can achieve good outcomes, and the most critical consideration of anesthetic management is the maintenance of a transpulmonary pressure gradient (central venous pressure–atrial pressure) to promote pulmonary blood flow.[5] Ideally, integrated multidisciplinary management can help these patients live longer with better quality of life.
SDGs
Type
other