Noninvasive diagnosis of liver fibrosis in the complex cardiac malformation survivors – a review of the literature

Abstract

The aim of this review is to summarize the information on the pathogenesis and diagnosis of congestive liver disease sec-ondary to the Fontan and Glenn surgery for complex cardiac malformations, focusing on non-invasive diagnostic modalities. We performed an electronic database search (Pubmed, Web of Science) with the data range from 2001 to 2020. We selected the studies that addressed the pathogenesis of congestive liver disease secondary to cardiac malformations and articles regarding noninvasive methods of determining liver fibrosis in this group. We found that conventional imaging methods do not allow the detection of the initial stages of liver fibrosis. Elastography results are altered by congestion and cut-off values are not yet validated. More studies are required in order to provide evidence-based guidelines regarding the non-invasive diagnosis of hepatic fibrosis secondary to congenital heart disease. Patients with congenital cardiac malformations require close monitoring and early diagnosis of liver complications to allow prompt therapeutic intervention.

Liver Disease Assessment in Children with Fontan and Glenn Surgeries for Univentricular Hearts—The Role of Elastography and Biochemical Fibrosis Markers

“…Liver damage secondary to Fontan surgery ranges from mild fibrosis to cirrhosis [5], but isolated cases of hepatocellular carcinoma have also been reported in adolescents and young adults [6]. Liver disease in patients with Fontan surgery occurs secondary to increased central venous pressure due to hemodynamic changes, decreased cardiac output and preoperative injuries (medication, hypoxia, perioperative injuries during systemic-pulmonary shunt or Glenn surgery) [7,8]. Liver biopsy is the gold standard for diagnosing fibrosis in many pathologies.…”