Objective: Fontan-associated liver disease (FALD) is widely recognised as a common complication in patients long after the Fontan operation. However, data on the predictors of FALD that can guide its screening and management are lacking. The present study aimed to identify the predictors of liver cirrhosis (LC) and hepatocellular carcinoma (HCC) in post-Fontan patients.
Methods: This was a multi-institutional retrospective cohort study. Clinical data of all perioperative survivors of Fontan operation before 2011 who underwent postoperative catheterisation were collected through a retrospective chart review.
Results: A total of 1117 patients (538 women, 48.2%) underwent their first Fontan operation at a median age of 3.4 years. Postoperative cardiac catheterisation was conducted at a median of 1.0 year. During a median follow-up period of 10.3 years, 67 patients (6.0%) died; 181 (16.2%) were diagnosed with liver fibrosis, 67 (6.0%) with LC, 54 (4.8%) with focal nodular hyperplasia and 7 (0.6%) with HCC. On multivariable analysis, high central venous pressure (CVP) (HR, 1.28 (95% CI 1.01 to 1.63) per 3 mm Hg; p=0.042) and severe atrioventricular valve regurgitation (HR, 6.02 (95% CI 1.53 to 23.77); p=0.010) at the postoperative catheterisation were identified as independent predictors of LC/HCC.
Conclusions: Patients with high CVP and/or severe atrioventricular valve regurgitation approximately 1 year after the Fontan operation are at increased risk of developing advanced liver disease in the long term. Whether therapeutic interventions to reduce CVP and atrioventricular valve regurgitation decrease the incidence of advanced liver disease requires further elucidation.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1136/heartjnl-2022-320940 | DOI Listing |
J Physiol Sci
December 2024
Department of Cardiovascular Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
Fenestration has been reported to enhance Fontan hemodynamics in several cases of Fontan circulation. However, the indication criteria for fenestration remain under discussion. To assess the effectiveness of fenestration in Fontan circulation, we conducted a theoretical analysis using a computational model of the fenestrated Fontan circulation.
View Article and Find Full Text PDFPediatr Cardiol
December 2024
Department of Cardiovascular Sciences, KU Leuven, Leuven, Belgium.
Patients with a Fontan circulation are at risk for chronic kidney disease (CKD), which is defined as persistently reduced glomerular filtration rate (GFR) <60ml/min/1.73 or elevated marker of kidney injury such as urinary albumin-to-creatinine ratio (UACR) >30mg/g. We determined the prevalence of albuminuria in patients with a Fontan circulation.
View Article and Find Full Text PDFEur Heart J Case Rep
December 2024
Department of Cardiovascular Surgery, Shandong Second Provincial General Hospital, No. 4 Duanxing West Road, Jinan 250022, China.
Diagnostics (Basel)
November 2024
Department of Pediatric Cardiac Surgery and Congenital Heart Disease, Instituto Nacional de Cardiología Ignacio Chávez, Mexico City 14080, Mexico.
Background: Fontan circulation maintains an elevated venous pressure; this promotes venous and lymphatic congestion and may lead to late circuit failure. Our objective was to determine the association between thoracic lymphatic perfusion patterns assessed by magnetic resonance imaging and late Fontan failure.
Methodology: A retrospective study was performed.
Indian J Thorac Cardiovasc Surg
January 2025
Division of Cardiothoracic and Vascular Anaesthesiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala India.
Unlabelled: Appearance of a thrombus in the graft intraoperatively during Fontan surgery is not reported and warrants careful evaluation. Our patient was an 18-year-old lady who underwent fenestrated extracardiac Fontan surgery. Intraoperatively, transesophageal echocardiography revealed an echogenic structure inside the conduit.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!