Liver Cirrhosis After the Fontan Procedure: Impact of Atrioventricular Valve Failure |
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Institution: | 2. DZHK (German Center for Cardiovascular Research), Partner Site Hamburg/Kiel/Lübeck, Germany;3. Department of Radiology and Neuroradiology, University Hospital Schleswig-Holstein, Campus Kiel;4. Department of Cardiovascular Surgery, University Hospital Schleswig-Holstein, Campus Kiel;1. Department of Pediatric Cardiology, Charité - Universitätsmedizin Berlin, Germany;2. Department of Congenital Heart Disease/Pediatric Cardiology, German Heart Center Berlin, Germany;3. Department of Congenital Heart Surgery, German Heart Center Berlin, Germany;4. DZHK (German Center for Cardiovascular Research), Partner Site Berlin, Germany |
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Abstract: | BackgroundLiver cirrhosis is now well recognized as a potential complication after the Fontan procedure, although associated risk factors and optimal timing of liver screening remain unclear.MethodsAll patients who underwent an extracardiac conduit Fontan procedure at The Royal Children’s Hospital, Melbourne, were identified using the Australia and New Zealand Fontan Registry. Cirrhosis was diagnosed based on liver biopsy, or a combination of imaging findings and clinical evaluation by a hepatologist.ResultsBetween 1997 and 2020, 398 patients underwent an extracardiac conduit Fontan procedure at our center, and 276 had ongoing follow-up in Victoria. Ninety-five patients (34%) underwent liver assessment at a mean age of 18.2 ± 6.7 years (11.8 ± 5.5 years post-Fontan). Fifteen patients (16%) were diagnosed with cirrhosis at a mean age of 22.7 ± 5.9 years (14.0 ± 5.2 years post-Fontan). The need for prior or concomitant atrioventricular valve repair or replacement was associated with an increased risk of cirrhosis (univariable hazard ratio HR] 7.09, 95% confidence interval CI] 2.13-23.61, P = .001). By multivariable analysis, factors associated with development of cirrhosis were atrioventricular valve failure prior to Fontan (HR 3.27, 95% CI 1.15-9.31, P = .026) and older age at Fontan operation (HR 1.13 per year increase, 95% CI 1.01-1.26, P = .034). The proportion of patients alive, nontransplanted, and without cirrhosis at 10, 15, and 20 years was 93.4% (95% CI 88.4%-98.7%), 79.6% (95% CI 69.7%-90.8%), and 64.6% (95% CI 51.0%-81.9%), respectively.ConclusionsEarly commencement of liver screening should be considered for patients with a history of atrioventricular failure during Fontan palliation. |
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Keywords: | AVV"} {"#name":"keyword" "$":{"id":"kwrd0015"} "$$":[{"#name":"text" "_":"atrioventricular valve AVVR"} {"#name":"keyword" "$":{"id":"kwrd0025"} "$$":[{"#name":"text" "_":"atrioventricular valve regurgitation CI"} {"#name":"keyword" "$":{"id":"kwrd0035"} "$$":[{"#name":"text" "_":"confidence interval ECC"} {"#name":"keyword" "$":{"id":"kwrd0045"} "$$":[{"#name":"text" "_":"extra-cardiac conduit FALD"} {"#name":"keyword" "$":{"id":"kwrd0055"} "$$":[{"#name":"text" "_":"Fontan-associated liver disease HCC"} {"#name":"keyword" "$":{"id":"kwrd0065"} "$$":[{"#name":"text" "_":"hepatocellular carcinoma HR"} {"#name":"keyword" "$":{"id":"kwrd0075"} "$$":[{"#name":"text" "_":"hazard ratio MELD-XI"} {"#name":"keyword" "$":{"id":"kwrd0085"} "$$":[{"#name":"text" "_":"model for end-stage liver disease excluding international normalised ratio |
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