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      Hepatopatía tras la cirugía de Fontan Translated title: Liver disease after Fontan procedure

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          Abstract

          Resumen: La enfermedad hepática relacionada con cirugía de Fontan es la afectación estructural y funcional del hígado tras realizar una cirugía de Fontan. Como en otras hepatopatías, el curso puede ser progresivo, y abocar potencialmente a la aparición de complicaciones, algunas de ellas importantes, como cirrosis, ascitis, varices esofágicas o encefalopatía. Su origen no es inflamatorio, sino congestivo. Entre las pruebas complementarias, destacan la ecografía Doppler, la elastografía y la biopsia hepática. Su tratamiento es multidisciplinar e incluye optimizar la función cardiaca, prevención y manejo de las complicaciones hepáticas y prevención del hígado graso.

          Translated abstract

          Abstract: Liver disease related to Fontan's surgery is a structural and functional liver disease after performing the Fontan procedure. As in other liver diseases, the course can be progressive, potentially leading to the appearance of complications, such as cirrhosis, ascites, esophageal varices or encephalopathy. Its origin is not inflammatory, but congestive. Among the complementary tests, the Doppler ultrasound, elastography and liver biopsy stand out. Its treatment is multidisciplinary, and includes optimizing cardiac function, prevention and management of liver complications, and prevention of fatty liver disease.

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          Most cited references10

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          Transient elastography: a new noninvasive method for assessment of hepatic fibrosis.

          Chronic hepatitis is accompanied by progressive deposit of hepatic fibrosis, which may lead to cirrhosis. Evaluation of liver fibrosis is, thus, of great clinical interest and, up to now, has been assessed with liver biopsy. This work aims to evaluate a new noninvasive device to quantify liver fibrosis: the shear elasticity probe or fibroscan. This device is based on one-dimensional (1-D) transient elastography, a technique that uses both ultrasound (US) (5 MHz) and low-frequency (50 Hz) elastic waves, whose propagation velocity is directly related to elasticity. The intra- and interoperator reproducibility of the technique, as well as its ability to quantify liver fibrosis, were evaluated in 106 patients with chronic hepatitis C. Liver elasticity measurements were reproducible (standardized coefficient of variation: 3%), operator-independent and well correlated (partial correlation coefficient = 0.71, p /= F2) and with cirrhosis ( = F4), respectively. The Fibroscan is a noninvasive, painless, rapid and objective method to quantify liver fibrosis.
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            Late hepatic complications after Fontan operation; non-invasive markers of hepatic fibrosis and risk factors.

            To identify the prevalence, clinical characteristics, risk factors of hepatic complications after a Fontan operation. This was a cross-sectional study of 139 Fontan patients who underwent cardiac CT scans out of a total of 204 patients who had undergone the Fontan procedure between 1986 and 2003. Mean age was 19.0 ± 6.3 years and mean elapsed time since the initial Fontan operation was 11.5 ± 4.7 years. Subjects' clinical features, echocardiograms, radiological features and biochemical test results were reviewed. Various non-invasive hepatic fibrosis blood markers were also evaluated. Fifty-seven patients had hepatic complications, including radiological features of liver cirrhosis (25.9%), thrombocytopenia (7.2%), hyperbilirubinaemia (20.9%) and hepatic masses (2.9%). Hepatic complications were also significantly associated with ventricular dysfunction (p=0.020), absence of fenestration (p=0.004), thrombus in the Fontan tract (p=0.027), sinus node dysfunction (p=0.034) and tachyarrhythmia (p<0.001). In a multivariate analysis, the elapsed time since the initial Fontan operation was the only measure that was correlated with hepatic complications; the odds ratio of the post-Fontan duration of 16-20 years to that of 0-5 years was 9.00 (CI 2.24 to 36.17). The non-invasive hepatic fibrosis blood marker (Forns index) was also correlated with the elapsed time since the initial Fontan operation (r=0.718, p<0.001). Late hepatic dysfunction and cirrhotic change were often seen in Fontan patients. Moreover, hepatic complications were correlated with the duration of Fontan circulation. Therefore, after a Fontan operation, regular evaluation of the hepatic condition is required--for which some non-invasive hepatic fibrosis markers can be effectively used.
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              Congenital heart disease and the liver.

              There are approximately 1 million adult patients with congenital heart disease (CHD) in the United States, and the number is increasing. Hepatic complications are common and may occur secondary to persistent chronic passive venous congestion or decreased cardiac output resulting from the underlying cardiac disease or as a result of palliative cardiac surgery; transfusion or drug-related hepatitis may also occur. The unique physiology of Fontan circulation is particularly prone to the development of hepatic complications and is, in part, related to the duration of the Fontan procedure. Liver biochemical test abnormalities may be related to cardiac failure, resulting from intrinsic liver disease, secondary to palliative interventions, or drug related. Complications of portal hypertension and, rarely, hepatocellular carcinoma (HCC) may also occur. Abnormalities such as hypervascular nodules are often observed; in the presence of cirrhosis, surveillance for HCC is necessary. Judicious perioperative support is required when cardiac surgery is performed in patients with advanced hepatic disease. Traditional models for liver disease staging may not fully capture the severity of disease in patients with CHD. The effectiveness or safety of isolated liver transplantation in patients with significant CHD is limited in adults; combined heart-liver transplantation may be required in those with decompensated liver disease or HCC, but experience is limited in the presence of significant CHD. The long-term sequelae of many reparative cardiac surgical procedures are not yet fully realized; understanding the unique and diverse hepatic associations and the role for early cardiac transplantation in this population is critical. Because this population continues to grow and age, consideration should be given to developing consensus guidelines for a multidisciplinary approach to optimize management of this vulnerable population. Copyright © 2012 American Association for the Study of Liver Diseases.
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                Author and article information

                Journal
                pap
                Pediatría Atención Primaria
                Rev Pediatr Aten Primaria
                Asociación Española de Pediatría de Atención Primaria (Madrid, Madrid, Spain )
                1139-7632
                June 2020
                : 22
                : 86
                : 185-188
                Affiliations
                [2] Madrid orgnameHospital Universitario 12 de Octubre orgdiv1Servicio de Radiodiagnóstico orgdiv2Sección de Radiología Infantil España
                [1] Madrid orgnameHospital Universitario 12 de Octubre orgdiv1Servicio de Pediatría orgdiv2Sección de Gastroenterología, Hepatología y Nutrición Pediátrica España
                Article
                S1139-76322020000300013 S1139-7632(20)02208600013
                b64bc7fe-f2d1-468c-841a-49c1ee96112a

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

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                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 10, Pages: 4
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                SciELO Spain

                Categories
                Casos clínicos en Digestivo

                Liver,Cardiac surgery,Fontan,Cirugía cardiaca,Hígado
                Liver, Cardiac surgery, Fontan, Cirugía cardiaca, Hígado

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