John Libbey Eurotext

Hépato-Gastro & Oncologie Digestive


Hepatopathy associated with the total cavo-pulmonary connection (Fontan intervention) Volume 26, issue 6, Juin 2019


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1 CHRU Hôpitaux de Tours, Hôpital Trousseau, Service d’hépato-gastro entérologie, Avenue de la République, 37170 Chambray-lès-Tours
2 CHRU Hôpitaux de Tours, Service d’anatomopathologie
3 CHRU Hôpitaux de Tours, Service d’imagerie
4 CHRU Hôpitaux de Tours, Service de cardiologie pédiatrique et congénitale adulte
5 Université François Rabelais, Tours
* Correspondance

The total cavo-pulmonary connection (TCPC) has been proposed since the early 1970s for the treatment of single-ventricle congenital heart disease. This procedure consists in anastomosing during the first years of life successively the superior vena cava and then the inferior vena cava to the pulmonary arteries. It allowed a frank improvement of the cardiac prognosis, however it induces an increase of the central venous pressure which is associated with various organ dysfunctions. Hepatic complications related to TCPC have been described for about fifteen years and have been grouped under the acronym “FALD” for Fontan Associated Liver Disease. The hemodynamic changes and more particularly the venous hyperpressure secondary to the surgical assembly induce a dilatation then a peri-sinusoidal fibrosis more or less marked. It can extend to the portal zone and evolve towards an authentic “cardiac” cirrhosis. Currently there is no non-invasive way of accurately assessing the degree of liver fibrosis so that biopsy is most often required. The clinical impact of the FALD is difficult to assess with sometimes late and severe complications. Thus, several cases of hepatocellular carcinoma (HCC) have been reported in the second decade after the intervention. No specific follow-up and no treatment is currently recommended to decrease the risk of FALD. The creation of large cohorts is therefore necessary in order to optimize the care of these young patients.

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