Fontan-Associated Liver Disease: Current status and future directions

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Background

Fontan procedure to treat congenital univentricular heart lesions was firstly proposed by the French surgeon Francis Fontan in 1971. Since then, the Fontan procedure has allowed thousands of congenital heart patients, previously inoperable, to survive adulthood. Unfortunately, as the same surgeon who proposed the procedure pointed out, Fontan physiology is an ongoing disease, and several associated problems have dramatically appeared in the last decade, with an increasing number of Fontan adult patients. In the Fontan circulation, the abdominal organs suffer from the high venous pressure necessary to cross the pulmonary vascular bed, without the aid of a pumping chamber. The liver is the organ suffering the most. Liver enlargement, with increased stiffness, and progressive fibrosis are present in all the long Fontan survivors. In an unknown percentage, liver cirrhosis and hepatocellular carcinoma will eventually develop.

Liver surveillance is now globally included in the routine follow-up of these patients in all the centers treating adult congenital heart disease. However, the instruments we have at our disposal, validated for the follow-up of the liver infective disease, are not validated also in this population of patients with chronic stasis and venous hypertension. So far, we don’t know what markers are predicting the fatal evolution in cirrhosis or hepatocarcinoma. Furthermore, the non-invasive tests at our disposal, hematological and imaging, cannot differentiate between mild and severe liver fibrosis. The only diagnostic test seems to be the liver biopsy, which is intrinsically invasive. We should be able to understand what the no-return point is and propose to these patients a heart transplant before irreversible liver damage or hepatocellular carcinoma develops.

The congenital cardiac community should develop recommendations on how to follow most accurately the cardiac functions of the univentricular heart patients since birth, and prevent -better than treat- any functional or anatomical dysfunction or anomaly of the pulmonary circulation and the single ventricle. A low-pressure Fontan circuit, as low as reasonably achievable, should create less damage to the abdominal organs. A liver-respecting lifestyle should be taught to the mothers of the future Fontan patients, and all the Fontan adolescents and adults. On the other hand, it is important to create shared knowledge with hepatologists on how to diagnose, treat, and follow this special group of patients who present with specific liver disease, cardiac cirrhosis, and whose existence in the past was denied, or in case reserved to rare cases of old patients with long-lasting heart failure.

This collection of articles aims to shed light on all the aspects of the pathogenesis, epidemiology, prevention, clinical and instrumental manifestation, and medical and surgical treatment of Fontan-Associated Liver Disease. To put together all the pediatric and congenital cardiologists and hepatologists, asking for contribute with their experience to the knowledge in the topic can be a way to find a possible solution, or, at least, to understand the evolution of the liver damage and to anticipate the no-return point.

Keywords: single ventricle, Fontan intervention, Fontan physiology, liver dysfunction, liver cirrhosis, hepatocarcinoma

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