Congenital heart disease-associated liver disease: a narrative review

Abstract

Congenital heart diseases (CHD) can be associated with liver dysfunction. The cause for liver impairment can result out of a wide spectrum of different causes, including liver congestion, hypoxemia or low cardiac output. Fortunately, most CHD show a good long-term outcome from a cardiac perspective, but great attention should be paid on non-cardiac health problems that develop frequently in patients suffering from CHD. The treatment of liver dysfunction in CHD requires a close multidisciplinary management in a vulnerable patient collective. Unfortunately, structured recommendations on the management of liver dysfunction in patients with CHD are scarce. The objective of this review is to provide insights on the pathophysiology and etiologies of liver dysfunction as one of the most relevant non-cardiac problems related to CHD. Furthermore, we advise here on the management of liver disease in CHD with special attention on assessment of liver dysfunction, management of portal hypertension as well as on surveillance and management of hepatocellular carcinoma (HCC). A multidisciplinary perspective may help to optimize morbidity and mortality in the long-term course in these patients. However, as evidence is low in many aspects, we encourage the scientific community to perform prospective studies to gain more insights in the treatment of liver dysfunction in patients with CHD.

Keywords: Congenital heart disease (CHD); liver disease; liver fibrosis.

Figure 1

Architecture of the liver acinus and its metabolic zones. The figure illustrates a liver acinus schematically. The unique blood supply of the liver is the essential basis for understanding pathophysiology of liver diseases in CHD. The blood supply enters the liver by the hepatic artery and the portal venous system. Thereby, the blood passes the capillary sinusoidal system of the liver lobules and enters the central vein, which in turn drains the blood to the inferior caval vein. There are three defined metabolic zones in respect to the mentioned blood supply. The pericentral zone III is thereby highly susceptible to hemodynamic alterations not only due to the fact that venous congestion distresses the central vein first but also because ischemic or hypoxic alterations predominantly affect this region as distance to oxygen and nutrient rich blood supply shows the longest distance in zone III. It is plausible when typical changes of cardiac hepatopathy such as sinusoidal dilatation, hemorrhagic necrosis and fibrosis occur predominantly in the pericentral zone III (Figure created by Florian P. Reiter).

Figure 2

Fibrosis pattern in liver congestion. The figure illustrates schematically the typical pattern of fibrosis between two central veins that can be observed in liver fibrosis related to liver congestion (Figure created by Florian P. Reiter).

Figure 3

Anatomy after atrial switch operation (according to Mustard or Senning). The figure illustrates schematically the arterial and venous blood flow after arterial switch operation (Schema modified from (11) (https://www.sciencedirect.com/science/article/pii/S1361841515001383?via%3Dihub https://creativecommons.org/licenses/by/4.0/ and modified by Florian P. Reiter).

Figure 4

Schematic illustration of Fontan procedures. The figure illustrates schematically different Fontan procedures. (A) Atriopulmonary Fontan, (B) extracardiac Fontan and (C) lateral tunnel Fontan accordingly to (35) (illustration^© Beth Croce).