Fig 1.
Fontan procedure and Fontan-associated liver diseases.
After the Fontan procedure, the hepatic venous flow empties directly into the Fontan circuit; thus the liver is susceptible to the effects of central venous hypertension. High central venous pressure leading to chronic passive congestion occurs subsequent to the sinusoidal dilatation that follows increased intrahepatic resistance and reduced portal flow. Increased intrahepatic resistance promotes liver fibrogenesis, which manifests as Fontan-associated liver disease (e.g., cirrhosis and hepatocellular carcinoma). In addition, both increased intrahepatic resistance and reduced portal flow contribute to the development of portal hypertension. HV, hepatic vein; IVC, inferior vena cava; PA, pulmonary artery; PV, pulmonary vein; SVC, superior vena cava; GI, gastrointestinal.
Table 1.
Baseline characteristics and laboratory and CT findings in 27 patients who underwent Fontan surgery.
Fig 2.
CT and endoscopic features of gastroesophageal varices in patients who underwent a Fontan procedure.
(a, b) Abdominal dynamic CT images of a 19-year-old woman at 17 years after her Fontan procedure. (a) A portal phase image reveals a gastric varix (white arrow). (b) Three-dimensional CT image (anterior view) shows a gastric varix (blue, indicated by black arrow) draining into a gastrorenal shunt (red, indicated by black arrowheads). (c) Endoscopic image from the patient shown in Fig 2a and 2b. Esophagogastroduodenoscopy (EGD) reveals an enlarged nodular gastric varix (Lg-cf, F2, CW, RC0; indicated by dotted white circle). (d, e) Abdominal dynamic CT images of an 18-year-old man 14 years after his Fontan procedure. (d) Portal phase image reveals a gastric varix (indicated by white arrow). (e) Three-dimensional CT image (posterior view) shows a tortuous gastric varix (purple, indicated by black arrow) that drains into a gastrorenal shunt (yellow, indicated by black arrowheads). (f) Endoscopic image from the patient shown in Fig 2d and 2e. EGD reveals an enlarged nodular gastric varix (Lg-cf, F2, CW, RC0; indicated by dotted white circle). (g, h) Abdominal dynamic CT images of a 19-year-old man 13 years after a Fontan procedure. (g) Portal phase image reveals a gastric varix (indicated by white arrow) that drains into a gastrorenal shunt (indicated by black arrowheads). (h) Three-dimensional CT image (anterior view) shows a gastric varix (blue, indicated by black arrow) that drains into a gastrorenal shunt (red, indicated by black arrowheads). (i) Endoscopic image from the patient shown in Fig 2g and 2h. EGD reveals an enlarged nodular gastric fundal varix (Lg-f, F2, Cb, RC0) (indicated by dotted white oval). (j, k) Abdominal dynamic CT images of a 14-year-old boy 12 years after his Fontan procedure. The sagittal view (j) and coronal view (k) at the portal phase reveal a small esophageal varix (indicated by white arrows). (l) Endoscopic image of the patient shown in Fig 2j and 2k. EGD reveals a straight, small-caliber esophageal varix in the lower esophagus (Li, F1, Cw, RC0) (indicated by dotted white oval). GEV, gastroesophageal varix; IVC, inferior vena cava; Ki, kidney; L, left; Li, liver; R, right; Sp, spleen; St, stomach.
Table 2.
Comparison of demographic, laboratory, hemodynamic, and CT findings between Fontan patients with and without GEVs.
Table 3.
Correlations between demographic and laboratory data and the presence of GEVs.
Table 4.
Platelet count to predict the presence of gastroesophageal varices.