Hematologic, neurologic, renal, and rheumatic complications in patients with cyanotic
congenital heart disease are well known. However, the effects of this condition on the liver are poorly described. Between April 2005 and April 2010, 25 adults with cyanotic
congenital heart disease were studied to determine clinical history, liver ultrasonographic data, and liver histological presentation. Twenty-five patients, with a median age of 28.7 ± 8.3 years and a basal tissue
hemoglobin oxygen saturation of 83.3% ± 6.8%, were studied. Liver ultrasonographic examination showed abnormalities in 10 of 20 patients (50%): 6 patients (30%) had
hepatomegaly, 2 patients (10%) heterogeneous parenchyma echo pattern, and 2 patients (10%) increased portal echogenicity.
Ascites was found in 7 patients (28%): 4 patients had refractory
ascites and 3 patients
anasarca. Patients with
anasarca responded well to oral and intravenous
furosemide, but those with isolated
ascites did not. No data to indicate severe
ventricular dysfunction or severe valve regurgitation were seen. In patients with refractory
ascites who had therapeutic paracentesis, serum-
ascites albumin gradient in
ascites was greater than 1.1 g/dL. No significant association was found between patients with or without
ascites when laboratory data and New York Heart Association functional class were compared. Liver biopsy was performed in 6 patients (24%). The most remarkable liver histological finding, in those with refractory
ascites, was the existence of periportal
fibrosis associated with sinusoidal dilatation. Periportal
liver fibrosis associated with
congestive heart failure,
sepsis, or a long-term
Fontan procedure can trigger refractory
ascites formation.