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.
Keywords: ascites; congenital heart disease; cyanosis; portal fibrosis.