Seminars in thoracic and cardiovascular surgery | 2021
Surveillance of Fontan associated liver disease in childhood and adolescence.
Abstract
Fontan associated liver disease (FALD) has been recognized as a potentially serious sequela of the Fontan circulation. Prevalence of FALD among different age groups and risk factors for advanced changes were assessed. FALD screening included abdominal ultrasound and laboratory tests. A liver disease score (LDS) incorporating items from ultrasound and blood testing was calculated to grade FALD severity (5 items each, maximum score 10 points). 240 patients (male: n=139, female: n=101, systemic right ventricle: n=160) underwent FALD screening 10 (IQR 7-15) years after Fontan surgery. Ultrasound was abnormal in 184 (76.6%) patients (surface nodularity / blunted liver edge: n=133, 55.4%; heterogeneous parenchyma: n=93, 38.8%; splenomegaly: n=68, 28.3%; ascites: n=23, 9.6%). At least one abnormal laboratory test was detected in 218 (90.8%) patients. Gamma-glutamyl-transpeptidase was elevated in the majority of patients (n=206, 85.8%). Median LDS was 3 (2-4). Scores ≥5 were observed in 32 (13.3%) patients. Longer follow-up (15 (11-20) vs. 9 (6-14) years, p<0.001), higher central venous (13 (11-15) vs. 10 (9-12) mmHg, p<0.001) and end-diastolic pressure (8 (5-10) vs. 6 (5-7) mmHg, p=0.001), impaired ventricular function and absence of sinus rhythm were associated with LDS ≥5. Longer follow-up (OR 1.2 (1.1-1.3), p<0.001) and higher central venous pressure (OR 1.6 (1.3-2.1), p<0.001) were the only independent predictors of advanced FALD. Abdominal ultrasound and laboratory abnormalities suggestive of FALD are common during routine follow-up already in childhood and adolescence irrespective of ventricular morphology. More advanced findings are associated with longer follow-up and higher central venous pressure.