Hepatic pathology in patients after Fontan operation

Fontan‐associated liver disease and total cavopulmonary anatomical flow effectors

“…1,2 Such effectors may include, but are not limited to, Fontan connection compliance, Fontan fenestrations and venovenous collaterals, disturbances at Fontan connection junctions, compliance of the large pulmonary arteries and vascular obstructions, pulmonary arterial microvascular cross-sectional area, pulmonary venous vascular compliance and obstructions, and functional univentricular diastolic performance. [13][14][15][16][26][27][28][29][30][31][32][33][34][35][36] In particular, a neonatal history of obstructed pulmonary blood, chiefly when accompanied by a functional univentricle of right-right ventricular type, appears to especially negatively affect the outcome of FALD. 14 In conclusion, in a relatively young, stable extracardiac Fontan patient cohort, average composite anatomical risk scores strongly correlated with average hepatic total fibrosis scores by anatomical group.…”

“…Total cavopulmonary passive flow is subject to multiple macro‐ and micro‐anatomical effectors over its circuit from the systemic and portal venous outflows to the functional univentricle 1,2 . Such effectors may include, but are not limited to, Fontan connection compliance, Fontan fenestrations and venovenous collaterals, disturbances at Fontan connection junctions, compliance of the large pulmonary arteries and vascular obstructions, pulmonary arterial microvascular cross‐sectional area, pulmonary venous vascular compliance and obstructions, and functional univentricular diastolic performance 13–16,26–36 . In particular, a neonatal history of obstructed pulmonary blood, chiefly when accompanied by a functional univentricle of right–right ventricular type, appears to especially negatively affect the outcome of FALD 14 .…”

“…12 Patients were assigned to anatomical groups (with a minimum n ≥ 3) by functional univentricle type, presence or absence of venovenous collaterals and pulmonary artery stents, and neonatal history of obstructed or unobstructed pulmonary blood flow. Based on previous investigations, [13][14][15][16][26][27][28][29][30][31][32][33][34][35][36] we developed a composite anatomical risk score, which resulted in the best correlation with total fibrosis scores. We assigned 0 points for functional univentricles of either right and left ventricular type without venovenous collaterals or pulmonary artery stents.…”