Fontan venovenous collaterals and hepatic fibrosis

Abstract

Objective: We hypothesized that a relationship might exist between angiographically demonstrable, post-Fontan venovenous collaterals, and hepatic fibrosis. Methods: We analyzed data from post-Fontan patients that underwent cardiac catheterization and transvenous-hepatic biopsy procedures between March 2012 and March 2020. From innominate vein angiography, we determined those that either had or lacked venovenous collaterals. Additionally, we examined data from post-Fontan patients that underwent hepatic ultrasound, shear-wave elastography between January 2017 and March 2020. Results: We identified 164 patients that met inclusion criteria. Of the 164, 101 (62%) had venovenous collaterals. Of the 101 with collaterals, average total fibrosis score (TFS) was 3.2 and the average rate of fibrosis progression was 0.28 vs an average TFS of 2.1 and an average fibrosis progression rate of 0.22 for those without collaterals (P = .00001 and P = .01, respectively). Of the 101 with collaterals, oxygen saturation was 91% ± 4% vs 93% ± 3% (P = .048) without collaterals. Of the 164, 86 (52%) underwent ultrasound shear-wave elastography. Of the 86 patients undergoing elastography, 50 (58%) were performed in those with collaterals, and 36 (42%) in those without collaterals. For the 50 with collaterals, average elastography values were 13.3 vs 11.2 kPa for the 36 without collaterals (P = .006). We found no statistically significant differences for age at biopsy, Fontan duration, Fontan-type, type of functional univentricle, laboratory, clinical, or hemodynamic values between those with or without collaterals. Conclusions: The presence of angiographically demonstrated venovenous collaterals was associated with statistically, significantly more advanced liver fibrosis than those without collaterals.

Prevalence of Venovenous Shunting and High-Output State Quantified with 4D Flow MRI in Patients with Fontan Circulation

“…From this experience, we observe that shunt lesions are very common in this population, predominantly through venovenous collaterals that decompress the passive systemic venous return, bypassing directly into the pulmonary venous system and atrium. Our results mirror the experience of catheterization studies of pediatric (28) and adult (4,29) patients with a Fontan circuit in observing a high frequency of these collaterals.…”

Fontan‐associated liver disease and total cavopulmonary anatomical flow effectors

“…Our previous works and this current study have investigated the anatomic variables that may affect the rate and degree of hepatic fibrosis. [13][14][15][16] From such variables, we have attempted to construct a useful method for assisting with routine, long-term, post-Fontan follow-up.…”

“…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.…”

“…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.…”

“…This study builds on the findings from our previous reports that demonstrated hepatic fibrosis risk associated with a neonatal history of obstructed pulmonary blood flow and a functional univentricle of right ventricular type, presence of venovenous collaterals, and the presence of pulmonary artery stents. [13][14][15][16]…”