The Rate of Hepatic Fibrosis Progression in Patients Post-Fontan

Evans, William N.; Acherman, Ruben J.; Mayman, G. A.; Galindo, Alvaro; Rothman, Abraham; Winn, Brody J.; Yumiaco, Noel S.; Restrepo, Humberto

doi:10.1007/s00246-020-02331-0

Cited by 13 publications

(27 citation statements)

References 28 publications

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“…We also found that, similarly, ultrasound, shear-wave elastography scores were higher in those with venovenous collaterals vs those without col- past studies failed to show a statistically significant effect from a range of inferior vena caval pressures on either TFS or FPR. 17,20 Additional strengths include our robust data management and electronic health records on all diagnosed patients in Nevada, avoiding the reliance on third-party sources such as hospital discharge information or state databases. Finally, data were culled from a relatively young, stable population.…”

Section: Discussionmentioning

confidence: 99%

Fontan venovenous collaterals and hepatic fibrosis

Evans

Acherman

Mayman

et al. 2020

Journal of Cardiac Surgery

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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.

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Section: Discussionmentioning

confidence: 99%

Fontan venovenous collaterals and hepatic fibrosis

Evans

Acherman

Mayman

et al. 2020

Journal of Cardiac Surgery

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“…21 This experience led us to investigate and report statistically significant differences in hepatic FPR between those with extracardiac versus lateral tunnel Fontan connections. 3 However, despite extensive experience with hepatic FPRs in conditions such as hepatitis C, our recent report in comparing rates between patients with extracardiac versus lateral tunnel connections was, to the best of our knowledge, the first such analysis in FALD. Further, hepatitis C investigations have analyzed thousands of patients versus the small number of Fontan patients previously reported.…”

Section: Discussionmentioning

confidence: 96%

“…Our previous report on FPR showed that values were significantly higher in extracardiac Fontans versus lateral tunnel Fontans, a finding we speculated might be from compliance differences between the two connection types. 3 Reports have suggested that a functional univentricle of right ventricular type is itself a risk factor for protein-losing enteropathy and poorer long-term survival compared to functional univentricles of left ventricular type. 2,22,23 Thus, possibly a combination of reduced compliance from an extracardiac connection and a pulmonary artery stent, coupled with a right ventricular type univentricle, contributes to the significantly different hepatic fibrosis pathology associated with these anatomical variants.…”

Section: Discussionmentioning

confidence: 99%

“…Our previous FALD-related hepatic pathology publications have investigated two indexes of liver fibrosis: (1) the TFS, a sum of 0-4 scoring methods for both portal and sinusoidal fibrosis and (2) the FPR, a product of the TFS score divided by Fontan duration in years at liver biopsy. 2,3,[10][11][12] Others have also used combined scoring systems for hepatic fibrosis but by a different methodology. 8 A potential future investigation comparing these two methods may be of value.…”

Section: Discussionmentioning

confidence: 99%

“…Our previous reports suggest that the degree of hepatic fibrosis, assessed by total fibrosis scores (TFS) and fibrosis progression rates (FPRs) that were determined by liver biopsy, appears to be influenced by Fontan types and anatomical variants. 2,3 The study investigates possible associations between FALD-related hepatic fibrosis and additional anatomical variants, including previously placed pulmonary artery stents.…”

Section: Introductionmentioning

confidence: 99%

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Fontan-Associated Anatomical Variants and Hepatic Fibrosis

Evans

Acherman

Mayman

et al. 2021

World J Pediatr Congenit Heart Surg

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Objective: We hypothesized that a relationship between post-Fontan hepatic fibrosis and anatomical variants might exist. Methods: Attempting to limit confounding variables, we analyzed data from living, stable, post-extracardiac Fontan patients who underwent cardiac catheterization and transvenous hepatic biopsy procedures between March 2012 and June 2020. Results: We identified 120 patients who met the inclusion criteria. Of the 120, 35 (29%) had pulmonary artery stents. For the 35 with pulmonary artery stents, the average total fibrosis score was 3.2 ± 1.9 and the fibrosis progression rate was 0.36 ± 0.33, and for those with no pulmonary artery stents, the total fibrosis score was 2.6 ± 1.8 and the fibrosis progression rate was 0.27 ± 0.33 ( P = .13 and P = .11, respectively). Of the 120, 65 had functional univentricles of right ventricular type. Of these 65, 27 had pulmonary artery stents. For the 27 with pulmonary artery stents, the average total fibrosis score was 3.4 ± 1.8 and the average fibrosis progression rate was 0.39 ± 0.30, and for the 38 without pulmonary artery stents, the average fibrosis score was 2.3 ± 1.5 and the average fibrosis progression rate was 0.23 ± 0.21 ( P = .01 for comparison of both values). Conclusions: This study’s findings suggest that a post-extracardiac Fontan with a functional univentricle of right ventricular type plus a pulmonary artery stent may have more advanced liver pathology than those without a pulmonary artery stent at similar Fontan duration years and ages at liver biopsy.

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