Fontan‐Associated Liver Disease: Trends Over Time and Relationship With Outcomes
Bibliographic record
Abstract
Background Fontan associated liver disease is a poorly understood complication of long‐term single ventricle palliation. This study sought to describe serial liver function changes in patients with a Fontan circulation from 2014 to 2020 and determine if these findings can be used to predict adverse clinical outcomes. Methods We conducted a retrospective chart review of Fontan patients age≥18 who were followed by a specialist clinic between 2014 and 2020. Annual bloodwork, including liver enzymes and AFP (alpha‐fetoprotein), and liver imaging were analyzed. Common liver function scores were calculated. Trends over time and their correlation to clinical events were assessed. Events were defined as congestive heart failure, cardiac hospitalization, thrombosis, protein losing enteropathy, and death. The study assessed possible associations with the composite event of adverse outcomes. Results One hundred twelve Fontan patients were included with a mean age of 24.9±8.1 years. Age‐adjusted AFP and creatinine increased over the study period, whereas alkaline phosphatase decreased. There was no significant change in other liver biomarkers over the study period. The proportion of patients with higher liver imaging scores increased over the study period. Over a follow‐up time of 6.1 years (95% control limit, 5.5–6.9 years), 44 (39.3%) patients experienced at least 1 event, most commonly cardiac hospitalization. In multivariable analysis, age was associated with a greater risk of events (hazard ratio, 1.035 [95% CI, 1.008–1.063]; P =0.010); no other biomarkers or scores were significant. Conclusions Routine liver biomarkers do not increase over time despite increasing proportion of patients with higher liver imaging scores over the same time. Although our sample size was limited, current modalities to assess progression of liver dysfunction on bloodwork and imaging were not related to clinical outcomes.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.003 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.002 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.001 |
| Insufficient payload (model declined to judge) | 0.001 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".