Fontan circulation and systemic disease – a retrospective cohort analysis over 35 years of follow-up
Bibliographic record
Abstract
BACKGROUND: The Fontan operation provides lifesaving palliation for individuals with single ventricle (SV) physiology. Given recent concerns of systemic disease (SD) for patients with a Fontan circulation, we sought to (1) quantify the increase in SD incidence associated with the Fontan circulation; (2) identify the risk factor of SD; (3) assess the association between SD and mortality in patients with a Fontan circulation. METHODS: A matched retrospective cohort study design was adopted. From the Quebec Congenital Heart Disease (CHD) Database with up to 35 years of follow-up, patients who survived at least 30 days after the Fontan operation were identified. For each Fontan patient, patients with isolated ventricular septal defect (VSD) with the same sex and age were identified and 20 of them were randomly selected to form the control group. The presence of SD was defined as at least 1 hospitalization due to extra-cardiac complications including liver, respiratory, gastrointestinal or renal disease. Time-to-event analysis including Kaplan-Meier curve analysis and Cox proportional hazard models were conducted to assess the cumulative risk of SD, risk factors of SD, and the association between SD and 10-year mortality. RESULTS: A total of 533 patients with Fontan circulation were identified and matched with 10,280 VSD patients. The cumulative probabilities of SD at 10- and 35-years follow-up were 59.02% and 89.66% in patients with a Fontan circulation, 4 to 7 times of the probabilities in VSD patients (8.68% and 23.34%, respectively; LogRank tests P < .0001). In Fontan patients, cardiovascular complications were associated with a 4.1-fold (95% CI: 3.52-4.88) higher risk of developing SD. Multisystem disease (>1 extra-cardiac organ affected) disease was associated with a 3.38-fold (95%CI: 1.73-6.60) increase in 10-year mortality risk when comparing to the absence of SD. CONCLUSIONS: This population-based study demonstrated that patients with a Fontan circulation had increased risk of SD, which in turn led to higher risk of mortality. These findings underscore the need for more systematic surveillance of cardiac and systemic disease for patients after Fontan operation.
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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 | 0.002 |
| Meta-epidemiology (narrow) | 0.000 | 0.001 |
| Meta-epidemiology (broad) | 0.000 | 0.001 |
| Bibliometrics | 0.001 | 0.002 |
| Science and technology studies | 0.001 | 0.000 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.000 | 0.001 |
| Research integrity | 0.001 | 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".