Impact of Cardiac Position, Morphology and Operative Technique on Long-Term Fontan Outcomes in Heterotaxy
Bibliographic record
Abstract
Abstract Objectives Heterotaxy is characterized by complex venous connections and cardiac position requiring individualized strategies for Fontan. This study aimed to assess 25-year outcomes of heterotaxy patients undergone Fontan operation, with particular focus on morphological features and surgical techniques. Methods 82 consecutive heterotaxy patients who underwent Fontan operation from 1985 to 2021 were compared to 150 with tricuspid atresia (TA) and 144 with hypoplastic left heart syndrome (HLHS). Kaplan-Meier method and Cox proportional hazard model were used for transplant-free survival and predictor analysis. Results Right Atrial Isomerism (RAI) was present in 45 cases and Left Atrial Isomerism (LAI) in 37. The 20-year transplant-free survival was comparable between the groups (RAI vs. LAI, 76% [95% confidence interval, 57-87%] vs. 68% [47-82%], p=0.22). Techniques were extracardiac in 66%, intra-atrial conduit in 9%, lateral tunnel in 18%. More intervention on pulmonary veins occurred in the RAI group. Cardiac position and apicocaval juxtaposition did not influence outcome. The IVC-contralateral PA Fontan was associated with 100% survival, while the IVC-ipsilateral PA Fontan at the side of the cardiac apex showed 67% [34-87%] survival at 20 years. Moderate or severe ventricular dysfunction at 20 years was found in 15% (6-39%) and 0% of RAI and LAI patients among hospital survivors, respectively (p=0.09). In-hospital mortality was higher in heterotaxy (9.8% [5-19%]) compared to TA (1.3% [0.3-5.3%], p<.01) and HLHS (2.8% [1.1-7.3%], p=0.02). There was no in-hospital death after 2000 in any of groups. The 20-year transplant-free survival in heterotaxy (72% [59-82%]) was worse than that in TA (95% [89-97%], p<0.001) but was not significantly different from that in HLHS (80% [69-87%], p=0.11). Reintervention rate at 20 years was significantly higher in heterotaxy (18% [11-29%]) vs. HLHS (8% [4-16%]) (p=0.01). Ventricular dysfunction was a predictor for death (univariate p<0.001). Conclusion Various routing techniques can be successfully applied to overcome the anatomical challenges of heterotaxy. Although higher in-hospital mortality and reintervention rate were found in heterotaxy in the early era, overall survival post Fontan was similar to that in HLHS. RAI had comparable survival to LAI with greater proportion requiring pulmonary vein intervention at time of Fontan.
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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 | 0.000 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| 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".