The paradox of choice in the surgical management of congenitally corrected transposition: what should we do with all of these options supported by little evidence?
Bibliographic record
Abstract
Despite the conceptually proper physiology consisting of independent in-series pulmonary and systemic circuits, the prognosis in patients with unoperated congenitally corrected transposition of the great arteries (ccTGA) is reserved, even in the absence of associated defects. This is attributable to several factors. Although reports have described unoperated patients with normal or near-normal systemic right ventricular (RV) function in their 60s and beyond (1,2), it is the exception rather than the rule. Faced with a systemic circulation, the morphologic RV fails in one third of patients with no associated lesions by the fifth decade of life, and in two thirds with prior surgery for concomitant defects by 45 years of age (3). When confronted with chronic pressure overload, the systemic RV invariably undergoes hypertrophic remodelling. Owing to its limited coronary reserve from the concordant predominantly single right coronary circulation, myocardial oxygen/supply mismatch can promote subendocardial ischemia, progressive fibrosis, and ultimately, overt heart failure (4-6). Second, the tricuspid valve, again part of the systemic circulation, frequently displays Ebsteinoid features with propensity for regurgitation. While the direction of the causal relationship between tricuspid insufficiency and RV dysfunction remains debated, RV failure often follows systemic atrioventricular (AV) regurgitation (7,8). Incompetence of the tricuspid valve correlates with excess mortality, with poorer outcomes following AV valve replacement when the pre-operative RV ejection fraction is ≤40% (9). In addition, the AV conduction system is displaced and susceptible to AV block (10,11). Longterm studies report a prevalence of complete AV block of 24–39% (3,12), with an annual incidence estimated to be approximately 2% per year (13). Pacemakers are, therefore, frequently indicated. In the absence of cardiac resynchronization therapy, pacing of the subpulmonary left ventricle can alter the ventricular depolarization pattern thereby precipitating systemic AV valve regurgitation and heart failure (14). This confluence of factors contributes to the risk for ventricular arrhythmias and sudden death (15).
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.000 | 0.001 |
| Science and technology studies | 0.000 | 0.001 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.001 |
| Insufficient payload (model declined to judge) | 0.000 | 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 teacher head, 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".