Persistent Left Superior Vena Cava: Does it Have a Role in the Pathogenesis of Hypoplastic Left Heart Syndrome?
Bibliographic record
Abstract
The coexistence of a persistent left superior vena cava (PLSVC) and congenital anomalies, both cardiac and noncardiac, is well documented, but whether PLSVC contributes to the development of cardiac malformations is controversial. We conducted a retrospective review of perinatal and pediatric autopsies to determine the association between PLSVC and other congenital anomalies. Of 362 patients, 91 (25%) had congenital heart disease and 19 (5.2%) had PLSVC. Eight cases (47%) were associated with specific syndromes, including heterotaxy syndrome, trisomy 18, trisomy 13, and Jacobsen syndrome. Seventeen cases of PLSVC (89%) were associated with congenital heart disease, most of which were complex. Isolated PLSVC was found in 2 cases (11%). Eight of the 19 PLSVC cases (47%) were associated with hypoplastic left heart syndrome (HLHS), a result that was statistically significant (P = 0.041). Left ventricle inflow/outflow obstruction is believed to be a critical pathogenic factor in the development of HLHS. Whereas 5 of 8 cases of HLHS had additional obstructive cardiac outflow tract lesions, 3 of 8 cases did not. PLSVC is known to be able to compromise left ventricle inflow via a dilated coronary sinus, and we speculate that PLSVC may have played a contributing role in the pathogenesis of HLHS in these three cases. As an isolated lesion, PLSVC would not be sufficient to cause HLHS, but it might contribute in combination with other obstructive lesions, or in the setting of other genetic and/or environmental factors still to be defined for HLHS. A larger series will be needed to confirm this hypothesis.
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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) | 0.001 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.000 | 0.002 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.000 |
| Research integrity | 0.002 | 0.000 |
| Insufficient payload (model declined to judge) | 0.002 | 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".