Abstract 14502: Contemporary Practices and Outcomes in Primary Pulmonary Vein Stenosis: A PVS Network Study
Bibliographic record
Abstract
Background and Study Aim: Pediatric pulmonary vein stenosis (PVS) remains a disease associated with high mortality, with studies primarily limited to single center reports. Using the PVS Network Registry, we report on contemporary practices and outcomes for a large cohort of children with primary PVS across North America. Methods: Children with a diagnosis of primary PVS (congenital, normal pulmonary venous connections) in the retrospective PVS Network Registry (2009-2019, n=600, 19 institutions) were analyzed using descriptive statistics, survival analysis and Cox regression analysis. Results: In the PVS Network Registry cohort, 393 patients had a diagnosis of primary PVS. In the primary PVS cohort, 199 (50%) were male, 105 (27%) had a genetic diagnosis and 192 (49%) were premature (median 29 (22-37) weeks). Medical therapies were used in 53(14%) patients, with Imatinib being most common (58%). Pulmonary hypertension medications were used in 182 (46%) patients, with sildenafil most reported (90%). Surgical and catheter-based interventions for PVS were performed in 334 (85%) patients. 203 (60%) patients had at least one PVS surgery and 231(71%) had at least one PVS catheter intervention. At the time of first procedure for PVS, 133 (39%) had bilateral disease, while 100 (30%) had ≥3 pulmonary veins involved. The median age was 0.54 years (IQR 0.91). Catheter based interventions were more common on the left PVs (60%). Repeat procedures were common (53%) with the majority being catheter reinterventions (78%), with an average of 3.24 (±2.75) catheter procedures per patient. The overall survival for children with primary PVS was 76% and 71% at 1 and 2 years. In children with ≥4 veins involved, survival was 59% and 52% at 1 and 2 years. Older age at intervention was protective (HR 0.66, P<0.02), while ≥3 vein involvement was associated with poor outcome (HR 1.8, p=0.05). Conclusion: This is the largest primary PVS cohort reported and demonstrates overall 1 year survival of 76%, however, children with multivessel disease remain at high risk. Reinterventions are common, with many undergoing repeat catheterization procedures in our cohort. The PVS Network retrospective cohort provides insights into clinical practices and will provide benchmarking for future studies.
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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 | 0.004 |
| 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 | 0.001 | 0.001 |
| Research integrity | 0.000 | 0.001 |
| 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".