Pulmonary atresia with intact ventricular septum: management of, and outcomes for, a cohort of 210 consecutive patients
Bibliographic record
Abstract
OBJECTIVES: We sought to determine trends, and outcomes, for a cohort of patients with pulmonary atresia with intact ventricular septum born between 1965 and 1998. BACKGROUND: Pulmonary atresia with intact ventricular septum is a complex lesion that remains a therapeutic challenge, particularly regarding the suitability for biventricular repair. METHODS: We identified 210 consecutive patients, and reviewed their medical records, initial angiograms, and echocardiograms, along with the relevant surgical and pathology reports. RESULTS: The mean initial Z-score for the diameter of the tricuspid valve was -0.99 +/- 1.95, with Ebstein's malformation in 8%. A right ventricular dependent coronary arterial circulation was found in 23%. The proportion of patients who received treatment increased over time, although placement of an arterial shunt was the predominant initial procedure throughout the experience. At the last follow-up, 107 patients had not reached the planned final stage of their repair, and 79% of these had died. Of the 103 reaching the final stage of planned repair, 58 had undergone attempted biventricular repair, with 34% dying; 14 had undergone attempted one and a half ventricular repair, with 7% dying, and 31 had undergone attempted functionally univentricular repair, with 10% dying. Overall, survival was 57% at the age of 1 year, 48% at 5 years, and 43% at 10 years. Survival improved over time, with survival of 75% at 1 year, and 67% at 5 years, for patients born between 1992 and 1998. An earlier date of birth, the presence of Ebstein's malformation, and prematurity were all significant independent factors associated with decreased survival. A greater severity of coronary arterial abnormalities was significantly associated with a greater likelihood of left ventricular dysfunction during follow-up. CONCLUSIONS: The outcomes for patients born with pulmonary atresia with intact ventricular septum have improved over time, albeit that careful initial management, and better selection, is still indicated for those planned to undergo biventricular repair.
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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 | 0.002 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.001 | 0.000 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.000 | 0.000 |
| 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".