Abstract 16801: Improved Transplant-Free Survival of Children With Dilated Cardiomyopathy: Analysis of Two Decades From the Pediatric Cardiomyopathy Registry
Bibliographic record
Abstract
Background: Previous studies of pediatric patients with dilated cardiomyopathy (DCM) have suggested improvement in survival has been due to utilization of heart transplantation. We sought to characterize the changing clinical characteristics and outcomes of pediatric patients with DCM phenotype over the past two decades. Methods: Longitudinal data from 1953 children diagnosed with DCM in the NHLBI Pediatric Cardiomyopathy Registry (PCMR) from 1990-2009 were divided into two cohorts based upon year of diagnosis: “Era 1” (1990-1999, n=1199) and “Era 2” (2000-2009, n=754). Clinical information at diagnosis and incidence of transplant and death without transplant were analyzed by DCM etiology and era. Competing risks methodology was used to estimate the cumulative incidence of death or transplant by era. Results: The majority of patients in both groups had idiopathic DCM (71 vs. 71%, p=0.96). Median age (1.6 vs. 1.7 yrs, p=0.45), left ventricular end diastolic Z scores (+4.2 vs. +4.2, p=0.84) and fractional shortening (16 vs. 17%, p=0.28) at diagnosis were similar. Era 2 patients were more likely to be treated with ACE inhibitors (71 vs. 62%, p=0.004), beta-blockers (24 vs. 6%, p<0.001), and diuretics (89 vs. 84%, p=0.002). A total of 291 patients (15%) died without transplantation, with a median time from diagnosis to death of 0.4 years. Cox regression modeling demonstrated Era 1 was associated with a higher rate of death (HR=1.5, 95% CI=1.2-2.0, p=0.002) but not transplant (0.9, 95% CI=0.7-1.1, p=0.25) when controlling for etiology group. Competing risks estimate (Figure) showed that Era 1 was associated with a higher death rate (p<0.001), while heart transplant rate was not significantly different by era (p=0.068). Conclusions: Children with DCM phenotype have improved survival in the more recent era. This appears to be associated with factors other than the availability of transplantation, which was equally prevalent in both eras.
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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 | 0.002 | 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 | 0.000 | 0.001 |
| Research integrity | 0.000 | 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".