Abstract 9202: Sudden Cardiac Death and Icd Use in Rasopathy-Associated Hypertrophic Cardiomyopathy: An International Multi-Centre Study
Bibliographic record
Abstract
Introduction: Sudden cardiac death (SCD) burden in RASopathy patients (pts) with hypertrophic cardiomyopathy (RAS-HCM) is not known. We compared SCD events and transplant-free survival in RAS versus primary HCM (P-HCM) pts. Methods: The international 22-center retrospective cohort study (PRIMaCY) included 190 pediatric RAS-HCM and 568 P-HCM pts. The primary outcome was 10-year freedom from SCD events (composite of SCD, resuscitated sudden cardiac arrest, and appropriate shock from a prophylactic ICD), estimated using Kaplan Meier method. Results: Of 190 RAS-HCM pts, 82% had Noonan syndrome. Age at diagnosis was lower in RAS-HCM vs P-HCM (0.9 vs 9.7 yrs, p<0.001). There was no difference in baseline LV posterior wall or septal thickness, but LVOT gradient was higher in RAS-HCM vs P-HCM (30 vs 13mmHg, p<0.001). However, frequency of myectomy was not different in RAS-HCM vs P-HCM (15.2% vs 16.7%, p=0.55). Eighteen RAS-HCM pts died (4 SCD, 2 bradycardic arrests, 5 heart failure, 3 respiratory failure, 2 other) with lower 10-year transplant-free survival in RAS-HCM vs P-HCM (84.2% vs 91.6%, p=0.026). Although 10-year cumulative proportion of SCD events was lower in RAS-HCM than P-HCM (5.4% vs 15.1% respectively, p<0.001) (Figure 1), the median age at event was not different (12yrs in RAS-HCM vs 15.2yrs in P-HCM). Of 5 RAS-HCM pts with a SCD event, 4 died and 1 received a secondary prevention ICD. An additional 10 pts received a prophylactic ICD with no appropriate shock during follow-up. The 10-year cumulative proportion of prophylactic ICD was lower at 6.3% in RAS-HCM vs 27.2% in P-HCM (p<0.001). Conclusion: Transplant-free survival was lower in RAS-HCM than P-HCM with mortality related primarily to non-SCD events. Of the RAS-HCM patients with SCD events, 4 died without an ICD highlighting the need for better risk prediction models for both SCD and heart failure mortality in this population.
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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 | 0.001 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.001 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.001 | 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".