Abstract 14221: The Role of Left Ventricular Dysfunction in Children With Arrhythmogenic Right Ventricular Cardiomyopathy
Bibliographic record
Abstract
Introduction: We sought to evaluate the impact of left ventricular dysfunction (LVDfx) in children with a definite arrhythmogenic right ventricular cardiomyopathy (ARVC) diagnosis. Methods: We retrospectively reviewed patients 0-18 years of age diagnosed with ARVC at our institution between 2001-2018. Patients with LVDfx, defined as an ejection fraction of <55% on echocardiogram, were identified and their outcomes compared to ARVC patients without LVDfx. Kaplan-Meier transplant-free survival curves were utilized. Results: Thirty-five children with a diagnosis of ARVC were identified. Twelve patients (34%) had LVDfx, of which 7 (20%) had moderate-severe LVDfx. The median age at diagnosis was 15 years (IQR 13.2 - 15.8) with a median follow-up time of 2.1 years (IQR 1.1 - 4.3). There was no association with increased left ventricular dimension or mitral regurgitation in either group. Cardiac magnetic resonance imaging demonstrated that LVDfx was associated with moderate to severe right ventricular dysfunction (41.6% vs 17.4% p = 0.009) and late gadolinium enhancement (58.3% vs 8.7% p = 0.004). Arrhythmia burden was greater in the LVDfx group with a higher incidence of ectopy (83% vs 50% p = 0.04) and non-sustained ventricular tachycardia (83% vs 22% p = 0.0009). The LVDfx group was significantly more likely to receive an angiotensin converting enzyme inhibitor (41.6% vs 0% p = 0.002) or a beta-blocker (75% vs 8.7% p = 0.0001). Total length of hospital stay was significantly longer in the LVDfx cohort (median 2 days vs 0 days p = 0.001). The two-year transplant free survival was significantly worse in the LVDfx group (88% vs. 100%, p=0.04, figure 1). Conclusions: ARVC is not always confined to a single chamber and LVDfx confers a greater risk to the healthcare trajectory of children with ARVC. Therapeutic strategies for LVDfx may have a substantial impact on outcomes 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 | 0.002 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 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".