Atrial arrhythmias in the young: early onset atrial arrhythmias preceding a diagnosis of a primary muscular dystrophy
Bibliographic record
Abstract
AIMS: The aetiology of atrial arrhythmias in the otherwise healthy and young is usually unrecognized. We hypothesized that rare cases of atrial arrhythmias in the young may represent the initial manifestation of a muscular dystrophy syndrome. METHODS AND RESULTS: We describe the clinical characteristics, disease progression, results of electrophysiological study, and genetic findings in four patients (age <40 years) presenting with idiopathic atrial arrhythmias who subsequently received a diagnosis of a muscular dystrophy syndrome. The mean age at presentation with atrial arrhythmias was 29.5 years (range, 21-37 years), and the mean delay to diagnosis of muscular dystrophy was 3.6 years (range, 0.5-6 years). Two patients received a subsequent diagnosis of myotonic dystrophy type 1 and 2 a diagnosis of Emery-Dreifuss muscular dystrophy. Disease-causing genetic defects were identified in all four patients. One patient underwent catheter ablation of atrial flutter, experiencing improvement in arrhythmia symptoms. Two patients required device therapy, each receiving cardiac resynchronization therapy-defibrillator implantation for progressive left ventricular dysfunction. CONCLUSION: Early onset atrial arrhythmias may be the first clinical manifestation of a muscular dystrophy syndrome. Appropriate clinical assessment and surveillance may uncover this primary cause and provide an opportunity for timely genetic counselling and family screening.
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.002 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.000 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 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 teacher head, 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".