(Un)natural history of right ventricular function and exercise tolerance during adolescence following tetralogy of fallot repair
Bibliographic record
Abstract
Abstract Introduction Patients with repaired tetralogy of Fallot (rTOF) develop progressive right ventricular (RV) dilatation and dysfunction in adulthood, which may have origins in childhood. Cohorts from previous eras studied patients who underwent primary repair at an older age with frequent use of palliative shunts. The trajectory of RV functional decline and its relation to exercise capacity and outcomes over childhood is poorly characterized. Purpose We sought to delineate changes in RV size, function, and exercise tolerance and occurrence of ventricular arrhythmia in a contemporary cohort of pediatric and adolescent rTOF. Methods We retrospectively reviewed serial echo, cardiac magnetic resonance (CMR) and clinical data from all rTOF patients between 2010-2020. We excluded patients with significant residual lesions, RV pressure overload (RVSP> half systemic) or without CMR data. Clinical data was recorded at 2-year intervals. We then compared CMR data and exercise tolerance at 10 to 16 years of age. Ventricular arrhythmias were defined based on Holter monitoring. Results We studied 95 rTOF patients (61% male). Pulmonary insufficiency fraction (31% vs 32%, p=0.8), RV size (RV end diastolic indexed volume 130 ml/m2 (117 – 167) vs. 140 ml/m2 (124 – 166), p=0.6) and RV free wall longitudinal strain (19.4±4.3% vs. 19.3±4.7%, p=0.86) remained stable at 10 and 16 years of age. CMR RVEF (51±6% vs. 47±5.3%, p=0.01) and exercise tolerance (95±17% vs. 81±14.4%, % predicted VO2, p=0.002, figure B) decreased with the advent of incident ventricular arrhythmias (figure A). There were no deaths. Conclusions Despite excellent survival in childhood, patients with rTOF develop worsening RV function, exercise intolerance and ventricular arrhythmias during adolescence. These are worrisome findings given the known increase in sudden death in the 3-4th decades of life that warrant further investigation into the underlying causes, their temporal development and therapeutic approaches. Figure 1: A-Kaplan-Meier curve of incident ventricular and atrial arrhythmias. B- percentage of predicted VO2 during childhood.
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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 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.002 |
| Bibliometrics | 0.000 | 0.000 |
| 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".