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Record W4414433887 · doi:10.3390/jcdd12100374

Rethinking Childhood-Onset Hypertrophic Cardiomyopathy: A Review of Molecular Mechanisms and Unique Therapy Considerations

2025· review· en· W4414433887 on OpenAlexafffund
Caitlin Menzies, Vernon W. Dolinsky

Bibliographic record

VenueJournal of Cardiovascular Development and Disease · 2025
Typereview
Languageen
FieldMedicine
TopicCardiomyopathy and Myosin Studies
Canadian institutionsUniversity of ManitobaChildren's Hospital Research Institute of Manitoba
FundersCanadian Institutes of Health ResearchFondation Brain CanadaHeart and Stroke Foundation of Canada
KeywordsHypertrophic cardiomyopathyDiseaseRisk stratificationPrecision medicinePersonalized medicineClinical PracticeClinical trial

Abstract

fetched live from OpenAlex

Childhood-onset hypertrophic cardiomyopathy (HCM) is a cardiac disorder presenting unique diagnostic and therapeutic challenges in children that require tailored clinical attention compared to HCM arising at other life stages. Despite this, current treatment strategies specific to childhood-onset HCM are lacking and are predominantly extrapolated from adult-specific treatment guidelines. This review explores the molecular basis, clinical implications, and management strategies specific to childhood-onset HCM. Advances in molecular genetics have elucidated diverse pathogenic pathways and genotype-phenotype correlations, revealing age-specific disease modifiers distinct from adult-onset forms. Current management includes pharmacologic, surgical, and device-based interventions, tailored to individualized needs. However, there is a lack of evidence for the efficacy and safety profiles of these treatments in children, meaning children may be receiving sub-optimal care. Emerging approaches, such as gene-targeted therapies and precision medicine frameworks, show promise, but require further investigation. Enhancing early diagnosis and personalized care is crucial for improving outcomes and reducing long-term disease burden in affected children. This review underscores the necessity for specific research to refine risk stratification and treatment paradigms for childhood-onset HCM.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame distilled prediction

Teacher imitation

Not 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.

metaresearch head score (Codex)0.002
metaresearch head score (Gemma)0.001
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesMeta-epidemiology (narrow)
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Other design · Consensus signal: none
GenreCandidate signal: Review · Consensus signal: Review
Teacher disagreement score0.790
Threshold uncertainty score1.000

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0020.001
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0040.003
Bibliometrics0.0000.000
Science and technology studies0.0000.000
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0000.000

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.

Opus teacher head0.028
GPT teacher head0.279
Teacher spread0.252 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one teacher head, not a consensus.

Study designOther design
Domainnot available
GenreReview

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".

Quick stats

Citations0
Published2025
Admission routes2
Has abstractyes

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