Evidence map›Paper›PMID 41057251›Full record

ArticleHeart (British Cardiac Society)2026

Characterisation and prognostic implications of the 12-lead electrocardiogram in children with RASopathy-associated hypertrophic cardiomyopathy.

Olga Boleti, Angela Sunjaya, Ella Field, Gabrielle Norrish, Jennifer Tollit, Elena Cervi, Juan Pablo Kaski

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Article in Heart (British Cardiac Society), 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

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2 · The registry

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3 · Its place in the literature

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4 · The record

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5 · Who and what money

Authors and funding

7 authors.

Olga BoletiCentre for Paediatric Inherited and Rare Cardiovascular Disease, Institute of Cardiovascular Science, University College London, London, UK.ORCID 0000-0002-0254-0217
Angela SunjayaCentre for Paediatric Inherited and Rare Cardiovascular Disease, Institute of Cardiovascular Science, University College London, London, UK.
Ella FieldCentre for Paediatric Inherited and Rare Cardiovascular Disease, Institute of Cardiovascular Science, University College London, London, UK.
Gabrielle NorrishCentre for Paediatric Inherited and Rare Cardiovascular Disease, Institute of Cardiovascular Science, University College London, London, UK.ORCID 0000-0003-2064-237X
Jennifer TollitCentre for Paediatric Inherited and Rare Cardiovascular Disease, Institute of Cardiovascular Science, University College London, London, UK.
Elena CerviCentre for Paediatric Inherited and Rare Cardiovascular Disease, Institute of Cardiovascular Science, University College London, London, UK.
Juan Pablo KaskiCentre for Paediatric Inherited and Rare Cardiovascular Disease, Institute of Cardiovascular Science, University College London, London, UK j.kaski@ucl.ac.uk.ORCID 0000-0002-0014-9927

Funding

Medical Research Council MR/T024062/1
6 · The paper itself

Abstract

backgroundThe 12-lead ECG is a simple, inexpensive clinical tool with a key role in the assessment of patients with hypertrophic cardiomyopathy (HCM). The aims of this single centre, retrospective cohort study were to characterise ECG findings and to identify potential ECG predictors of major adverse cardiovascular events (MACE-cardiovascular mortality, resuscitated cardiac arrest, ventricular arrhythmias with haemodynamic compromise, appropriate implantable cardioverter defibrillator therapy or heart failure hospitalisation) in children with RASopathy-associated HCM (RAS-HCM).

methodsThe resting 12-lead ECGs of 84 children with RAS-HCM were compared with those from 113 patients with sarcomeric HCM (s-HCM).

resultsA significant proportion of ECGs in RAS-HCM had superior axis deviation (29.8% vs 2.5%, p value<0.001) and voltage criteria for right ventricular hypertrophy (52.4% vs 28.3%, p value<0.001), and a significantly lower prevalence of pathological Q waves (27.4% vs 47.8%, p value<0.001). Over a median follow-up period of 6.8 years (3.1-9.7), 19 patients (22.6%) with RAS-HCM suffered an MACE. Right atrial enlargement and ST segment changes>2 mm correlated with MACE on univariate analysis, with the latter remaining significant after adjustment in a multivariate model (adjusted relative risk (RR) 2.33, 95% CI 1.12 to 4.86, p value 0.024).

conclusionThese findings suggest that the 12-lead ECG may be a useful screening tool to distinguish RAS-HCM from s-HCM in everyday practice and could have potential implications for prediction of adverse outcomes.

Indexed as

Cardiomyopathy, HypertrophicElectrocardiographyAdolescentChildChild, PreschoolFemaleHumansInfantMalePredictive Value of TestsPrognosisRetrospective Studies

Identifiers

PMID41057251
PMCPMC13217015

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