Boleti, Olga;
Sunjaya, Angela;
Field, Ella;
Norrish, Gabrielle;
Tollit, Jennifer;
Cervi, Elena;
Kaski, Juan Pablo;
(2025)
Characterisation and prognostic implications of the 12-lead electrocardiogram in children with RASopathy-associated hypertrophic cardiomyopathy.
Heart
10.1136/heartjnl-2025-326268.
(In press).
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Abstract
BACKGROUND: The 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). METHODS: The resting 12-lead ECGs of 84 children with RAS-HCM were compared with those from 113 patients with sarcomeric HCM (s-HCM). RESULTS: A 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). CONCLUSION: These 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.
| Type: | Article |
|---|---|
| Title: | Characterisation and prognostic implications of the 12-lead electrocardiogram in children with RASopathy-associated hypertrophic cardiomyopathy |
| Location: | England |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1136/heartjnl-2025-326268 |
| Publisher version: | https://doi.org/10.1136/heartjnl-2025-326268 |
| Language: | English |
| Additional information: | Copyright © Author(s) (or their employer(s)) 2025. This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/. |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science > Childrens Cardiovascular Disease |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10215533 |
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