Heart failure & cardiomyopathies
Characterisation and prognostic implications of the 12-lead electrocardiogram in children with RASopathy-associated hypertrophic cardiomyopathy
Authors' 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.
Study context
- Population
- Children with RASopathy-associated hypertrophic cardiomyopathy (RASopathy-associated HCM (RAS-HCM)), compared with patients with sarcomeric hypertrophic cardiomyopathy (HCM) (sarcomeric HCM (s-HCM))RAS-HCM 84 · s-HCM 113
- Participants
- 84 vs 113
- Exposure
- The resting 12-lead ECGs of 84 children with RAS-HCM were compared with those from 113 patients with sarcomeric HCM (s-HCM)
- RAS-HCMn = 84
- s-HCMn = 113
- Outcome
- Superior axis deviation
Main finding
As reported in the abstract
P < 0.001
Observational data: associations, not effects.
Further findings
Voltage criteria for right ventricular hypertrophy
P < 0.001
Pathological Q waves · significantly lower prevalence in RAS-HCM
P < 0.001
ECG predictors of MACE in RAS-HCM
| Comparison | Adjusted relative risk (RR) (95% CI) | P value | |
|---|---|---|---|
| ST segment changes>2 mmMultivariate model | 2.33 (1.12–4.86) | p value 0.024 | |
| 0.20.5125Adjusted relative risk (RR) (log scale) |
- MACE in RAS-HCM · median follow-up 6.8 years (3.1-9.7)
- 22.6% · 19 of 84
ECG predictors of MACE in RAS-HCM
- Right atrial enlargement and ST segment changes>2 mm correlated with MACE on univariate analysis
Original publication
Characterisation and prognostic implications of the 12-lead electrocardiogram in children with RASopathy-associated hypertrophic cardiomyopathy. Heart. Published 7 October 2025. doi:10.
Summarised from the published abstract; every number is checked against it