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

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