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To compare cardiac function in patients with juvenile dermatomyositis (JDM) with matched controls, and examine associations between pathological electrocardiography (ECG), echocardiographic abnormalities and disease variables in patients with JDM.A total of 59 patients with JDM, examined a median 16.8 years (range 2-38 years) after disease onset, were compared with 59 age-matched and sex-matched controls. Echocardiography, including early diastolic transmitral flow/early diastolic tissue velocity (E/E') as a marker for diastolic dysfunction, and 12-channel ECG were performed and analysed blinded to patient information. Disease activity and damage were assessed by clinical examination at follow-up and chart review.E/E' was elevated (9.5) in 13 (22%) patients versus 0 controls (p0.001). In all, 10 patients presented with pathological ECG compared to 4 controls (p=0.054). Previous or current hypertension was found in 12 patients versus 0 controls (p0.001). Among the patients, pathological ECG was found in 6/13 patients with versus 4/44 without elevated E/E' (p=0.002); and systolic blood pressure was correspondingly 132±24 mm Hg versus 112±18 mm Hg in the groups (p=0.012). E/E' correlated with cumulative organ damage assessed at follow-up (r(sp) 0.41, p=0.001) and disease activity at 1 year (r(sp) 0.56, p0.001), which also predicted pathological E/E' after controlling for age and gender. During disease course, 12% of patients with JDM developed pericarditis.Only patients with JDM and no controls had subclinical left ventricular diastolic dysfunction; the patients with elevated E/E' also had high prevalence of pathological ECG and hypertension. High disease activity 1-year post diagnosis predicted high E/E' at follow-up. The findings suggest that subclinical heart disease is related to the systemic nature of JDM.