Background/Purpose: Cardiac involvement is one of the worst prognostic factors in JSSc. The diagnosis is usually based on clinical symptoms, EKG and conventional echocardiography, but histological studies proved that the prevalence of cardiac involvement is higher than expected. Some studies on adults demonstrated that cardiac Magnetic Resonance Imaging and  Strain Rate imaging (SR) identify early cardiac involvement in SSc but little is known about their use in juvenile SSc.  In this study, we investigated whether subclinical cardiac involvement can be detected by using cardiac MRI and echocardiographic SR imaging in a cohort of JSSc patients.

Methods: Consecutive patients with JSSc, according to the PRES/EULAR/ACR criteria (1), evaluated from January to May 2015, were enrolled in this observational cross sectional study. Demographic and clinical features were collected from the clinical reports. Disease severity was evaluated by the J4S score (2). Each patient underwent EKG, conventional echocardiography, speckle tracking SR and cardiac contrast enhanced MRI. Normal standard for age and body surface area-matched values validated for pediatric age were used as reference (3,4).

Results: Twelve patients, 8 females,  mean age 12.8 years, mean disease duration 5.0 years entered the study. Ten had a diffuse form of disease, 2 limited. Three patients (25%) had previously had symptomatic cardiac involvement: 1 cardiac arrest during sustained ventricular tachycardia, 1 not sustained ventricular tachycardia, 1 pericarditis. Conventional echocardiography showed normal left ventricular ejection fraction and diastolic function in all patients, except for the one with previous episode of ventricular fibrillation.  Also EKG was abnormal only in this patient. SR imaging (mean -17,9%) was abnormal in 4 patients (33,3%), all with the diffuse SSc; two of them never had cardiac symptoms and their EKG and echocardiography were normal.  Cardiac MRI was altered in 2 (16,6%) patients: fibrosis and dyskynesias were found in a symptomatic patient with abnormal EKG, conventional echocardiography and SR; another asymptomatic patient showed dilated ventricles but no fibrosis. No correlation with J4S score was found.

Conclusion: Cardiac MRI and SR imaging allowed to detect early cardiac abnormalities in 3 patients (25%) with neither cardiac symptoms nor abnormal EKG and echocardiography, so they could be considered as valid, non invasive tools for the assessment of early cardiac involvement in JSsc. Prospective multicentric studies  are needed to confirm these data.

References

1. Zulian F, et al. The PRES/EULAR/ACR provisional classification criteria for Juvenile Systemic Sclerosis. Arthritis Rheum 2007;57(2):203-12
2. La Torre F,  et al. A preliminary disease severity score for juvenile systemic sclerosis. Arthritis Rheum 2012;64:4143-50
3. Kawel-Boehm N, et al. Normal values for cardiovascular magnetic resonance in adults and children. J Cardiovasc Magn Reson 2015;17:29

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/magnetic-resonance-and-echocardiographic-strain-rate-imaging-for-the-early-detection-of-cardiac-involvement-in-juvenile-systemic-sclerosis/