THU0520 EARLY IDENTIFICATION OF VENTRICULAR DYSFUNCTION IN JUVENILE SYSTEMIC SCLEROSIS BY SPECKLE TRACKING ECHOCARDIOGRAPHY

Abstract

Background: Juvenile Systemic Sclerosis (JSSc) is a rare multisystemic connective tissue disease, with onset before the age of 16. Cardiac involvement, recognized in 8-24% of the patients [1], begins in early stages of JSSc and has a poor prognosis. The traditional cardiac US imaging, including the left ventricular ejection fraction (EF), evaluates the global function of the heart, thus being inappropriate to assess the subclinical course of the disease. A new echocardiographic technique, the speckle tracking echocardiography (STE), has been shown to be able to identify regional ventricular dysfunctions also in early stages of adult-onset SSc[2,3]. Objectives: Aim of our study was to assess the longitudinal strain of right and left ventricle in JSSc patients, in order to identify ventricular dysfunctions earlier and more effectively than with traditional echocardiography. Furthermore, we investigated the evolution of cardiac involvement during the follow-up and possible correlations with the overall disease severity, measured by the Juvenile Systemic Sclerosis Severity Score (J4S)[4]. Methods: Consecutive patients with JSSc underwent clinical and cardiologic evaluation. This included traditional echocardiography (such as M-Mode, EF, Pulsed- and Tissue-Doppler), 3D-Echocardiography and STE, measuring the global longitudinal strain of left ventricle (GLS) and the longitudinal strain of right ventricle free-wall (RVLS). Each patient was assessed several times by pediatric rheumatologists for J4S and by cardiologists with STE and standard echo. Results: 18 JSSc patients (12 F, 6 M), mean age 12.3 years, disease duration 4.5 years, entered the study. At baseline evaluation, EF was abnormal in 1 patient, whereas GLS and RVLS were abnormal in 5. The diagnostic sensitivity of cardiac involvement of STE increased, with a prevalence rising from 22.2% to 38.8%. During the follow-up, lasted mean 30 months (range 17–43), the mean GLS values gradually worsened (-21.2; -20.1; -19.4%) while there was no significant variations of EF. The strong correlation between GLS and J4S, found at baseline, vanished during the follow-up. Conclusion: Speckle tracking echocardiography is a useful technique to evaluate the cardiac involvement in patients with JSSc. In comparison with traditional EKG or echocardiography, it allows to increase the diagnostic sensitivity of cardiac involvement. Over time, we observed a gradual worsening of GLS, sign of a progressive left ventricular dysfunction, that was not identified by EF. It is possible that the coronary microvascular damage compromises the subendocardial fibers function which are more sensitive to ischemia and whose contractility is well assessed by GLS [5]. Finally, the initial correlation between strain and J4S disappeared during the follow-up, maybe because of the pharmacological therapy, which was effective on several aspects of the disease but had low impact on the ventricular function.