Research Information System
PEDIATRIC CARDIOLOGY, vol.38, no.8, pp.1686-1695, 2017 (SCI-Expanded)
Cardiac manifestations in juvenile scleroderma or systemic sclerosis (JSSc) have poor prognosis, begin in early stages of the disease, and remain clinically asymptomatic. New echocardiography modalities, such as 2D/3D speckle tracking (STE, strain analysis for regional and global ventricular functions), can detect cardiac involvement in early stages. We assessed 21 JSSc patients and 19 controls using 2D/3D STE. The left ventricular end diastolic volume, end systolic volume, and ejection fraction of the patient and control groups were significantly different (99.2 +/- 23.8 vs. 52 +/- 23.8, 40.6 +/- 16.0 vs. 20.2 +/- 17.4 and 59.2 +/- 7.5 vs. 65.6 +/- 5.2, respectively). Global longitudinal strain (GLS) and global circumferential strain (GCS) were lower in the patient group (18.4 +/- 4.7 vs. 22.4 +/- 3.7, 26.4 +/- 5.8 vs. 31.4 +/- 3.5), as were the peak systolic strain values of the right ventricular longitudinal strain (RVLS) septum and RVLS free wall (18.1 +/- 6.8 vs. 24.8 +/- 6.0 and 22.8 +/- 5.9 vs. 28.0 +/- 6.9, respectively). 3D measurements of RVEDV, RVESV, and RVSV were higher in the patient group (88.2 +/- 31.3 vs. 50.8 +/- 23.5, 43.1 +/- 17.6 vs. 19.0 +/- 12.2, and 45.0 +/- 16.2 vs. 31.7 +/- 12.6). RVLS freewall results were lower in the JSSc patients with interstitial lung fibrosis, arthritis, muscle weakness, weight loss, and anti-scl 70 antibodies than in the JSSc patients without these variables. We found that a GCS of < 34.5% could identify patients for left ventricular (LV) dysfunction with a sensitivity of 93.3, specificity of 92.9, while an RVEF of < 60.7% could identify patients for left ventricular (RV) dysfunction with a sensitivity of 92.9 and specificity of 21.4%. We highlighted key advantages of 3D STE for the tracking of early systolic dysfunction in patients with JSSc who would benefit from medical intervention for cardiac complications.