New Insights into Cardiac Involvement in Juvenile Scleroderma: A Three-Dimensional Echocardiographic Assessment Unveils Subclinical Ventricle Dysfunction

Abstract

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.