Unveiling Cardiac Involvement in Juvenile Dermatomyositis Through Speckle-Tracking Echocardiography

Pediatr Cardiol. 2024 Jun;45(5):1007-1014. doi: 10.1007/s00246-024-03438-4. Epub 2024 Mar 28.

Abstract

Early detection of cardiac involvement in Juvenile Dermatomyositis (JDM) is difficult due to the absence of clinical signs and symptoms, with systolic dysfunction often emerging in late stages and associated with a poor prognosis. This study aimed to employ two-dimensional speckle-tracking echocardiography (STE) for subclinical assessment of left ventricular (LV) systolic failure in JDM and explore potential associations between impaired LV systolic function (LV-GLS) and disease activity. A prospective study enrolled 20 healthy volunteers and 26 JDM patients (< 18 years old) without cardiac symptoms. Clinical data were collected from medical records, and echocardiograms were conducted by a pediatric cardiologist. Our study cohort demonstrated similar age to controls (13.5 ± .6 vs. 13.8 ± 4.7; p = 0.465). Median illness duration at echocardiography was 5 (1.5-17.5) years, and conventional echocardiography indicated normal LV ejection fraction (> 55%) in all participants. However, STE revealed lowered LV GLS in JDM patients (- 22.2 ± 4.1% vs. - 26.5 ± 5.3% p = 0.022). Pulse steroid users displayed lower GLS average values compared to non-users (β = 4.99, 95% CI 1.34-8.64, p = 0.009). Negative correlations existed between LV-GLS and age at diagnosis (r = - 0.499; p = 0.011), diastolic parameters (E/E' ratio) and age at diagnosis (r = - 0.469; p = 0.018), as well as RV global strain and age at diagnosis (r = - 0.443; p = 0.024). Employing STE in JDM patients facilitated the identification of preclinical cardiac dysfunction. Given JDM patients' younger age, early myocardial damage detection through STE may impact treatment decisions and long-term cardiovascular prognosis.

Keywords: Cardiac dysfunction; Global longitudinal strain; Juvenile dermatomyositis; Speckle tracking echocardiography.

MeSH terms

  • Adolescent
  • Case-Control Studies
  • Child
  • Dermatomyositis* / complications
  • Dermatomyositis* / diagnostic imaging
  • Dermatomyositis* / physiopathology
  • Echocardiography* / methods
  • Female
  • Humans
  • Male
  • Prospective Studies
  • Stroke Volume
  • Systole
  • Ventricular Dysfunction, Left* / diagnostic imaging
  • Ventricular Dysfunction, Left* / etiology
  • Ventricular Dysfunction, Left* / physiopathology