Subclinical right ventricular dysfunction in juvenile systemic sclerosis: a cross-sectional echocardiographic study

Area of Science:

• Cardiology
• Rheumatology
• Pediatrics

Background:

• Juvenile systemic sclerosis (JSSc) is a rare autoimmune disease with potential for multi-organ involvement.
• Cardiac complications, particularly subclinical myocardial dysfunction, can occur in asymptomatic JSSc patients.
• Early detection of cardiac involvement is crucial for timely intervention and improved prognosis.

Purpose of the Study:

• To evaluate subclinical myocardial involvement in asymptomatic JSSc patients.
• To compare cardiac function between JSSc patients and healthy controls using advanced echocardiography.
• To assess the utility of conventional Doppler (CD), tissue Doppler imaging (TDI), and speckle tracking echocardiography (STE) in detecting early cardiac dysfunction.

Main Methods:

• A cross-sectional, retrospective study involving nine asymptomatic JSSc patients and ten age- and gender-matched healthy controls.
• All participants underwent comprehensive echocardiographic assessment including CD, TDI, and STE.
• Comparison of right and left ventricular systolic and diastolic parameters between the JSSc group and the control group.

Main Results:

• JSSc patients exhibited altered diastolic function (lower tricuspid E/A ratio, shorter deceleration time) and increased myocardial performance index (MPI) compared to controls.
• Right ventricular ejection fraction (RVEF) was significantly lower in JSSc patients, indicating subclinical right ventricular dysfunction.
• While left ventricular global longitudinal strain (GLS) and circumferential strain (GCS) were preserved, TDI-derived MPI was elevated in JSSc patients, suggesting early global cardiac dysfunction.

Conclusions:

• Subclinical right ventricular dysfunction is an early finding in asymptomatic JSSc patients, even without pulmonary hypertension.
• Advanced echocardiographic techniques like STE and TDI-derived MPI are valuable for early detection of cardiac involvement in JSSc.
• Timely diagnosis and intervention based on these findings can potentially improve long-term outcomes for JSSc patients.