Background/Purpose: Evaluation of right ventricle (RV) systolic function by standard echocardiogram remains a challenge because of the complex geometry of this chamber. Recently, two-dimensional Speckle-tracking derived strain (2DST) was proven to have a better accuracy in detecting subtle RV dysfunction in children with congenital heart diseases, however this new technique was not systematically studied in childhood-onset SLE patients. Therefore, the aim of this study was to evaluate strain imaging by 2DST in cSLE patients and healthy controls and possible association of RV dysfunction with demographic data, clinical manifestations, laboratory and treatment.

Methods: A cross-sectional study was conducted at our Pediatric Rheumatology Unit from September 2012 to September 2013. Exclusion criteria were heart failure, congenital heart disease, pericardial effusion, history of infectious myocarditis or pulmonary obstructive diseases and poor quality echocardiographic imaging. Thirty-five cSLE patients and 33 healthy volunteers were submitted to standard echocardiogram, and 2DST. Conventional parameters included: RV diastolic diameter (RVDD), tricuspid annular plane systolic excursion (TAPSE), tricuspid annular systolic wave velocity (S), Tei index and the presence of pulmonary hypertension (velocity of tricuspid regurgitation jet>2.5m/s). 2DST analyzed global systolic longitudinal strain and strain rate of RV. Demographic data, clinical features, SLEDAI-2K, SLICC/ACR-DI and treatment were also assessed.

Results: The median current age was similar in cSLE patients and controls (14.75 vs. 14.88 years, p=0.62). Standard echocardiogram analysis revealed that cSLE patients had a significant increase RVDD (14.65±3.68 mm/m² vs. 12.31±2.43 mm/m², p=0.031) and Tei index compared to controls [0.49 (0.3-0.96) vs. 0.32 (0.22-0.45), p<0.0001]. They also had reduced S wave velocity [0.13 (0.09-0.2) vs. 0.16 (0.13-0.23)m/s, p<0.0001] and TAPSE (1.84±0.26 vs. 2.59±0.41cm, p<0.0001). Four patients had mild pulmonary hypertension. Further evaluation of function by 2DST showed that RV longitudinal peak systolic strain was significantly reduced in cSLE (-24.5±5.09 vs. -27.62±3.02%, p=0.003).  The same finding was observed with the exclusion of four patients with mild pulmonary hypertension (-24.62 ± 4.87 vs. -27.62 ± 3.02, 0.0041, p=0.0041). RV longitudinal peak systolic strain was positively correlated with TAPSE (r=+0.49, p=0.0027) and negatively correlated with Tei index (r=–0.34, p=0.04) in cSLE patients. Further analysis of cSLE patients revealed higher frequencies of neuropsychiatric manifestations (39% vs. 0%, p=0.007) and antiphospholipid antibodies (55% vs. 18%, p=0.035) in those with reduced strain (≤ -23.7%) compared to high strain values (>-23.7%). No differences were evidenced in demographic data, disease activity/damage and treatments (p>0.05).

Conclusion: This is the first study to identify, using a more accurate methodology, subclinical RV systolic dysfunction in cSLE patients. The novel association of asymptomatic cardiac dysfunction with neuropsychiatric manifestations and antiphospholipid antibodies may suggest a common underlying mechanism.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/subclinical-right-ventricle-systolic-dysfunction-by-two-dimensional-speckle-tracking-echocardiography-in-childhood-onset-systemic-lupus-erythematosus-patients/