title: Subclinical right ventricle systolic dysfunction in childhood-onset systemic lupus erythematosus: insights from two-dimensional speckle-tracking echocardiography.
abstract: OBJECTIVE
  The objective of this article is to evaluate right ventricle strain imaging by two-dimensional speckle-tracking (2DST) in childhood-onset systemic lupus erythematosus (c-SLE).
  
  
  METHODS
  Thirty-five c-SLE patients with no signs or symptoms of heart failure and 33 healthy volunteers were evaluated by standard echocardiogram and 2DST. Conventional parameters included tricuspid annular plane systolic excursion (TAPSE), RV tissue-Doppler-derived Tei index and systolic pulmonary artery pressure. Global peak longitudinal systolic strain (PLSS) and strain rate (PLSSR) of RV were obtained by 2DST. Demographic/clinical features, SLEDAI-2K/SLICC/ACR-DI and treatment were also assessed.
  
  
  RESULTS
  The median current age was similar in patients and controls (14.75 vs. 14.88 years, p = 0.62). RV PLSS was significantly reduced in c-SLE (-24.5 ± 5.09 vs. -27.62 ± 3.02%, p = 0.003). Similar findings were observed after excluding patients with pulmonary hypertension (-24.62 ± 4.87% vs. -27.62 ± 3.02%, p = 0.0041). RV PLSS was positively correlated with TAPSE (r = +0.49, p = 0.0027) and negatively correlated with Tei index (r = -0.34, p = 0.04) in c-SLE. RV PLSSR was not different comparing patients and controls (-0.65 s(-1 )± 0.47 vs. -1.87 ± 0.49 s(-1), p = 0.07). Further analysis of c-SLE 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 RV PLSS ≤ -23.7% vs >-23.7%. No differences were evidenced in demographic data, disease activity/damage or treatments (p > 0.05).
  
  
  CONCLUSIONS
  The present study, using a new and more sensitive technique, revealed subclinical RV systolic dysfunction in c-SLE patients that may have future prognostic implications. The novel association of asymptomatic RV dysfunction with neuropsychiatric manifestations and antiphospholipid antibodies may suggest common physiopathological pathways.
authors.affiliation: Radiology Unit, Instituto da Criança, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
authors.forename: G N
authors.initials: GN
authors.lastname: Leal
authors.affiliation: Radiology Unit, Instituto da Criança, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
authors.forename: K F
authors.initials: KF
authors.lastname: Silva
authors.affiliation: Pediatric Rheumatology Unit, Instituto da Criança, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
authors.forename: C M P
authors.initials: CM
authors.lastname: França
authors.affiliation: Radiology Unit, Instituto da Criança, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
authors.forename: A C
authors.initials: AC
authors.lastname: Lianza
authors.affiliation: Department of Radiology, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
authors.forename: J L
authors.initials: JL
authors.lastname: Andrade
authors.affiliation: Pediatric Rheumatology Unit, Instituto da Criança, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
authors.forename: L M A
authors.initials: LM
authors.lastname: Campos
authors.affiliation: Department of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
authors.forename: E
authors.initials: E
authors.lastname: Bonfá
authors.affiliation: Pediatric Rheumatology Unit, Instituto da Criança, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil clovisaasilva@gmail.com.
authors.forename: C A
authors.initials: CA
authors.lastname: Silva
mesh_terms: D000293:Adolescent; D017152:Antibodies, Antiphospholipid / Q000276:immunology; D002648:Child; D003430:Cross-Sectional Studies; D004452:Echocardiography / Q000379:methods*; D015150:Echocardiography, Doppler / Q000379:methods*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000503:physiopathology; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000503:physiopathology*; D020945:Lupus Vasculitis, Central Nervous System / Q000000981:diagnostic imaging / Q000503:physiopathology; D008297:Male; D015203:Reproducibility of Results; D013599:Systole / Q000502:physiology; D018497:Ventricular Dysfunction, Right / Q000000981:diagnostic imaging* / Q000503:physiopathology*; D055815:Young Adult
chemical_list: D017152:Antibodies, Antiphospholipid; D007166:Immunosuppressive Agents
keywords: Childhood-onset systemic lupus erythematosus; antiphospholipid antibodies; neuropsychiatric manifestations; right ventricle systolic function; speckle-tracking echocardiography