GABRIELA NUNES LEAL

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Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina - Médico

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  • article 4 Citação(ões) na Scopus
    Echocardiographic Follow-up of Perinatally HIV-infected Children and Adolescents Results From a Single-center Retrospective Cohort Study in Brazil
    (2020) VALLILO, Nathalia Gaspar; DURIGON, Giuliana Stravinskas; LIANZA, Alessandro Cavalcanti; DINIZ, Maria de Fatima Rodrigues; SAWAMURA, Karen Saori Shiraishi; BRITO, Carolina Rocha; MARQUES, Heloisa Helena de Souza; FERRARO, Alexandre Archanjo; LEAL, Gabriela Nunes
    Background: The effects of HIV and antiretroviral therapy on cardiovascular system of perinatally infected children throughout their development are not fully understood. Objectives: To determine the prevalence of cardiac abnormalities in a retrospective cohort of perinatally HIV-infected patients and to investigate associations between echocardiographic and clinical data during their follow-up. Methods: Review of medical records and echocardiogram reports of 148 perinatally HIV-infected patients between January 1991 and December 2015. Results: Four hundred and eighty echocardiograms were analyzed and 46 (31%) patients showed cardiac abnormalities, frequently subclinical and transient. Nadir CD4 count was higher in patients with consistently normal echocardiogram: 263 (4-1480) versus 202 (5-1746) cells/mu L, P = 0.021. Right ventricular (RV) dilation was detected in 18.9%, left ventricular (LV) dilation in 21.6%, septal hypertrophy in 12.2%, LV posterior wall hypertrophy in 6%, LV systolic dysfunction in 8% and pulmonary hypertension in 8.7% of patients. Opportunistic infections were associated with RV dilation [odds ratio (OR = 4.34; 1.78-10.53; P < 0.01)], pulmonary hypertension (OR = 8.78; 2.80-27.51; P < 0.01) and LV systolic dysfunction (OR = 5.38; 1.55-18.71; P < 0.01). Longer duration of highly active antiretroviral therapy was associated with reduced risk of LV dilation (OR = 0.91; 0.85-0.97; P < 0.01) and systolic dysfunction (OR = 0.71; 0.59-0.85; P < 0.01). Protease inhibitors use was associated with reduced risk of RV dilation (OR = 0.54; 0.30-0.97; P < 0.05), LV dilation (OR = 0.35; 0.21-0.60; P < 0.01) and LV systolic dysfunction (OR = 0.07; 0.02-0.31; P < 0.01). Higher CD4 count was associated with lower risk of LV systolic dysfunction (OR = 0.82; 0.69-0.98; P < 0.05). Conclusions: Echocardiograms identified cardiac abnormalities among children with perinatally acquired HIV infection, and data suggest that immunologic status and therapeutic strategies throughout development can influence cardiac disease burden in this population.
  • article 3 Citação(ões) na Scopus
    Impact of ERT and follow-up of 17 patients from the same family with a mild form of MPS II
    (2022) STEPHAN, Bruno de Oliveira; QUAIO, Caio Robledo; SPOLADOR, Gustavo Marquezani; PAULA, Ana Carolina de; CURIATI, Marco Antonio; MARTINS, Ana Maria; LEAL, Gabriela Nunes; TENORIO, Artur; FINZI, Simone; CHIMELO, Flavia Teixeira; MATAS, Carla Gentile; HONJO, Rachel Sayuri; BERTOLA, Debora Romeo; KIM, Chong Ae
    Background: Mucopolysaccharidosis type II, also known as Hunter syndrome, is a rare X-linked recessive disorder caused by deficiency of the lysosomal enzyme Iduronate-2-Sulfatase (IDS), leading to progressive accumulation of Glycosaminoglycans (GAGs) in several organs. Over the years, Enzyme Replacement Therapy (ERT) has provided significant benefits for patients, retarding the natural progression of the disease. Results: The authors evaluated 17 patients from the same family with a mild form of MPS type II; the proband had developed acute decompensated heart failure refractory to clinical measurements at 23 years and needed a rather urgent heart transplant; however, he died from surgical complications shortly after the procedure. Nevertheless, subsequent to his tragic death, 16 affected male relatives were detected after biochemical tests identifying the low or absent activity of the IDS enzyme and confirmed by molecular analysis of the IDS gene. Following diagnosis, different options of treatment were chosen: 6 patients started ERT with Elaprase (R) (Idursulfase) soon after, while the other 10 remained without ERT. Eventually, 4 patients in the latter group began ERT with Hunterase (R) (Idursulfase Beta). None presented adverse effects to either form of the enzyme. Among the 6 individuals without any ERT, two died of natural causes, after reaching 70 years. Despite the variable phenotype within the same family (mainly heart dysfunctions and carpal tunnel syndrome), all 14 remaining patients were alive with an independent lifestyle. Conclusion: Here, the authors report the variable progress of the disease with and without ERT in a large Brazilian family with a slowly progressive form of MPS II, harboring the same missense variant in the IDS gene.
  • article 0 Citação(ões) na Scopus
    Heart function in juvenile idiopathic arthritis patients: A biventricular two-dimensional speckle-tracking echocardiography study
    (2022) LIANZA, Alessandro C.; LEAL, Gabriela N.; AIKAWA, Nadia E.; KOZU, Katia T.; DINIZ, Maria De Fatima R.; SAWAMURA, Karen S. S.; MENEZES, Carolina R. B.; MARTINS, Camila Lino; CAMPOS, Lucia M.; ELIAS, Adriana M.; SILVA, Clovis A.
    Objectives We evaluated cardiac function in juvenile idiopathic arthritis (JIA) patients by 2D speckle-tracking echocardiography (2DSTE) and to assess possible associations with clinical, laboratorial, and treatment data. Methods A group of 42 JIA patients and 42 healthy controls were evaluated using both conventional echocardiography and 2DSTE. JIA patients underwent clinical and laboratory assessment. Results Conventional echocardiography data demonstrated normal left ventricular (LV) ejection fraction in both groups (71 vs. 71%; p = .69). 2DSTE analysis demonstrated that JIA patients presented significantly lower LV global systolic longitudinal strain (LVGLS) (-18.76 vs. -22%; p < .0001), LV systolic strain rate (LVSSR) (1.06 vs. 1.32 s(-1); p < .0001), LV diastolic strain rate (LVDSR) (1.58 vs. 1.8 s(-1); p < .0137), right ventricular global systolic strain (RVGLS) (-24.1% vs. -27.7%; p = .0002), and right ventricular systolic strain rate (RVSSR) (1.4 vs. 1.8 s(-1); p = .0035). JIA patients under biological agents presented higher LVGLS (p = .02) and RVLS (p = .01). We also detected an association between LVGLS and C-reactive protein [CRP; -20% in normal CRP (10/42) vs. -18% in elevated CRP patients (32/42), p = .03]. Conclusions JIA patients present different echocardiographic status from healthy patients. Moreover, our data suggest that JIA patients under biological agents present association with better cardiac function as shown by strain analysis.
  • article 18 Citação(ões) na Scopus
    Subclinical left ventricular dysfunction in childhood-onset systemic lupus erythematosus: a two-dimensional speckle-tracking echocardiographic study
    (2016) LEAL, G. N.; SILVA, K. F.; LIANZA, A. C.; GIACOMIN, M. F.; ANDRADE, J. L.; KOZU, K.; BONFA, E.; SILVA, C. A.
    Objectives: The main purpose of the study was to investigate left ventricular (LV) subclinical systolic and diastolic dysfunction in childhood-onset systemic lupus erythematosus (c-SLE) patients using two-dimensional speckle-tracking (2DST) echocardiography. We also interrogated possible correlations between impairment of myocardial deformation and the SLE Disease Activity Index 2000 (SLEDAI-2K), as well as the presence of traditional and disease-related cardiovascular risk factors (CRFs).Method: A total of 50 asymptomatic patients and 50 controls (age 14.74 vs. 14.82years, p=0.83) were evaluated by standard and 2DST echocardiography.Results: Despite a normal ejection fraction (EF), there was reduction in all parameters of LV longitudinal and radial deformation in patients compared to controls: peak longitudinal systolic strain (PLSS) [-20.3 (-11 to -26) vs. -22 (-17.8 to -30.4)%, p<0.0001], PLSS rate [-1.190.21 vs. -1.3 +/- 0.25s(-1), p=0.0005], longitudinal strain rate in early diastole [1.7 (0.99-2.95) vs. 2 (1.08-3.00) s(-1), p=0.0034], peak radial systolic strain [33.09 +/- 8.6 vs. 44.36 +/- 8.72%, p<0.0001], peak radial systolic strain rate [1.98 +/- 0.53 vs. 2.49 +/- 0.68s(-1), p<0.0001], and radial strain rate in early diastole [-2.31 +/- 0.88 vs. -2.75 +/- 0.97s(-1), p=0.02]. Peak circumferential systolic strain [-23.67 +/- 3.46 vs. -24.6 +/- 2.86%, p=0.43] and circumferential strain in early diastole [0.37 +/- 0.17 vs. 0.41 +/- 0.15, p=0.27] were similar between patients and controls, although peak circumferential systolic strain rate [-1.5 +/- 0.3 vs. -1.6 +/- 0.3s(-1), p=0.036] was reduced in c-SLE. Further analysis of patients revealed a negative correlation between LV PLSS and SLEDAI-2K (r= -0.52, p<0.0001), and also between LV PLSS and the number of CRFs per patient (r= -0.32, p=0.024).Conclusions: 2DST echocardiography has identified subclinical LV deformation impairment in c-SLE patients. Disease activity and cumulative exposure to CRFs contribute to myocardial compromise.
  • article 2 Citação(ões) na Scopus
    Strain Echocardiography in Pediatric Sepsis: Direct Guide to Hemodynamic Therapy in the Future?
    (2016) LEAL, Gabriela Nunes; COLLETI JR., Jose; CARVALHO, Werther Brunow de
  • article 8 Citação(ões) na Scopus
    Echocardiographic study of juvenile dermatomyositis patients: new insights from speckle-tracking-derived strain
    (2021) DINIZ, Maria de Fatima Rodrigues; KOZU, Katia Tomie; ELIAS, Adriana Maluf; LIANZA, Alessandro Cavalcanti; SAWAMURA, Karen Saori Shiraishi; MENEZES, Carolina Rocha Brito; FERREIRA, Juliana Caires de Oliveira Achili; SILVA, Clovis Artur; LEAL, Gabriela Nunes
    Objectives This study aimed to investigate subclinical left ventricle (LV) systolic dysfunction in juvenile dermatomyositis (JDM) using two-dimensional speckle-tracking echocardiography (2DST). Possible associations between LV deformation impairment and disease activity/cumulative damage were also evaluated. Methods Thirty-fiveconsecutive JDM patients without cardiac symptoms and 35 healthy volunteers were enrolled. Clinical data were collected from medical records, and echocardiograms were performed by a pediatric cardiologist, unaware of patients' conditions. Results Patients and controls had similar age (12.6 +/- 0.7 vs.12.5 +/- 0.6;p= 0.97) and gender (11F:24M vs.11F:24M;p= 1.0). Median of JDM duration was 4.6 (0.04-17.6) years, and only 6/35 (17%) had active disease (disease activity score (DAS > 3)). Conventional echocardiogram revealed preserved LV ejection fraction (EF) (>= 55%) in all individuals. In JDM, 2DST identified reduction of LV longitudinal [-22(-17.2 to -27.9) % vs. -23(-20.8 to -27.4) %;p= 0.028)] and circumferential -23.9 +/- 2.8% vs. -26.7 +/- 2.9%;p= 0.0002) strain. Lower longitudinal strain was associated with DAS >3 -19.9(-17.2 to -26.5)% vs. -22.1-18.9 to -27.9)%;p= 0.046], MDI extent > 0 [-19(-17.2 to -22.5)% vs. -22.1-19.2 to -27.9)%;p= 0.0008], MDI severity > 0 [-19(-17.2 to -22.1)% vs. -22.3(-20.3 to -27.9)%;p= 0.0001] and calcinosis[-20.6(-17.2 to -23)% vs. -22.3(-20.3 to -27.9)%;p= 0.03]. Lower circumferential strain was associated with MDI extent > 0 (-22.1 +/- 3.87% vs. -24.4 +/- 2.3%;p= 0.039), MDI severity > 0 (-21.7 +/- 3% vs. 24.7 +/- 2.3%;p= 0.004) and calcinosis (-22.5 +/- 3.3% vs. -24.8 +/- 2.1%;p= 0.02). There was a negative correlation between longitudinal strain and cumulative dose of prednisone (r= -0.44;p= 0.009) and methotrexate (r= -0.33;p= 0.0008). Conclusions LV 2DST detected early systolic myocardial compromise in asymptomatic pediatric JDM patients, with preserved EF. Longitudinal strain impairment was associated with disease activity and cumulative damage, whereas circumferential strain impairment was associated exclusively with cumulative damage.
  • article 13 Citação(ões) na Scopus
    Long-term evaluation of cardiac function in juvenile idiopathic arthritis under anti-TNF therapy
    (2014) LIANZA, A. C.; AIKAWA, N. E.; MORAES, J. C. B.; LEAL, G. N.; MORHY, S. S.; ANDRADE, J. L.; BONFA, E.; SILVA, C. A.
    Objective This paper aims to perform global assessment of long-term cardiac function in juvenile idiopathic arthritis (JIA) patients under TNF blockage therapy. Methods Twenty-five polyarticular-course JIA patients pre-anti-TNF and 22 healthy controls underwent conventional/tissue Doppler echocardiography and cardiac biomarkers measurements (N-terminal pro-brain natriuretic peptide [NT-pro-BNP] and troponin T) at baseline (BL). Twenty-one JIA patients completed six evaluations during two consecutive years. Clinical/laboratorial evaluations were assessed before and during TNF blockage therapy. Results JIA patients and controls were comparable regarding current age (p=0.898) and female gender (p=0.38). At BL isoyolumetric relaxation time of left ventricle (p=0.03), ventricular septum (VS), E' wave (p=0.014) and VS S wave velocity (p=0.03) were significantly reduced in JIA patients compared to controls. Frequencies of elevated NT-pro-BNP and troponin T levels were similar in JIA and controls (p=0.297 and p=0.756) and levels remained within normal range throughout the study, except for one patient with mild troponin T elevation. During TNF blockage therapy, none of the 21 participants had heart failure, ejection fraction or other parameters alterations in conventional and tissue Doppler. Only one had mild pulmonary hypertension. Further analysis revealed that JIA patients with elevated levels of NT-pro-BNP at BL had significantly more active joints (p=0.025) and higher ESR (p=0.034). Conclusion Long-term TNF blockage safety was demonstrated in JIA patients in spite of the observed subclinical diastolic involvement. Elevated cardiac biomarker in these patients was associated with inflammatory parameters reinforcing the need for a careful interpretation of this finding in patients with active disease.
  • article 8 Citação(ões) na Scopus
    In-depth cardiovascular and pulmonary assessments in children with multisystem inflammatory syndrome after SARS-CoV-2 infection: A case series study
    (2022) ASTLEY, Camilla; PEREIRA, Maria Fernanda Badue; LIMA, Marcos Santos; BUCHPIGUEL, Carlos Alberto; CARNEIRO, Camila G.; SAPIENZA, Marcelo Tatit; LEAL, Gabriela Nunes; PRADO, Danilo Marcelo Leite do; PECANHA, Tiago; SIECZKOWSKA, Sofia Mendes; MATSUO, Olivia Mari; LINDOSO, Livia; MARQUES, Heloisa Helena; SILVA, Clovis Artur; GUALANO, Bruno
    We assessed PET-CT myocardial blood flow (MBF) using N-13 ammonia, brachial flow-mediated dilation, and cardiopulmonary exercise test in five post-discarged MIS-C survivors. None of the patients (median age: 9, range: 7-18 years; 3 females; 2 males) had preexisting pediatric chronic conditions. At the follow-up visit, two patients exhibited severe perfusion defect developed in the left ventricular cavity, suggesting extensive myocardial ischemia (MBF <2.0) and one patient showed persistent mild pericardial effusion. Others two patients demonstrated endothelial dysfunction. Nevertheless, all patients had lower predicted values in the VO2peak, VO2VAT, OUES, and O-2 Pulse (range: 35.2%-64.5%; 15.6%-38.2%; 1.0-1.3 L/min; 4-7 ml/beat), respectively. Our d suggested that previously health MIS-C patients had impaired MBF, endothelial dysfunction and lower cardiopulmonary capacity at follow-up analysis. Multidisciplinary further investigations should be conducted to reinforce these findings.
  • article 59 Citação(ões) na Scopus
    Severe clinical spectrum with high mortality in pediatric patients with COVID-19 and multisystem inflammatory syndrome
    (2020) PEREIRA, Maria Fernanda Badue; LITVINOV, Nadia; FARHAT, Sylvia Costa Lima; EISENCRAFT, Adriana Pasmanik; GIBELLI, Maria Augusta Bento Cicaroni; CARVALHO, Werther Brunow de; FERNANDES, Vinicius Rodrigues; FINK, Thais de Toledo; FRAMIL, Juliana Valeria de Souza; GALLETI, Karine Vusberg; FANTE, Alice Lima; FONSECA, Maria Fernanda Mota; WATANABE, Andreia; PAULA, Camila Sanson Yoshino de; PALANDRI, Giovanna Gavros; LEAL, Gabriela Nunes; DINIZ, Maria de Fatima Rodrigues; PINHO, Joao Renato Rebello; SILVA, Clovis Artur; MARQUES, Heloisa Helena de Sousa
    OBJECTIVES: To assess the outcomes of pediatric patients with laboratory-confirmed coronavirus disease (COVID-19) with or without multisystem inflammatory syndrome in children (MIS-C). METHODS: This cross-sectional study included 471 samples collected from 371 patients (age<18 years) suspected of having severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. The study group comprised 66/371 (18%) laboratory-confirmed pediatric COVID-19 patients: 61 (92.5%) patients tested positive on real-time reverse transcription-polymerase chain reaction tests for SARS-CoV-2, and 5 (7.5%) patients tested positive on serological tests. MIS-C was diagnosed according to the criteria of the Center for Disease Control. RESULTS: MIS-C was diagnosed in 6/66 (9%) patients. The frequencies of diarrhea, vomiting, and/or abdominal pain (67% vs. 22%, p=0.034); pediatric SARS (67% vs. 13%, p=0.008); hypoxemia (83% vs. 23%, p=0.006); and arterial hypotension (50% vs. 3%, p=0.004) were significantly higher in patients with MIS-C than in those without MIS-C. The frequencies of C-reactive protein levels >50 mg/L (83% vs. 25%, p=0.008) and D-dimer levels >1000 ng/mL (100% vs. 40%, p=0.007) and the median D-dimer, troponin T, and ferritin levels (p < 0.05) were significantly higher in patients with MIS-C. The frequencies of pediatric intensive care unit admission (100% vs. 60%, p=0.003), mechanical ventilation (83% vs. 7%, p < 0.001), vasoactive agent use (83% vs. 3%, p <0.001), shock (83% vs. 5%, p<0.001), cardiac abnormalities (100% vs. 2%, p <0.001), and death (67% vs. 3%, p < 0.001) were also significantly higher in patients with MIS-C. Similarly, the frequencies of oxygen therapy (100% vs. 33%, p=0.003), intravenous immunoglobulin therapy (67% vs. 2%, p < 0.001), aspirin therapy (50% vs. 0%, p < 0.001), and current acute renal replacement therapy (50% vs. 2%, p=0.002) were also significantly higher in patients with MIS-C. Logistic regression analysis showed that the presence of MIS-C was significantly associated with gastrointestinal manifestations [odds ratio (OR)=10.98; 95%CI (95% confidence interval)=1.20-100.86; p=0.034] and hypoxemia [OR=16.85; 95%CI=1.34-211.80; p=0.029]. Further univariate analysis showed a positive association between MIS-C and death [OR=58.00; 95%CI=6.39526.79; p <0 .0001]. CONCLUSIONS: Pediatric patients with laboratory-confirmed COVID-19 with MIS-C had a severe clinical spectrum with a high mortality rate. Our study emphasizes the importance of investigating MIS-C in pediatric patients with COVID-19 presenting with gastrointestinal involvement and hypoxemia.
  • article 5 Citação(ões) na Scopus
    Segmental cardiac strain assessment by two-dimensional speckle-tracking echocardiography in surviving MIS-c patients: Correlations with myocardial flow reserve (MFR) by 13 N-ammonia PET-CT
    (2022) LEAL, Gabriela Nunes; ASTLEY, Camila; LIMA, Marcos Santos; DINIZ, Maria de Fatima Rodrigues; LIANZA, Alessandro Cavalcanti; SAWAMURA, Karen Saori Shiraishi; MENEZES, Carolina Rocha Brito; SILVA, Camila Lino Martins Rodrigues da; BAIN, Vera; IMADA, Rodrigo; CHALELA, William; PEREIRA, Maria Fernanda Badue; MARQUES, Heloisa Helena de Sousa; BUCHPIGUEL, Carlos Alberto; GUALANO, Bruno; SILVA, Clovis Artur
    Background Multisystem inflammatory syndrome in children (MIS-c) is associated with severe cardiovascular impairment and eventually death. Pathophysiological mechanisms involved in myocardial injury were scarcely investigated, and cardiovascular outcomes are uncertain. Autopsy studies suggested that microvascular dysfunction may be relevant to LV impairment. Objective We aimed to evaluate segmental LV longitudinal strain by 2DST echocardiography and myocardial flow reserve (MFR) by 13 N-ammonia PET-CT, in six surviving MIS-c patients. Methods Each patient generated 34 LV segments for combined 2DST and MRF analysis. MFR was considered abnormal when <2, borderline when between 2 and 2.5 and normal when >2.5. Results From July 2020 to February 2021, six patients were admitted with MIS-c: three males, aged 9.3 (6.6-15.7) years. Time from admission to the follow-up visit was 6.05 (2-10.3) months. Although all patients were asymptomatic and LV EF was >= 55%, 43/102 (42.1%) LV segments showed MFR r = .36, p = .03 for basal segments; r = .41, p = .022 for mid segments; r = .42, p = .021 for apical segments. Median peak systolic longitudinal strain was different among MRF categories: 18% (12%-24%) for abnormal, 18.5% (11%-35%) for borderline, and 21% (12%-32%) for normal MFR (p = .006). Conclusion We provided preliminary evidence that surviving MIS-c patients may present subclinical impairment of myocardial microcirculation. Segmental cardiac strain assessment 2DST seems useful for MIS-c cardiovascular follow-up, given its good correlation with 13 N-ammonia PET-CT derived MFR.