Zilda Maria Alves Meira

Evaluation of secondary prophylactic schemes, based on benzathine penicillin G, for rheumatic fever in children

Serum concentrations of penicillin were measured in children with rheumatic fever. The adequacy of the values after administration of 1.2 million units of benzathine penicillin G every 2 or 3 weeks was confirmed; the adequacy of a 4-week regimen was questionable. The administration of 0.6 million units every 3 weeks was found to be inadequate to maintain serum levels high enough for the secondary prophylaxis of rheumatic fever.

Myocardial Fibrosis Progression in Duchenne and Becker Muscular Dystrophy: A Randomized Clinical Trial

Importance In Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD), interventions reducing the progression of myocardial disease could affect survival. Objective To assess the effect of early angiotensin-converting enzyme (ACE) inhibitor therapy in patients with normal left ventricular function on the progression of myocardial fibrosis (MF) identified on cardiovascular magnetic resonance (CMR). Design, Setting, and Participants A randomized clinical trial conducted in 2 centers included 76 male patients with DMD or BMD undergoing 2 CMR studies with a 2-year interval for ventricular function and MF assessment. In a non–intent-to-treat trial, 42 patients with MF and normal left ventricular ejection fraction (LVEF) were randomized (1:1) to receive or not receive ACE inhibitor therapy. The study was conducted from June 26, 2009, to June 30, 2012. Data analysis was performed from June 30, 2013, to October 3, 2016. Interventions Randomization (1:1) to receive or not receive ACE inhibitor therapy. Main Outcomes and Measures Primary outcome was MF progression from baseline to the 2-year CMR study. Results Of the 76 male patients included in the study, 70 had DMD (92%) and 6 had BMD (8%); mean (SD) age at baseline was 13.1 (4.4) years. Myocardial fibrosis was present in 55 patients (72%) and LV systolic dysfunction was identified in 13 patients (24%). Myocardial fibrosis at baseline was an independent indicator of lower LVEF at follow-up (coefficient [SE], −0.16 [0.07]; P = .03). Among patients with MF and preserved LVEF (42 [55%]), those randomized (21 patients in each arm) to receive ACE inhibitors demonstrated slower MF progression compared with the untreated group (mean [SD] increase of 3.1% [7.4%] vs 10.0% [6.2%] as a percentage of LV mass; P = .001). In multivariate analysis, ACE inhibitor therapy was an independent indicator of decreased MF progression (coefficient [SE], −4.51 [2.11]; P = .04). Patients with MF noted on CMR had a higher probability of cardiovascular events (event rate, 10 of 55 [18.2%] vs 0 of 21 [0%]; log-rank P = .04). Conclusions and Relevance In this 2-year, follow-up, randomized clinical trial of patients with Duchenne or Becker muscular dystrophy whose LVEF was preserved and MF was present as determined on CMR, ACE inhibitor therapy was associated with significantly slower progression of MF. The presence of MF was associated with worse patient prognosis. Trial Registration clinicaltrials.org Identifier: NCT02432885

Frequency and significance of vocalizations in Sydenham's chorea.

Sydenhams chorea (SC) is a complication of Streptococcus infection characterized by a combination of motor and non-motor features. We have investigated the presence of vocalizations in 89 consecutive patients with SC evaluated during a one-year period in the UFMG Movement Disorders Clinic. Seven (4/3 M/F) of the 89 patients (29/60 M/F) presented with simple vocalizations not preceded by premonitory sensations but in association with facial chorea in five patients. These findings suggest that vocalizations are not a common feature in SC and their phenomenology is quite distinct from the characteristics of vocal tics in tic disorders.

Echocardiographic prevalence of rheumatic heart disease in Brazilian schoolchildren: Data from the PROVAR study

BACKGROUND Accurate estimates of Rheumatic Heart Disease (RHD) burden are needed to justify improved integration of RHD prevention and screening into the public health systems, but data from Latin America are still sparse. OBJECTIVE To determine the prevalence of RHD among socioeconomically disadvantaged youth (5-18years) in Brazil and examine risk factors for the disease. METHODS The PROVAR program utilizes non-expert screeners, telemedicine, and handheld and standard portable echocardiography to conduct echocardiographic screening in socioeconomically disadvantaged schools in Minas Gerais, Brazil. Cardiologists in the US and Brazil provide expert interpretation according to the 2012 World Heart Federation Guidelines. Here we report prevalence data from the first 14months of screening, and examine risk factors for RHD. RESULTS 5996 students were screened across 21 schools. Median age was 11.9 [9.0/15.0] years, 59% females. RHD prevalence was 42/1000 (n=251): 37/1000 borderline (n=221) and 5/1000 definite (n=30). Pathologic mitral regurgitation was observed in 203 (80.9%), pathologic aortic regurgitation in 38 (15.1%), and mixed mitral/aortic valve disease in 10 (4.0%) children. Older children had higher prevalence (50/1000 vs. 28/1000, p<0.001), but no difference was observed between northern (lower resourced) and central areas (34/1000 vs. 44/1000, p=0.31). Females had higher prevalence (48/1000 vs. 35/1000, p=0.016). Age (OR=1.15, 95% CI:1.10-1.21, p<0.001) was the only variable independently associated with RHD findings. CONCLUSIONS RHD continues to be an important and under recognized condition among socioeconomically disadvantaged Brazilian schoolchildren. Our data adds to the compelling case for renewed investment in RHD prevention and early detection in Latin America.

Prognostic value of clinical and Doppler echocardiographic findings in children and adolescents with significant rheumatic valvular disease

Introduction: The diagnosis of acute rheumatic fever (RF) is based on clinical findings. However, during the chronic phase of the disease, the clinical approach is not sufficient for the follow-up of the patients and the Doppler echocardiography is a tool for the diagnosis of cardiac involvement. Prognostic variables that influence long-term outcomes are not well known. Methods: 462 patients with RF according to Jones criteria were studied, and followed-up from the initial attack to 13.6 ± 4.6 years. All patients underwent clinical assessment and Doppler echocardiography for the detection of heart valve involvement in the acute and chronic phases. Multivariate logistic regression analysis was used to identify the factors influencing long-term heart valve disease. Results: Carditis occurred in 55.8% and subclinical valvulitis in 35.3% patients. In the chronic phase, 33% of the patients had significant valvular heart disease. No normal Doppler echocardiography exam was observed on patients who had severe valvulitis, although heart auscultation had become normal in 13% of these. In the multivariate analysis, only the severity of carditis and the mitral and/or aortic valvulitis were associated with significant valvular heart disease. Chorea or arthritis were protective factors for significant valvular heart disease, odds ratio 0.41 (95% C.I. 0.22 – 0.77) and 0.43 (95% C.I. 0.23 – 0.82), respectively. Conclusions: Our study suggests that the use of Doppler echocardiography during RF helps to identify prognostic factors regarding the development of significant valvular heart disease. Initial severe carditis is an important factor in the long-term prognosis of chronic RHD, whereas arthritis and chore during the initial episode of RF appears to be protective. Strict secondary prophylaxis should be mandatory in high risk patients.

Estudo comparativo das avaliações clínica e ecocardiográfica Doppler na evolução das lesões valvares em crianças e adolescentes portadores de febre reumática

OBJECTIVE Compare clinical and Doppler echocardiographic evaluations in assessing valvular diseases in children and adolescents with rheumatic fever, as well as assess the progression of the disease in light of these assessments. METHODS This is a longitudinal study of 258 children and adolescents diagnosed with rheumatic fever according to Jones criteria. The follow-up period ranged from 2-15 years. The presence and quantification of valve diseases were determined by means of clinical and Doppler echocardiographic evaluations performed during the acute and chronic phases. The Kappa statistics method was used to estimate the degree of agreement between clinical and Doppler echocardiographic evaluations. Comparisons between clinical and Doppler echocardiographic findings on the progress of carditis and valvulitis, respectively, were made using chi-square test or Fishers exact test, p< 0.05. RESULTS Of the 109 patients who underwent Doppler echocardiographic evaluation during the acute phase, 31 did not present clinical evidence of carditis, but the Doppler echocardiograms of 17 (54.8%) of them showed valve lesions (subclinical valvulitis). During the chronic phase, 153 of the 258 patients had normal cardiovascular examination results; however, Doppler echocardiograms showed that 81 of them (52.9%) had valve lesions (subclinical chronic valvular diseases). Involution of the valvular lesions, as shown by Doppler echocardiographic evaluations, was less frequent and occurred in 10 (25.0%) patients with mild valvulitis, in only one (2.5%) patient with moderate valvulitis, and in none of the patients with severe valvulitis. CONCLUSION The identification of rheumatic fever valve lesions can be enhanced when clinical evaluations are supplemented by Doppler echocardiographic examinations; also, clinical examinations are not as suitable to detect valvular lesion regression as the echocardiography. The diagnosis of subclinical valvulitis and valvulopathy influences the secondary prophylaxis of rheumatic fever and endocarditis.

Prevalence of Rheumatic Heart Disease in a Public School of Belo Horizonte

Background Previous studies indicate that compared with physical examination, Doppler echocardiography identifies a larger number of cases of rheumatic heart disease in apparently healthy individuals. Objectives To determine the prevalence of rheumatic heart disease among students in a public school of Belo Horizonte by clinical evaluation and Doppler echocardiography. Methods This was a cross-sectional study conducted with 267 randomly selected school students aged between 6 and 16 years. students underwent anamnesis and physical examination with the purpose of establishing criteria for the diagnosis of rheumatic fever. They were all subjected to Doppler echocardiography using a portable machine. Those who exhibited nonphysiological mitral regurgitation (MR) and/or aortic regurgitation (AR) were referred to the Doppler echocardiography laboratory of the Hospital das Clínicas of the Universidade Federal of Minas Gerais (HC-UFMG) to undergo a second Doppler echocardiography examination. According to the findings, the cases of rheumatic heart disease were classified as definitive, probable, or possible. Results Of the 267 students, 1 (0.37%) had a clinical history compatible with the diagnosis of acute rheumatic fever (ARF) and portable Doppler echocardiography indicated nonphysiological MR and/or AR in 25 (9.4%). Of these, 16 (6%) underwent Doppler echocardiography at HC-UFMG. The results showed definitive rheumatic heart disease in 1 student, probable rheumatic heart disease in 3 students, and possible rheumatic heart disease in 1 student. Conclusion In the population under study, the prevalence of cases compatible with rheumatic involvement was 5 times higher on Doppler echocardiography (18.7/1000; 95% CI 6.9/1000-41.0/1000) than on clinical evaluation (3.7/1000-95% CI).

Rheumatic Fever prevention program: long-term evolution and outcomes.

This investigation aims to analyze the profile of long-term evolution of rheumatic fever in children and adolescents and outcomes after the control of recurrences. The cohort involved 702 patients followed from 1.3 to 16.9 years covering the two periods, before and after the implementation of a prevention program. Besides the establishment of the Reference Center in the State of Minas Gerais and the implementation of strategies to promote the compliance to prophylaxis, a project for education of health professionals was carried out in 23 cities. In addition to the clinical and epidemiological profile, the severity of the disease was analyzed. Mixed lesions were found in 27.1%, valvar regurgitation in 72.9%, and complete regression of the valvar lesions was seen in 34.4% of the patients, mostly presenting mild dysfunctions. The recurrence rate per patient-year was 0.058 and out of a total of 85 recurrences, 21.4% occurred in the first and 7.5% in the second period. More severe degrees of carditis and significant valvar sequels presented a higher prevalence in patients with recurrences. The comparative analysis between the two periods showed no changes regarding the age at the primary attack, gender, type, and site of valvar lesions and affected joints; however, important modifications in the indices of severity were observed after the control of recurrences. A significant decrease in the prevalence of severe carditis, obstructive valvar sequels, hospital admissions, surgical approach, and deaths was seen. This investigation showed that although the clinical profile of presentation remains unchanged, the control of repeated attacks can improve the morbimortality rates. In this context, the secondary prophylaxis should be the first priority in the control of the disease in developing countries, taking into account the difficulties found for effective primordial and primary prevention.

Comparison between different strategies of rheumatic heart disease echocardiographic screening in Brazil: Data from the PROVAR (Rheumatic Valve Disease Screening Program) study

Background Considering the limited accuracy of clinical examination for early diagnosis of rheumatic heart disease (RHD), echocardiography has emerged as an important epidemiological tool. The ideal setting for screening is yet to be defined. We aimed to evaluate the prevalence and pattern of latent RHD in schoolchildren (aged 5–18 years) and to compare effectiveness of screening between public schools, private schools, and primary care centers in Minas Gerais, Brazil. Methods and Results The PROVAR (Rheumatic Valve Disease Screening Program) study uses nonexperts and portable and handheld devices for RHD echocardiographic screening, with remote interpretation by telemedicine, according to the 2012 World Heart Federation criteria. Compliance with study consent and prevalence were compared between different screening settings, and variables associated with RHD were analyzed. In 26 months, 12 048 students were screened in 52 public schools (n=10 901), 2 private schools (n=589), and 3 primary care centers (n=558). Median age was 12.9 years, and 55.4% were girls. Overall RHD prevalence was 4.0% borderline (n=486) and 0.5% definite (n=63), with statistically similar rates between public schools (4.6%), private schools (3.5%), and primary care centers (4.8%) (P=0.24). The percentage of informed consents signed was higher in primary care centers (84.4%) and private schools (66.9%) compared with public schools (38.7%) (P<0.001). Prevalence was higher in children ≥12 years (5.3% versus 3.1%; P<0.001) and girls (4.9% versus 4.0%; P=0.02). Only age (odds ratio, 1.12; 95% confidence interval, 1.09–1.17; P<0.001) was independently associated with RHD. Conclusions RHD screening in primary care centers seems to achieve higher coverage rates. Prevalence among schoolchildren is significantly high, with rates higher than expected in private schools of high‐income areas. These data are important for the formulation of public policies to confront RHD.

Accuracy of chest radiography for positioning of the umbilical venous catheter

OBJECTIVES To evaluate the accuracy of the simultaneous analysis of three radiographic anatomical landmarks - diaphragm, cardiac silhouette, and vertebral bodies - in determining the position of the umbilical venous catheter distal end using echocardiography as a reference standard. METHODS This was a cross-sectional, observational study, with the prospective inclusion of data from all neonates born in a public reference hospital, between April 2012 and September 2013, submitted to umbilical venous catheter insertion as part of their medical care. The position of the catheter distal end, determined by the simultaneous analysis of three radiographic anatomical landmarks, was compared with the anatomical position obtained by echocardiography; sensitivity, specificity, positive predictive value, negative predictive value, and accuracy were calculated. RESULTS Of the 162 newborns assessed by echocardiography, only 44 (27.16%) had the catheter in optimal position, in the thoracic portion of the inferior vena cava or at the junction of the inferior vena cava with the right atrium. The catheters were located in the left atrium and interatrial septum in 54 (33.33%) newborns, in the right atrium in 26 (16.05%), intra-hepatic in 37 (22.84%), and intra-aortic in-one newborn (0.62%). The sensitivity, specificity and accuracy of the radiography to detect the catheter in the target area were 56%, 71%, and 67.28%, respectively. CONCLUSION Anteroposterior radiography of the chest alone is not able to safely define the umbilical venous catheter position. Echocardiography allows direct visualization of the catheter tip in relation to vascular structures and, whenever possible, should be considered to identify the location of the umbilical venous catheter.