Difficulties in emotion regulation and well-being in breast cancer.

Objective: Breast cancer is responsible for disruptive changes in women's lives, causing them to experience diverse and intense negative emotions that can affect their perception of well-being. The present study aimed to characterize difficulties in emotion regulation (ER), according to Gratz and Roemer's multidimensional assessment, in women with breast cancer and to relate them with General Well-Being and its different domains: Physical, Social/Familial, Emotional, and Functional. Method: Ninety-five Portuguese women with breast cancer aged between 32 and 75 years answered a sociodemographic and clinical questionnaire and the Portuguese versions of the Difficulties in Emotion Regulation Scale and the Functional Assessment of Cancer Therapy - General. Data were collected in an oncology public hospital. Results: In general, difficulties in ER presented negative correlations with General Well-Being and its domains. The multiple regression analysis findings indicated that two specific types of difficulties, Limited Access to ER Strategies and Lack of Emotional Clarity, play a significant role in predicting well-being, especially in the Emotional domain, which was most compromised in these patients. Conclusions: These difficulties should be approached within psycho-oncological interventions as they are essential contributors to improving emotional and general well-being and fostering psychological adaptation to breast cancer.

Study of 138 vulvar lichen sclerosus patients and the malignant risk transformation.

Objective: To report the prevalence of malignant transformation of vulvar lichen sclerosus (VLS) and possible risk factors. Methods: This is a cohort study with data analysis from medical records of 138 patients with histological diagnosis of VLS registered at the Vulvar Pathology Outpatient Clinic of the University Hospital, between 2007 and 2017. Predominance of risk factors was performed using logistic regression analysis. The variables studied were the length of follow-up, age, regular or irregular follow up; presence of symptoms (dyspareunia, pruritus and/or vulvar burning); histology characteristics, the presence of epithelial hyperplasia; and the presence of autoimmune diseases. Results: There were 138 patients included in the study, and among them five progressed to malignant transformation. The patients had a median age of 59 years and 83% were symptomatic. The most frequent symptom was itching with 72%. Autoimmune diseases were present in 11.6%, the most prevalent being thyroid disease. All five case of malignant transformation (0.6%) had an irregular follow up. The logistic regression analysis was used among the studied variables, and no statistical significance was found among them (p ≥ 0.05). The relationship between hyperplasia and the clinical outcome of malignant transformation, in which non-significant but acceptable p value close to 0.05 was observed. Conclusion: The prevalence of malignant transformation in patients with VLS was 0.6%, and common factors were the lack of adherence to medical treatments and the loss of follow-up.

Do Acoustic Characteristics of Dysarthria in People With Parkinson's Disease Differ Across Languages?

PURPOSE: Cross-language studies suggest more similarities than differences in how dysarthria affects the speech of people with Parkinson's disease (PwPD) who speak different languages. In this study, we aimed to identify the relative contribution of acoustic variables to distinguish PwPD from controls who spoke varieties of two Romance languages, French and Portuguese. METHOD: This bi-national, cross-sectional, and case-controlled study included 129 PwPD and 124 healthy controls who spoke French or Portuguese. All participants underwent the same clinical examinations, voice/speech recordings, and self-assessment questionnaires. PwPD were evaluated off and on optimal medication. Inferential analyses included Disease (controls vs. PwPD) and Language (French vs. Portuguese) as factors, and random decision forest algorithms identified relevant acoustic variables able to distinguish participants: (a) by language (French vs. Portuguese) and (b) by clinical status (PwPD on and off medication vs. controls). RESULTS: French-speaking and Portuguese-speaking individuals were distinguished from each other with over 90% accuracy by five acoustic variables (the mean fundamental frequency and the shimmer of the sustained vowel /a/ production, the oral diadochokinesis performance index, the relative sound level pressure and the relative sound pressure level standard deviation of the text reading). A distinct set of parameters discriminated between controls and PwPD: for men, maximum phonation time and the oral diadochokinesis speech proportion were the most significant variables; for women, variables calculated from the oral diadochokinesis were the most discriminative. CONCLUSIONS: Acoustic variables related to phonation and voice quality distinguished between speakers of the two languages. Variables related to pneumophonic coordination and articulation rate were the more effective in distinguishing PwPD from controls. Thus, our research findings support that respiration and diadochokinesis tasks appear to be the most appropriate to pinpoint signs of dysarthria, which are largely homogeneous and language-universal. In contrast, identifying language-specific variables with the speech tasks and acoustic variables studied was less conclusive.

Position Statement on the Use of Myocardial Strain in Cardiology Routines by the Brazilian Society of Cardiology's Department Of Cardiovascular Imaging - 2023. / Posicionamento do Departamento de Imagem Cardiovascular da Sociedade Brasileira de Cardiologia sobre o Uso do Strain Miocárdico na Rotina do Cardiologista ­ 2023.

Central Illustration : Position Statement on the Use of Myocardial Strain in Cardiology Routines by the Brazilian Society of Cardiology's Department Of Cardiovascular Imaging - 2023 Proposal for including strain in the integrated diastolic function assessment algorithm, adapted from Nagueh et al.67 Am: mitral A-wave duration; Ap: reverse pulmonary A-wave duration; DD: diastolic dysfunction; LA: left atrium; LASr: LA strain reserve; LVGLS: left ventricular global longitudinal strain; TI: tricuspid insufficiency. Confirm concentric remodeling with LVGLS. In LVEF, mitral E wave deceleration time < 160 ms and pulmonary S-wave < D-wave are also parameters of increased filling pressure. This algorithm does not apply to patients with atrial fibrillation (AF), mitral annulus calcification, > mild mitral valve disease, left bundle branch block, paced rhythm, prosthetic valves, or severe primary pulmonary hypertension.

Figura Central : Posicionamento do Departamento de Imagem Cardiovascular da Sociedade Brasileira de Cardiologia sobre o Uso do Strain Miocárdico na Rotina do Cardiologista ­ 2023 Proposta de inclusão do strain no algoritmo integrado de avaliação da função diastólica, adaptado e traduzido de Nagueh et al. 67 AE: átrio esquerdo; Ap: duração da onda A reversa pulmonar; Am: duração da onda A mitral; DD: disfunção diastólica; FEVEr: fração de ejeção do ventrículo esquerdo reduzida; IT: insuficiência tricúspide; SAEr: strain do AE de reservatório; SLGVE: strain longitudinal global do ventrículo esquerdo. Se remodelamento concêntrico, confirmar com SLGVE. Na presença de FEVEr, tempo de desaceleração da onda E mitral (TDE) < 160 ms e onda S < D pulmonar também são parâmetros de pressão de enchimento aumentada. Esse algoritmo não se aplica a pacientes com fibrilação atrial (FA), calcificação do anel mitral ou valvopatia mitral maior que discreta, bloqueio de ramo esquerdo (BRE), ritmo de marca-passo, próteses valvares ou hipertensão pulmonar (HP) primária grave.

Association of Swede Score and 2011 IFCPC Nomenclature in Women with Abnormal Cytology / A Associação entre o escore Swede e a nomenclatura IFCPC 2011 em mulheres com citologia anormal

Abstract Objective To assess the association between two colposcopic indices, the Swede score and the 2011 International Federation of Cervical Pathology and Colposcopy (IFCPC) Nomenclature as well as to determine the efficacy of the Swede score with cutoffs of 7 and 8. Methods In the present cross-sectional pilot study, 34 women who had at least 1 colposcopy-directed biopsy due to abnormal cytology were enrolled. The colposcopic findings were scored by both the Swede score and the 2011 IFCPC Nomenclature and were compared with each other. The Kappa coefficient and the McNemar test were used. Accuracy, sensitivity, specificity, and positive and negative predictive values (NPV and PPV, respectively) were calculated, as well as the effectiveness with cutoffs of 7 and 8 in identifying cervical intraepithelial neoplasm (CIN) 2+ when using the Swede score. Results The correlation between the 2 colposcopic indices was 79.41%. The Kappa coefficient and the McNemar p-value were 0.55 and 0.37, respectively. The IFCPC Nomenclature had sensitivity, specificity, accuracy, PPV, and NPV of 85.71, 55.00, 67.64, 57.14, and 84.61%, respectively. The Swede score had sensitivity, specificity, accuracy, PPV, and NPV of 100, 63.15, 79.41, 68.18, and 100%, respectively. A Swede score cutoff of 7 for CIN 2+ detection had a specificity of 94.73%, while with a cutoff of 8 it increased to 100%. The sensitivity for both values was 60%. The PPV and NPV for cutoffs of 7 and 8 were 90 and 75 and 100 and 76%, respectively. Conclusion Although both colposcopic indices have good reproducibility, the Swede score showed greater accuracy, sensitivity, and specificity in identifying CIN 2 + , especially when using a cutoff of 8.

Resumo Objetivo Avaliar a associação entre dois índices colposcópicos, o escore Swede e a Nomenclatura International Federation of Cervical Pathology and Colposcopy (IFCPC, na sigla em inglês) 2011, assim como determinar a eficácia do escore Swede com os pontos de corte 7 e 8. Métodos Trata-se de um estudo transversal, com 34 mulheres incluídas, que realizaram colposcopia com biópsia dirigida devido a uma citologia anormal. Os achados colposcópicos foram categorizados pelo escore Swede e pela Nomenclatura IFCPC 2011 e comparados um com o outro. Foram avaliados o coeficiente Kappa e o teste de McNemar e foram calculados a acurácia, a sensibilidade, a especificidade e valores preditivos negativos e positivos (VPN e VPP, respectivamente) de cada índice, assim como a eficácia com os pontos de corte 7 e 8 do escore Swede para determinar as lesões de neoplasia intraepitelial cervical (NIC) 2 + . Resultados A concordância entre os 2 índices foi de 79,41% e o coeficiente Kappa e o valor-p do teste de McNemar foram 0.55 e 0.37, respectivamente. Pela Nomenclatura IFCPC 2011, obtivemos como sensibilidade, especificidade, acurácia, VPP e VPN, respectivamente: 85,71, 55,00, 67,64, 57,14 e 84,61%. Pelo escore Swede obtivemos como sensibilidade, especificidade, acurácia, VPP e VPN, respectivamente: 100, 63,15, 79,41, 68,18 e 100%. O uso do escore Swede para detecção das lesões NIC 2+ obteve como especificidade 94,73% com o valor de corte de 7, enquanto o valor de corte 8 obteve 100%. A sensibilidade para ambos os cortes foi de 60%. O VPP e o VPN com os cortes 7 e 8 foram, respectivamente: 90,00 e 75,00 e 100,00 e 76,00%. Conclusão Ambos os índices colposcópicos tiveram boa reprodutibilidade; no entanto, o escore Swede mostrou melhor acurácia, sensibilidade e especificidade em identificar as lesões NIC 2+ e o melhor ponto de corte para identificar as lesões NIC2+ foi com o valor 8.

Limitation of cytology and the impact on reduction of cervical cancer / Limitação da citologia e o impacto na redução do câncer de colo uterino

Introduction: Cervical cancer is the third most common malignant tumor in the female population and the fourth cause of death from cancer in women in Brazil. The squamocolumnar junction and the transformation zone concentrate 90% of pre-invasive and invasive cervical lesions. Objective: To evaluate the prevalence of cytology without cells of the squamocolumnar junction and feasibility of active search. Methods: Cross-sectional study at a university hospital between 2017 and 2018. The prevalence of cytology without squamocolumnar junction cells was calculated. A convenience sample was obtained and mean age and relationship with presence of transformation zone cells were calculated. An active search was performed and cytology collected, with estrogen preparation if indicated. Medical records of the other women were analyzed. Results: Squamocolumnar junction cells were not found in 28.84% of samples. Mean age was 53 years, without association with presence of squamocolumnar junction cells (p=0.409). Seventy-six women returned, 36 of which (47.37%) used estrogen. Level 2 or 3 cervical intraepithelial neoplasia, microinvasive carcinoma or cancer was not identified. A total of 134 medical records were analyzed; only 36 women (26.87%) completed screening. Conclusions: The presence of squamocolumnar junction cells indicates quality of cytology; the use of estrogen in postmenopausal women favors its collection. There were difficulties in active search. An immediate repetition of cytology should be considered.

Introdução: O câncer de colo uterino é o terceiro tumor maligno mais frequente na população feminina e a quarta causa de morte de mulheres por câncer no Brasil. A junção escamo-colunar e a zona de transformação concentram 90% das lesões pré-invasoras e invasoras cervicais. Objetivo: Avaliar prevalência de colpocitologias sem células da junção escamo-colunar e a viabilidade de busca ativa. Métodos: Estudo transversal em hospital universitário entre 2017 e 2018. Calculada prevalência de citologias sem células da junção escamo-colunar. Obtida amostra por conveniência, calculada média de idade e relação com a presença da junção escamo-colunar. Realizada busca ativa e colhidas citologias com preparo estrogênico, se indicado. Analisados os prontuários das demais mulheres. Resultados: A prevalência de ausência de células da junção escamo-colunar foi de 28,84%. A média de idade foi 53 anos, sem associação com presença da junção escamo-colunar (p=0,409). Retornaram 76 mulheres e 36 (47,37%) usaram estrogênio. Não identificamos neoplasia intraepitelial cervical graus 2 ou 3, carcinoma microinvasor e câncer. Analisados 134 prontuários, dos quais apenas 36 mulheres (26,87%) concluíram o rastreio. Conclusões: A presença de células da junção escamo-colunar indica qualidade da coleta, e o uso de estrogênio na pós-menopausa favorece sua obtenção. Houve dificuldade de busca ativa. A repetição imediata da citologia deve ser considerada.

Chikungunya associated vulvitis: case report and literature review / Vulvite associada a Chikungunya: relato de caso e revisão de literatura

Introduction: Chikungunya virus is spreading worldwide due to migration and globalization and could be presented with systemic and with unusual symptoms. Objective: To report a case of virus-transmitted infection detected in a woman during the gynecological examination at a vulvar clinic. Case report: A 73-year-old Caucasian woman attended a vulvar clinic because of dyspareunia and vulvar burning. Ulcers were observed on labia minora and perineum. A Chikungunya was diagnosed by seroconversion in paired specimens. She was prescribed prednisolone 40 mg once a day for 10 days. After oral steroid treatment, the woman had no body rashes or lesions on her genitals. Conclusion: This study emphasized that rare signs of unusual vulvitis with ulcers could be associated with Chikungunya infection.

Introdução: O vírus Chikungunya está se espalhando pelo mundo por conta da migração e da globalização, podendo apresentar sintomas sistêmicos e incomuns. Objetivo: Relatar um caso de infecção pelo vírus detectado em uma mulher por ocasião do exame ginecológico em clínica de patologia vulvar. Relato do caso: Uma mulher caucasiana de 73 anos foi a uma clínica vulvar por causa de dispareunia e queimação vulvar. Úlceras foram observadas nos pequenos lábios e no períneo. O diagnóstico de Chikungunya foi realizado por soroconversão em espécimes pareados. Foi prescrita prednisolona 40 mg uma vez ao dia por dez dias. Após o tratamento com esteróides orais, a mulher não apresentou erupções ou lesões nos órgãos genitais. Conclusão: Este estudo enfatizou que quadros raros de vulvite com úlcera podem estar associados à infecção por Chikungunya.

Clinical and Epidemiological Profiles of Patients Admitted to a Pediatric Cardiac Intensive Care Unit

Abstract Background: Congenital and acquired heart diseases are important causes of morbidity and mortality in children. In critical congenital heart defects, when treatment is not adequate, clinical manifestations may lead to death in the neonatal period. Objective: To establish the clinical and epidemiological profile of patients admitted to the pediatric cardiac intensive care unit (UTI) in a tertiary hospital. Methods: This was a cross-sectional study conducted from January 2013 to December 2014, based on analysis of patients' medical records. The study sample was composed of 307 children and adolescents with congenial and acquired heart diseases. The score Risk Adjustement for Congenital Heart Surgery 1 (RACHS-1) was used for categorization of the various surgical procedures. Descriptive statistics were calculated using the Satistical Package for Social Sciences (SPSS). Categorical variables were compared using the Pearson's chi-square test, considering a level of significance of 5%. Results: There was a predominance of patients aged between 28 days and one year (44%). Congenital heart diseases (91.9%) prevailed over acquired heart diseases (8.1%). Extracorporeal circulation was used in 138 patients who underwent surgical procedures, lasting from 12 to 261 minutes. Most patients (88.9%) were discharged from the ICU and 11.1% died. Using the score RACHS-1, corrective cardiac surgery was performed in 75.8% and paliative surgery in 24.2% of the patients. Conclusions: Patients aged between 28 days to one year, with cyanotic congenital heart disease, undergoing cardiac surgery with extracorporeal circulation duration longer than 120 minutes are at a higher risk of death.

Características clínicas e demográficas dos pacientes com endocardite infecciosa em um serviço de cardiologia pediátrica / Clinical and demographic characteristics of patients with infective endocarditis treated at a pediatric cardiology service / Características clínicas y demográficas de los pacientes con endocarditis infecciosa en un servicio de cardiología pediátrica

A endocardite infecciosa, um processo infeccioso da superfície do endocárdio, vem apresentando maiores taxas de incidência na população pediátrica, sendo considerada importante causa de mortalidade. A partir deste trabalho, busca-se descrever as principais características clínicas e demográficas dos pacientes com endocardite e avaliar os fatores preditores de óbito nessa população. Trata-se de um estudo de coorte retrospectiva, baseado na avaliação de prontuários do período de 2010 a 2016, em hospital terciário. A amostra contou com pacientes menores de 18 anos com diagnóstico de endocardite, pelos critérios de Duke. Os dados foram analisados com o programa estatístico SPSS 20.0. Para a análise inferencial bivariada foi utilizado o teste do qui-quadrado. Valores de p < 0,05 foram considerados estatisticamente significantes. O grupo de estudo incluiu 31 pacientes; 61,3% do sexo masculino. Todos os pacientes tinham cardiopatia prévia, sendo 74,2% cardiopatia congênita e 25,8% cardiopatia reumática crônica. Cirurgia prévia esteve presente em 45,2%; hemocultura positiva em 54,8%; vegetações visíveis ao ecocardiograma transtorácico em 83,9% e complicações em 38,7%, sendo a embolização a principal complicação (22,6%). Houve associação, estatisticamente significante, entre prótese valvar como sítio de endocardite e embolização, quando associadas ao óbito, com proporções de 100% (p = 0,001) e 60% (p = 0,029), respectivamente. A taxa de mortalidade foi de 16,1%. Conclui-se que a cardiopatia prévia é o principal fator predisponente para a endocardite infecciosa, principalmente cardiopatia congênita. Contudo, a cardiopatia reumática crônica ainda é um fator de risco importante. A mortalidade da endocardite infecciosa é elevada, e os preditores são: prótese valvar como sítio da endocardite e embolização sistêmica.

Infective endocarditis, an infectious process of the endocardial surface, has shown higher incidence rates in the pediatric population, constituting an important cause of mortality. Thus, this retrospective cohort study describes the main clinical and demographic characteristics of endocarditis patients, and evaluates the predictors of death in this population. Data were collected from medical records produced by a tertiary hospital, from 2010 to 2016. The sample included patients under 18 years of age diagnosed with endocarditis according to Duke's criteria. Data were analyzed using SPSS 20.0 statistical program. Bivariate inferential analysis used the chi-squared test. Values p <0.05 were considered statistically significant. The study group included 31 patients, 61.3% male. All patients had prior heart disease, with 74.2% having congenital heart disease and 25.8% having chronic rheumatic heart disease. Of the 31 patients, 45.2% presented prior surgery, 54.8% had positive blood culture, 83.9% showed vegetations visible on transthoracic echocardiography, and 38.7% presented complications, with embolization being the main one (22.6%). Results showed a statistically significant association between valvular prosthesis as an endocarditis site and embolization when associated with mortality at 100% (p = 0.001) and 60% (p = 0.029) proportions, respectively. Mortality rate was 16.1%. In conclusion, prior heart disease is the main predisposing factor for endocarditis, mainly congenital heart disease. However, chronic rheumatic heart disease remains an important risk factor. Mortality of endocarditis is high, having valvar prosthesis as an endocarditis site and systemic embolization as predictors.

La endocarditis infecciosa es una infección en la superficie del endocardio, la cual se viene presentando mayores tasas de incidencia en la población pediátrica y es considerada una importante causa de mortalidad. En este trabajo se pretende describir las principales características clínicas y demográficas de los pacientes con endocarditis, así como evaluar los factores predictores de defunción en esa población. Se realizó una cohorte retrospectiva, basada en la evaluación de los historiales de los pacientes en el período de 2010 a 2016, en un hospital terciario. La muestra constó de pacientes menores de 18 años con el diagnóstico de endocarditis por los criterios de Duke. Los datos se analizaron mediante el programa estadístico SPSS 20.0. Para el análisis inferencial bivariado se utilizó la prueba del chi-cuadrado. Los valores de p <0,05 fueron considerados estadísticamente significantes. El grupo del estudio incluyó a 31 pacientes, 61,3% del sexo masculino. Todos los pacientes tenían cardiopatía previa, el 74,2% cardiopatía congénita y el 25,8% cardiopatía reumática crónica. La cirugía previa estuvo presente en el 45,2%, hemocultura positiva en el 54,8%, vegetaciones visibles al ecocardiograma transtorácico en el 83,9% y complicaciones en el 38,7%, con la embolización como la principal complicación (22,6%). Se observó una asociación estadísticamente significativa entre prótesis valvular como sitio de endocarditis y embolización cuando la asociaron a la muerte, con proporciones del 100% (p=0,001) y del 60% (p=0,029), respectivamente. La tasa de mortalidad fue del 16,1%. Por lo tanto, la cardiopatía previa es el principal factor predisponente para la endocarditis, principalmente la cardiopatía congénita. Sin embargo, la cardiopatía reumática crónica sigue siendo un factor de riesgo importante. La mortalidad de la endocarditis es elevada, y sus predictores son prótesis valvular como sitio de la endocarditis y embolización sistémica.

Left ventricular assessment in patients with mucopolysaccharidosis using conventional echocardiography and myocardial deformation by two-dimensional speckle-tracking method / Avaliação do ventrículo esquerdo em pacientes com mucopolissacaridose através do ecocardiograma convencional e da deformação miocárdica pelo speckle-tracking bidimensional

Abstract Objective: Mucopolysaccharidosis is a rare genetic disease characterized by the intralysosomal deposition of glycosaminoglycans. Cardiovascular impairment is a common feature. Cardiac signs and symptoms are underestimated due to the disease involvement in other organs. Enzyme replacement therapy can be used in mucopolysaccharidosis I, II, IV, and VI. Thus, the knowledge about the use of new echocardiography tools is relevant to improve the care of this population. This study aimed to describe left ventricular function assessment by conventional echocardiography and left ventricular global longitudinal strain analysis and compare the alterations in patients receiving enzyme replacement therapy and who had different ages at the start of therapy. Method: Outpatient-based descriptive study. The patients were submitted to conventional echocardiography and left ventricular global longitudinal strain measurement. Results: Sixteen patients were evaluated; median age of 14.2 years (SD = 5.2 years). Left ventricular hypertrophy was found in nine patients (56.2%). All patients had preserved left ventricular systolic function (Simpson and Teichholz). Nine (56.2%) patients showed alterations in left ventricular global longitudinal strain. The study showed a positive association between left ventricular hypertrophy and alteration in the left ventricular global longitudinal strain, and late start of enzyme replacement therapy and alteration in the left ventricular global longitudinal strain. Conclusion: Echocardiographic alterations in patients with mucopolysaccharidosis were frequently observed, especially alterations in the left ventricular geometry and subclinical dysfunction. Patients who had a late enzyme replacement therapy start showed an association with worse left ventricular global longitudinal strain values, reinforcing the need for early diagnosis and treatment. The use of new echocardiographic tools may improve the follow-up of these patients.

Resumo Objetivo: A mucopolissacaridose é uma doença genética rara, caracterizada por depósito intralisossômico de glicosaminoglicanos. O comprometimento cardiovascular é frequente. Sinais e sintomas cardíacos são subestimados pelo envolvimento da doença em outros órgãos. A terapia de reposição enzimática pode ser usada em mucopolissacaridose I, II, IV e VI. Assim, o conhecimento da aplicação de novas ferramentas de ecocardiografia é relevante para melhorar a assistência dessa população. Este estudo visou descrever a função do ventrículo esquerdo pelo ecocardiograma convencional e pela análise do strain global longitudinal do ventrículo esquerdo e comparar as alterações em pacientes que fazem uso da terapia de reposição enzimática e que tiveram idades distintas de início da terapia. Método: Estudo descritivo de base ambulatorial. Os pacientes foram submetidos à ecocardiografia convencional e medida do strain global longitudinal do ventrículo esquerdo. Resultados: Foram avaliados 16 pacientes; mediana de 14,2 anos (desvio: 5,2 anos). Hipertrofia do ventrículo esquerdo foi encontrada em nove pacientes (56,2%). Todos os pacientes tiveram função sistólica do ventrículo esquerdo preservada (Simpson e Teichholz). Nove (56,2%) pacientes apresentaram alteração no strain global longitudinal do ventrículo esquerdo. O estudo mostrou associação positiva entre hipertrofia do ventrículo esquerdo e alteração no strain global longitudinal do ventrículo esquerdo e início tardio da terapia de reposição enzimática e alteração no strain global longitudinal do ventrículo esquerdo. Conclusão: Alterações ecocardiográficas em pacientes com mucopolissacaridose foram frequentes, especialmente alterações na geometria e disfunção subclínica do ventrículo esquerdo. Pacientes que iniciaram tardiamente a terapia de reposição enzimática apresentaram associação com piores valores de strain global longitudinal do ventrículo esquerdo, o que reforça a necessidade do diagnóstico e tratamento precoces. O uso de novas ferramentas de ecocardiografia pode melhorar o acompanhamento desses pacientes.

Brazilian fetal cardiology guidelines ­ 2019 / Diretriz brasileira de cardiologia fetal ­ 2019

Over the years, Fetal Cardiology have been incorporated into the daily practice of Pediatric Cardiology. What was once restricted to a few fetal heart researchers, has slowly been incorporated into health institutions that deal with congenital heart diseases (CHD). Fetal echocardiography has generated extensive knowledge of the natural and modified history of heart diseases in utero, and normal fetal heart physiology and anatomy. The benefits of fetal diagnosis have become unquestionable over the years. Pioneers in the area succeeded in demystifying the fetal heart examination and proving the importance of screening for cardiac abnormalities during obstetric examinations. Prenatal detection rates have increased, and interest in fetal echocardiography is, thus, no longer merely a diagnostic tool; it has gone on to become a tool of the utmost importance in assisting medical and, progressively, interventional treatment of specific anomalies that occur in fetal life. A vast body of literature currently supports the practice of Fetal Cardiology. In addition to diagnosis, anatomical and functional particularities may be identified in utero, with implications on the delivery planning and pre and postnatal management. Prenatal diagnosis has certainly led to increase the number of babies with complex heart diseases in Pediatric Cardiology hospital beds. Prior to this, children with complex heart diseases did not survive the immediate neonatal period and died in neonatal intensive care units without being diagnosed. Nowadays, these children require increasingly careful and specific management involving Pediatric Cardiology and thus modifying the practice of Neonatal Cardiology. Despite the vast literature pertinent to Fetal Cardiology, due to the restricted number of cases, there is a lack of studies with large populations and randomization processes, being the information based on observational studies and description of small samples or cases reports. However, the accumulated knowledge is already enough to develop scientific statements or guidelines. In April 2014, the American Heart Association (AHA) published the first scientific statement for Fetal Cardiology, encompassing all the practical aspects involved in this area, including screening, diagnosis, medical or interventional therapy, counseling, delivery planning, and neonatal treatment. Considering this extremely thorough and highly useful document, we have accepted the challenge of bringing together professionals dedicated to Fetal Cardiology from different regions of Brazil in order to jointly establish guidelines which are adapted to our reality and which also take into consideration knowledge created in Brazil. We believe that the information brought together in this document will be of great use to professionals who face the challenge of dealing with possible abnormalities that affect the fetal heart in their daily practice. (AU)

Saberes e práticas sobre controle do Aedes aegypti por diferentes sujeitos sociais na cidade de Salvador, Bahia, Brasil / Knowledge and practices in Aedes aegypti control among different social subjects in Salvador, Bahia State, Brazil / Conocimientos y prácticas sobre el control del Aedes aegypti realizado por diferentes sujetos sociales en la ciudad de Salvador, Bahía, Brasil

Resumo: Devido à persistência da dengue e de outras arboviroses no Brasil, o poder público tem intensificado as ações de combate ao mosquito vetor Aedes aegypti. Os agentes de combate às endemias (ACE) e agentes comunitários de saúde (ACS), dentre outras atribuições, tornaram-se interlocutores e disseminadores de conhecimento na comunidade. O objetivo deste trabalho foi analisar os saberes e práticas sobre controle da dengue por diferentes sujeitos sociais: moradores e agentes. Foram realizadas entrevistas com moradores, ACE de campo e de mobilização e ACS em dois bairros de Salvador, Bahia, por meio da metodologia de grupo focal. Os moradores demonstraram incerteza sobre a forma de contágio e o perigo da dengue. Os ACE de campo apresentam-se em conflito, pela necessidade de informar à comunidade sobre aspectos que não dominam e demonstram um descontentamento pessoal no trabalho com um sentimento de desvalorização pela falta de qualificação. Os ACE de mobilização culpam a população e enfatizam a importância de si próprios como solução para o controle da dengue. Os ACS não apropriaram sua experiência de campo em seu discurso e se sentem desobrigados com respeito ao controle vetorial. Todos os grupos entrevistados concordam que a culpa da dengue recai sobre o poder público, e a solução para o problema está na educação. Percebe-se uma grande necessidade de intervenções educativas regulares, pautadas no diálogo e na sensibilização para lidar com a realidade cotidiana dos moradores, trazendo os indivíduos (moradores e agentes) como sujeitos do processo de construção de conhecimento. Pois, na metodologia atual, a disseminação de informação e conhecimento não é suficiente para promover melhorias na comunidade para o controle da dengue.

Abstract: Due to the persistence of dengue and other arbovirus infections in Brazil, the government has stepped up measures to combat the Aedes aegypti mosquito vector. The responsibilities of community endemic disease workers (CEDW) and community health workers (CHW) include acting as intermediaries and disseminating knowledge in the community. The aim of this study was to analyze knowledge and practices in dengue control by different social subjects: residents and CEDW/CHW. Interviews were held with residents, field and mobilization CEDW, and CHW in two neighborhoods in Salvador, Bahia State, using focus groups. Residents expressed uncertainty on the form of transmission and hazards of dengue. Field CEDW voiced conflicting feelings due to the need to inform the community on issues over which they lack any control, while expressing personal dissatisfaction with their work and a feeling of underappreciation due to their lack of training. Mobilization CEDW blamed the population and emphasized their own importance as the solution to dengue control. CHW failed to reflect their field experience in their discourse and felt they had no responsibility over vector control. All the groups agreed that government is to blame for dengue and that the solution lies in education. There is an evident need for regular educational interventions, based on dialogue and awareness-raising to deal with residents' daily reality, drawing individuals (residents and CHW) into the knowledge-building process. Under the prevailing methodology, the dissemination of information and knowledge is insufficient to promote community improvements for dengue control.

Resumen: Debido a la persistencia del dengue y de otras arbovirosis en Brasil, el poder público ha intensificado las acciones de combate al mosquito vector Aedes aegypti. Los ACE (agentes de combate a endemias) y los ACS (agentes comunitarios de salud), entre otras atribuciones, se convirtieron en interlocutores y promotores de conocimiento sobre enfermedades en sus comunidades. El objetivo de este trabajo fue analizar los conocimientos y prácticas sobre el control del dengue por parte de los diferentes sujetos sociales implicados: residentes y agentes. Se realizaron entrevistas a residentes, ACE de campo y de movilización, así como ACS, en dos barrios de Salvador, Bahía, mediante una metodología de grupo focal. Los residentes mostraron inseguridad sobre la forma de contagio, así como el peligro que supone el dengue. Los ACE de campo se encontraron en conflicto, por su necesidad de informar a la comunidad sobre aspectos que no dominan, además de demostrar un descontento personal en el trabajo, unido a un sentimiento de desvalorización, por su falta de cualificación. Los ACE de movilización culpan a la población y enfatizan la importancia de ellos mismos como solución para el control del dengue. Los ACS no se apropiaron de su experiencia de campo en su discurso y no se sienten obligados al control vectorial. Todos los grupos entrevistados concuerdan en que la culpa del dengue recae sobre el poder público, y la solución para el problema está en la educación. Se percibe una gran necesidad de intervenciones educativas regulares, pautadas en el diálogo y en la sensibilización para lidiar con la realidad cotidiana de los residentes, considerando a los individuos (residentes y agentes) como sujetos del proceso de construcción del conocimiento. Así pues, en la metodología actual, la divulgación de información y conocimiento no es suficiente para promover mejoras en las comunidades, con el fin de controlar el dengue.

Mortality for Critical Congenital Heart Diseases and Associated Risk Factors in Newborns. A Cohort Study / Mortalidade para Cardiopatias Congênitas e Fatores de Risco Associados em Recém-Nascidos. Um Estudo de Coorte

Abstract Background: Congenital heart diseases are the most common type of congenital defects, and account for more deaths in the first year of life than any other condition, when infectious etiologies are ruled out. Objectives: To evaluate survival, and to identify risk factors in deaths in newborns with critical and/or complex congenital heart disease in the neonatal period. Methods: A cohort study, nested to a randomized case-control, was performed, considering the Confidence Interval of 95% (95% CI) and significance level of 5%, paired by gender of the newborn and maternal age. Case-finding, interviews, medical record analysis, clinical evaluation of pulse oximetry (heart test) and Doppler echocardiogram were performed, as well as survival analysis, and identification of death-related risk factors. Results: The risk factors found were newborns younger than 37 weeks (Relative Risk - RR: 2.89; 95% CI [1.49-5.56]; p = 0.0015), weight of less than 2,500 grams (RR: 2.33 [; 95% CI 1.26-4.29]; p = 0.0068), occurrence of twinning (RR: 11.96 [95% CI 1.43-99.85]; p = 0.022) and presence of comorbidity (RR: 2.27 [95% CI 1.58-3.26]; p < 0.0001). The incidence rate of mortality from congenital heart disease was 81 cases per 100,000 live births. The lethality attributed to critical congenital heart diseases was 64.7%, with proportional mortality of 12.0%. The survival rate at 28 days of life decreased by almost 70% in newborns with congenital heart disease. The main cause of death was cardiogenic shock. Conclusion: Preterm infants with low birth weight and comorbidities presented a higher risk of mortality related to congenital heart diseases. This cohort was extinguished very quickly, signaling the need for greater investment in assistance technology in populations with this profile.

Resumo Fundamento: As cardiopatias congênitas configuram o tipo mais comum de defeitos congênitos, sendo responsáveis por mais mortes no primeiro ano de vida do que em qualquer outra condição, quando etiologias infecciosas são excluídas. Objetivo: Avaliar a sobrevida e identificar os fatores de risco nos óbitos em recém-nascidos com cardiopatia congênita crítica e/ou complexa no período neonatal. Métodos: Realizou-se um estudo de coorte, aninhado a um caso-controle aleatorizado, considerando Intervalo de Confiança de 95% (IC95%) e nível de significância de 5%, pareado por sexo do recém-nascido e idade materna. Foram feitas buscas ativas de casos, entrevistas, análise de prontuário, avaliação clínica da oximetria de pulso (teste do coraçãozinho) e do ecoDopplercardiograma, bem como análise de sobrevida e identificação dos fatores de risco relacionados ao óbito. Resultados: Os fatores de risco encontrados foram recém-nascidos com menos de 37 semanas (Risco Relativo − RR: 2,89; IC95% 1,49-5,56; p = 0,0015), peso inferior a 2.500 g (RR: 2,33; IC95% 1,26-4,29; p = 0,0068), ocorrência de gemelaridade (RR: 11,96; IC95% 1,43-99,85; p = 0,022) e presença de comorbidade (RR: 2,27; IC95% 1,58-3,26; p < 0,0001). A taxa de incidência de mortalidade por cardiopatias congênitas foi de 81 casos por 100 mil nascidos vivos. A letalidade atribuída às cardiopatias congênitas críticas foi de 64,7%, com mortalidade proporcional de 12,0%. A taxa de sobrevida aos 28 dias de vida diminuiu em quase 70% nos recém-nascidos com cardiopatias congênitas. A principal causa de óbito foi o choque cardiogênico. Conclusão: Recém-nascidos prematuros, com baixo peso e presença de comorbidades apresentaram maior risco de mortalidade relacionada às cardiopatias congênitas. Esta coorte se extinguiu muito rapidamente, sinalizando para a necessidade de maior investimento em tecnologia assistencial em populações com este perfil.

Incidência de síndromes genéticas associadas às cardiopatias congênitas / Incidence of genetic syndromes associated with congenital heart diseases

Introdução: as anomalias cardíacas podem se apresentar isoladamente ou fazer parte de síndromes cromossômicas ou gênicas. As principais causas dos defeitos cardíacos congênitos estão relacionadas com a interação entre os agentes ambientais e os fatores genéticos. Objetivo: conhecer a incidência das síndromes genéticas associadas às cardiopatias congênitas. Metodologia: Trata-se de um estudo seccional descritivo, com base em prontuários de pacientes portadores de cardiopatias congênitas, no período 2013 a 2014. Resultados: foram avaliados 307 prontuários, dos quais 52,4% eram de pacientes do sexo masculino e 47,6% de pacientes do sexo feminino, com idades entre zero e 15 anos. As síndromes genéticas associadas às cardiopatias foram evidenciadas em 50 pacientes, com a seguinte distribuição: Síndrome de Down, 13%; Síndrome de Kartagener, 1,3%; Síndrome de Edwards, 1%; Síndrome de Noonan, 0,7%; e Síndrome Di George (0,3%). Conclusão: a síndrome de Down prevaleceu sobre as demais síndromes associadas às cardiopatias congênitas.

Introduction : Cardiac abnormalities may either present itself singly, or as part of chromosomal or genetic syndromes. The main causes of congenital heart defects are related to the interaction between environmental agents and genetic factors. Objective: To know the incidence of genetic syndromes associated with congenital heart diseases. Methodology: This is a descriptive cross-sectional study, based on medical records of patients with congenital heart defects, between 2013 and 2014. Results: 307 medical records were evaluated. 52.4% were male patients and 47.6% femal patients aged between zero and fifteen years old. The genetic syndromes associated with heart diseases were evidenced in fifty patients, according to the following distribution: Down syndrome, 13%; Kartagener syndrome, 1.3%; Edwards syndrome, 1%; Noonan syndrome, 0.7%; And Di George Syndrome (0.3%). Conclusion: Down syndrome prevailed over the other syndromes associated with congenital heart diseases.

Implante transcateter da valva pulmonar. Protocolo clínico de tratamento e requisitos para credenciamento de centros e operadores no Brasil / Transcatheter pulmonary valve implantation. Clinical treatment protocol and accreditation requirements for centers and interventionists in Brazil

O implante transcateter da valva pulmonar (ITVP) evoluiu significativamente desde sua introdução, no início dos anos 2000. Atualmente, esta técnica é uma opção segura e eficaz para o tratamento das disfunções valvares graves (estenose e/ou insuficiência) em biopróteses ou condutos cirúrgicos em posição pulmonar, em vários centros do mundo. No Brasil, os resultados iniciais com este procedimento em centros de referência foram similares àqueles observados na experiência global. O ITVP tem se demonstrado factível, seguro e eficaz em mãos treinadas na nossa realidade. Porém, antes da aplicação disseminada desta técnica em outros centros em nosso país, houve a necessidade de se estabelecerem alguns critérios para a seleção do paciente, a técnica de implante e o seguimento clínico, assim como para o treinamento e o credenciamento de novos operadores e centros. As orientações aqui descritas foram determinadas por um grupo de especialistas com experiência renomada em cardiopatias congênitas e ITVP, tendo sido encaminhadas ao Conselho Federal de Medicina (CFM). Representantes das diferentes sociedades médicas foram envolvidas na preparação deste documento, incluindo a Sociedade Brasileira de Cardiologia (SBC), a Sociedade Brasileira de Hemodinâmica e Cardiologia Invasiva (SBHCI) e a Sociedade Brasileira de Cirurgia Cardiovascular (SBCCV). Acreditamos que a rígida aderência às recomendações listadas neste documento oficial seja crucial para a segurança do paciente e para que ótimos resultados sejam alcançados imediatamente e a longo prazo. Uma vez consolidado em nosso meio, o ITVP abrirá caminho para a introdução de novas terapias valvares em cardiopatias congênitas

Transcatheter pulmonary valve implantation (TPVI) has evolved significantly since its introduction in the early 2000s. Currently, this technique is a safe and effective option for the treatment of severe valve dysfunction (stenosis and/or regurgitation) in bioprostheses or surgical conduits in pulmonary position, in several centers worldwide. In Brazil, the initial results with this procedure in reference centers were similar to those observed in the overall experience. TPVI was proven to be feasible, safe, and effective in trained hands in Brazil. However, prior to the widespread application of this technique to other centers in this country, it was necessary to establish some criteria for patient selection, implantation technique, and clinical follow-up, as well as for the training and accreditation of new interventionists and centers. The guidelines described here were determined by a group of experts with well-known experience in congenital heart disease and TPVI, and were referred to the Conselho Federal de Medicina (CFM, portuguese for Federal Council of Medicine). Representatives of the different medical societies were involved in the creation of this document, including the Sociedade Brasileira de Cardiologia (SBC, portuguese for Brazilian Society of Cardiology), the Sociedade Brasileira de Hemodinâmica e Cardiologia Invasiva (SBHCI, portuguese for Brazilian Society of Hemodynamics and Invasive Cardiology), and the Sociedade Brasileira de Cirurgia Cardiovascular (SBCCV, portuguese for Brazilian Society of Cardiovascular Surgery). The authors believe that strict adherence to the recommendations contained in this official document is crucial for patient safety and for optimal results to be achieved in both the short and long term. Once consolidated in Brazil, TPVI will open doors for the introduction of new valve therapies in congenital cardiopathies