Prótesis Starr-Edwards vigente y funcional por más de 40 años / Starr-Edwards prosthesis current and functional for more than 40 years

INTRODUCCIÓN. Las enfermedades valvulares cardíacas han sido un foco de estudio y de evolución continua, pues existen grupos de pacientes seleccionados en quienes el cambio valvular es parte de su terapéutica. En 1960 surgió el modelo de prótesis de válvula en forma de esfera enjaulada de Albert Starr y Lowell Edwards. El diseño fue perfeccionándose y es usado hasta la actualidad ya que muestra resultados clínicos aceptables. OBJETIVO. Describir el caso de un paciente portador, de uno de los primeros prototipos de prótesis valvular cardiaca metálica Starr-Edwards vigente y funcional por más de 40 años. CASO CLÍNICO. Paciente masculino con prótesis Starr-Edwards en posición aórtica, quien hasta la fecha lleva más de 40 años de uso, mantiene la clase funcional II de la New York Heart Association, realizando anticoagulación con Warfarina así como controles estrictos del índice internacional normalizado. DISCUSIÓN. Es primordial la evaluación y seguimiento de portadores de prótesis valvular para analizar anifestaciones clínicas (disnea) y hallazgos en los exámenes de laboratorio o de imagen (ecografía) para el control clínico y hemodinámica del paciente, con la intención de contemplar circunstancias que podrían determinar la nueva intervención valvular. CONCLUSIONES. Las válvulas metálicas, en particular las del tipo de prótesis de Starr-Edwards presentan alto riesgo trombótico por lo que es recomendable mantener anticoagulación plena evitando valores de índice internacional normalizado muy elevados que podrían crear escenarios de eventos hemorrágicos. Varios pacientes portadores de una de las prótesis más antiguas como es el modelo la prótesis Starr-Edwards, pueden llegar a tener una mejor supervivencia y resultados clínicos aceptables.

INTRODUCTION. Cardiac valve diseases have been a focus of study and continuous evolution, since there are selected groups of patients in whom valve replacement is part of their therapy. In 1960, the caged sphere valve prosthesis model of Albert Starr and Lowell Edwards was developed. The design was refined and is still used today because it shows acceptable clinical results. OBJECTIVE. To describe the case of a patient with one of the first prototypes of Starr- Edwards metallic heart valve prosthesis, which has been in use and functional for more than 40 years. CLINICAL CASE. Male patient with Starr-Edwards prosthesis in aortic position, who to date has been used for more than 40 years, maintains functional class II of the New York Heart Association, performing anticoagulation with Warfarin as well as strict controls of the international normalized index. DISCUSSION. The evaluation and follow-up of valve prosthesis carriers is essential to analyze clinical manifestations (dyspnea) and findings in laboratory or imaging tests (ultrasound) for the clinical and hemodynamic control of the patient, with the intention of contemplating circumstances that could determine the new valve intervention. CONCLUSIONS. Metallic valves, particularly those of the Starr-Edwards prosthesis type, present a high thrombotic risk and it is therefore advisable to maintain full anticoagulation, avoiding very high values of the international normalized index that could create scenarios of hemorrhagic events. Several patients carrying one of the older prostheses, such as the Starr-Edwards prosthesis model, may have better survival and acceptable clinical results.

Analysis of outcome indexes in randomized controlled trials of traditional Chinese medicine for rheumatic heart disease / 中国中药杂志

The present study analyzed the efficacy evaluation indexes of the randomized controlled trials(RCTs) of Chinese medi-cine in the treatment of rheumatic heart disease to lay the foundation for the construction of the corresponding core outcome index set. Clinical RCTs with a definite diagnosis of rheumatic heart disease were retrieved from CNKI, Wanfang, VIP, Sino Med, Pub Med, EMbase, and Cochrane Library from January 1, 2010, to December 31, 2020. Thirty-five RCTs were included, involving 3 314 patients and 41 efficacy evaluation indexes, which covered seven domains [traditional Chinese medicine(TCM) symptoms/syndromes, symp-toms/signs, physical and chemical examination, quality of life, long-term prognosis, economic evaluation, and safety events]. Physi-cal and chemical examination(56. 91%) and symptoms/signs(29. 27%) were the more frequently applied. The number of indexes used in a single trial ranged from 1 to 15, with an average of 4. The measurement time points of the top five indexes in the frequency of use were as follows: total response rate was reported at five measurement time points, ranging from 14 days to 6 months; left ventri-cular ejection fraction was measured at eight time points ranging from 5 days to 6 months; left ventricular end systolic diameter was measured at six time points, ranging from 5 days to 6 months; interleukin-2(IL-2) and tumor necrosis factor-α(TNF-α) were repor-ted 28 days after treatment. At present, there are many problems in the efficacy outcome indexes of RCTs in the treatment of rheumatic heart disease with TCM, such as large difference in quantity, unclear primary and secondary indexes, unreasonable selection of " surro-gate indexes", insufficient attention to long-term prognostic indexes and safety event indexes, non-standard application of composite in-dexes, long measurement period, and lack of TCM characteristics. It is urgent to establish the core outcome set for TCM treatment of rheumatic heart disease.

Níveis de Tiol Sérico e Homeostase Tiol/Dissulfeto em Pacientes com Doença Valvar Mitral Reumatismal e em Sujeitos Saudáveis / Serum Thiol Levels and Thiol/Disulfide Homeostasis in Patients with Rheumatic Mitral Valve Disease and Healthy Subjects

Resumo Fundamento A doença valvar mitral reumatismal (DVMR) é a apresentação mais comum das doenças cardíacas reumáticas (DCR). Os processos de inflamação e fibrose também têm papéis significativos em sua patogênese. Estudos recentes demonstram que os tióis e o tiol-dissulfeto são marcadores de stress oxidativo inéditos e promissores. Objetivos O objetivo deste estudo foi avaliar diferenças entre os níveis de tiol sérico e de tiol-dissulfeto em pacientes com DVMR e no grupo de controle. Métodos Noventa e dois pacientes com DVMR foram cadastrados no estudo. Cinquenta e quatro sujeitos saudáveis, e com correspondência de sexo e idade em relação ao grupo de estudo, também foram incluídos no estudo como um grupo de controle. Foram investigados os níveis de tiol nos pacientes com DVMR e o grupo de controle. Os p-valores menores que 0,05 foram considerados estatisticamente significativos. Resultados Os pacientes com DVMR apresentaram pressão sistólica da artéria pulmonar (PSAP) e níveis de diâmetro do átrio esquerdo (AE) mais altos que os do grupo de controle. Os níveis de tiol nativo (407±83 μmol/L vs. 297±65 μmol/L, p<0,001) e tiol total (442±82 μmol/L vs. 329±65 μmol/L, p<0,001) são mais altos no grupo de controle. Níveis de dissulfeto (16,7±4,9 μmol/L vs. 14,8±3,7 μmol/L, p=0,011) são mais altos no grupo de pacientes com DVMR. Foi identificada uma correlação positiva entre as razões dissulfeto/tiol nativo e dissulfeto/tiol total com PSAP, diâmetro de AE, e gravidade da EMi. A razão dissulfeto/tiol total é significativamente mais alta em pacientes com EMi grave que em pacientes com EMi leve a moderada. Conclusões Até onde se sabe, este é o único estudo que avaliou a homeostase tiol/dissulfeto como um preditor inédito, que está relacionado de forma mais próxima à DVMR e à gravidade da EMi.

Abstract Background Rheumatic mitral valve disease (RMVD) is the most common presentation of rheumatic heart disease (RHD). Inflammation and fibrosis processes also play significant roles in its pathogenesis. Recent studies showed that thiols and thiol-disulfide are promising novel oxidative stress markers. Objectives The present study aimed to evaluate differences in the serum thiol and thiol-disulfide levels in patients with RMVD and the control group. Methods Ninety-two patients with RMVD were enrolled in the study. Fifty-four healthy subjects, age, and gender-matched with the study group, were also included in the study as a control group. This study investigated thiol levels in patients with RMVD and the control group. P-values lower than 0.05 were considered statistically significant. Results The patients with RMVD presented higher systolic pulmonary artery pressure (SPAP) and left atrial (LA) diameter levels than the control group. Native thiol (407±83 μmol/L vs. 297±65 μmol/L, p<0.001) and total thiol (442±82 μmol/L vs. 329±65 μmol/L, p<0.001) levels were higher in the control group. Disulfide (16.7±4.9 μmol/L vs. 14.8±3.7 μmol/L, p=0.011) levels were higher in the group of patients with RMVD. A positive correlation was found between disulfide/native and disulfide/total thiols ratio with SPAP, LA diameter, and MS severity. Disulfide/total thiols ratio was significantly higher in patients with severe MS than with mild to moderate MS patients. Conclusions To the best of our knowledge, this is the only study of its kind that has evaluated thiol/disulfide homeostasis as a novel predictor, which was more closely related to RMVD and the severity of MS.

Caracterização Histológica das Lesões da Valva Mitral de Pacientes com Cardiopatia Reumática / Histopathological Characterization of Mitral Valvular Lesions from Patients with Rheumatic Heart Disease

Resumo Fundamentos: Os mecanismos subjacentes pelos quais a doença cardíaca reumática (DCR) levam à disfunção valvar grave não são totalmente compreendidos. Objetivo: O presente estudo avaliou as alterações histopatológicas nas valvas mitrais (VM) buscando uma associação entre o padrão de disfunção valvar predominante e os achados histopatológicos. Métodos: Em 40 pacientes submetidos à troca da VM devido a DCR e em 20 controles submetidos a transplante cardíaco, foram analisados os aspectos histológicos da VM excisada. Dados clínicos e ecocardiográficos também foram coletados. As análises histológicas foram realizadas usando coloração com hematoxilina-eosina. Determinou-se inflamação, fibrose, neoangiogênese, calcificação e metaplasia adiposa. Valores de p<0,05 foram considerados estatisticamente significativos. Resultados: A idade média dos pacientes com DCR foi de 53±13 anos, sendo 36 (90%) do sexo feminino, enquanto a idade média dos controles foi de 50±12 anos, semelhante aos casos, sendo a maioria do sexo masculino (70%). O endocárdio valvar reumático apresentou espessura maior que os controles (1,3±0,5 mm versus 0,90±0,4 mm, p=0,003, respectivamente), e infiltrado inflamatório mais intenso no endocárdio (78% versus 36%; p=0,004), com predominância de células mononucleares. Ocorreu fibrose moderada a acentuada mais frequentemente em válvulas reumáticas do que em válvulas controle (100% vs. 29%; p<0,001). Ocorreu calcificação em 35% das valvas reumáticas, principalmente entre as valvas estenóticas, associada à área valvar mitral (p=0,003). Conclusões: Apesar do intenso grau de fibrose, o processo inflamatório permanece ativo na valva mitral reumática, mesmo em doença tardia com disfunção valvar. A calcificação predominou em valvas estenóticas e em pacientes com disfunção ventricular direita.

Abstract Background: The underlying mechanisms by which rheumatic heart disease (RHD) lead to severe valve dysfunction are not completely understood. Objective: The present study evaluated the histopathological changes in mitral valves (MV) seeking an association between the pattern of predominant valvular dysfunction and histopathological findings. Methods: In 40 patients who underwent MV replacement due to RHD, and in 20 controls that underwent heart transplant, histological aspects of the excised MV were analyzed. Clinical and echocardiographic data were also collected. Histological analyses were performed using hematoxylin-eosin staining. Inflammation, fibrosis, neoangiogenesis, calcification and adipose metaplasia were determined. A p value<0.05 was considered to be statistically significant. Results: The mean age of RHD patients was 53±13 years, 36 (90%) were female, whereas the mean age of controls was 50±12 years, similar to the cases, with the majority of males (70%). The rheumatic valve endocardium presented greater thickness than the controls (1.3±0.5 mm versus 0.90±0.4 mm, p=0.003, respectively), and a more intense inflammatory infiltrate in the endocardium (78% versus 36%; p=0.004), with predominance of mononuclear cells. Moderate to marked fibrosis occurred more frequently in rheumatic valves than in control valves (100% vs. 29%; p<0.001). Calcification occurred in 35% of rheumatic valves, especially among stenotic valves, which was associated with the mitral valve area (p=0.003). Conclusions: Despite intense degree of fibrosis, the inflammatory process remains active in the rheumatic mitral valve, even at late disease with valve dysfunction. Calcification predominated in stenotic valves and in patients with right ventricular dysfunction.

Non-infectious thrombotic endocarditis associated with chronic rheumatic heart disease and disseminated tuberculosis

Rheumatic heart disease is still common in developing countries and requires prompt intervention to prevent chronic complications. Vegetations in rheumatic heart disease might be due to acute episodes of rheumatic fever itself or due to either infective endocarditis (IE) or Non-infectious thrombotic endocarditis (NITE). Each form of vegetations has specific pathological characteristics on gross and microscopic examination. However, clinically IE and NITE may have overlapping signs and symptoms. A chance of misdiagnosis of NITE as culture-negative infective endocarditis is higher if the former present with infective symptoms like fever. NITE of valves can be due to underlying associated malignant neoplasm, particularly mucinous adenocarcinoma, pneumonia, cirrhosis, autoimmune disorders, and hypercoagulable state. The coexistence of tuberculosis, non-infectious thrombotic endocarditis and rheumatic valvular heart disease was rarely documented in medical literature. We describe a case of chronic rheumatic heart disease with vegetations in the posterior mitral valve leaflet, treated as culture-negative infective endocarditis, which, at autopsy, reveals the presence of Nonbacterial thrombotic endocarditis vegetation over calcified, fibrosed mitral valve leaflets and associated disseminated tuberculosis along with classic pathological sequela findings of chronic rheumatic mitral valvular heart disease in lungs and liver.

The global burden of rheumatic heart disease: population-related differences (it is not all the same!)

Abstract Rheumatic heart disease (RHD) remains the most common cardiovascular disease in young adults and adolescents in need of heart surgery in low- and middle-income countries (LMICs). The mean age of patients is 20-25 years, often much younger. By contrast, the few patients with chronic RHD in developed countries present a mean age of around 55 years. It is absolutely fundamental to differentiate these two types of population. Pathology, lesions and surgical methods are different, and the results should not be compared. It is not all the same! A certain enthusiasm for mitral repair has recently surged, with several reports showing excellent results in children and young adults, resulting from the renewed interest of cardiac surgeons, also based on new and modified techniques developed in the meantime. While surgery is easily accessible to patients in developed countries, the situation in LMICs is often dramatic, with countries where there is a complete absence of or few surgical facilities absolutely unable to meet gigantic demands. Many foreign surgical teams conduct humanitarian missions in several of these countries. They are just a "drop of water in the ocean" of needs. In some cases, however, these missions led to the establishment of local teams that now work independently and, in some cases, outperform the foreign teams still visiting.

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.

Mortality Trends from Cardiovascular Diseases in the State of Bahia, Brazil, between 2000 and 2015

Abstract Background: Mortality from cardiovascular diseases has reduced in Brazil in recent decades, but this phenomenon is not clear in the northeast region of the country. Objectives: To describe the mortality trends from cardiovascular disease in Bahia from 2000-2015, in total population and by sex and age groups, and by specific causes (ischemic heart disease, cerebrovascular diseases, rheumatic heart disease and heart failure). Methods: This was a time series study. Data were obtained from SIM-DATASUS and IBGE, and the 10th revision of the ICD used for disease classification. Mortality rates (per 100,000 inhabitants) were calculated from total cardiovascular disease and specific causes, by sex and age groups. Direct standardization was used to adjust for age, using the population of 2010 as reference. Linear regression models estimated percentage variation. The significance level of 5% was adopted. Results: In Bahia, crude mortality rates from cardiovascular disease increased in the period; however, after standardization by age, mortality rates became stable for the total and female populations, with a slight reduction for the male population. An increase in mortality rates from cardiovascular disease was found in the elderly groups. For ischemic heart disease, a progressive increase in adjusted mortality rates was observed: 43%, 24% and 29% for the total, male, and female population, respectively. There was a progressive reduction in crude and age-standardized mortality rates from heart failure in all groups, a modest reduction in age-adjusted mortality rates from cerebrovascular diseases, and a slight reduction in age-standardized mortality rate from rheumatic heart disease, especially in the subgroup <40 years. Conclusions: Mortality from cardiovascular disease in Bahia did not follow the decreasing trend of other Brazilian states, especially in relation to ischemic heart disease, which showed an increase in mortality rates.

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.

Enfermedad reumática cardiaca: ¿Estamos realmente haciendo lo necesario? / Rheumatic heart disease: are we really doing what is necessary?

Resumen Objetivo: Revisar las características demográficas, clínicas y ecocardiográficas, así como el abordaje quirúrgico de pacientes con fiebre reumática menores de 18 años, durante un periodo de diez años. Materiales y métodos: Estudio descriptivo, retrospectivo, en el cual se incluyeron pacientes menores de 18 años, con diagnóstico de fiebre reumática entre los años 2006 a 2016. Resultados: Se incluyeron 22 niños con edades entre los 10 a los 14 años, de los cuales 59% (13/22) eran mujeres; 13,6% (3/22) provenían de Arauca y el 18,1% (4/22) eran indígenas. Se diagnosticó carditis en el 63,6% (14/22) seguido de poliartralgia y fiebre en un 59% (13/22) y 54% (12/22) respectivamente; así mismo, se detectó corea de Sydenham y eritema marginado 4,5% (1/22). Se determinó compromiso severo de válvula mitral en el 72,7% (16/22). 15 pacientes fueron llevados a cirugía; se realizó reemplazo valvular mitral en 13,3% (2/15), plastia mitral y tricúspide en el 40% (6/15), plastia mitral, tricúspide y aórtica en el 6,66%(1/15) y reemplazo aórtico en el 5,2% (1/15). Un paciente requirió oxigenación por membrana extracorpórea. La mortalidad fue del 0% (0/22). Conclusiones: Se observó que la fiebre reumática ocasionó grave compromiso cardiaco, que requirió un alto grado de intervención quirúrgica. Las poblaciones rurales e indígenas se beneficiarían notablemente de una prevención primordial, primaria y secundaria, al igual que de un diagnóstico y tratamiento oportunos.

Abstract Objective: The aim of this study is to review the demographic, clinical, and cardiac ultrasound characteristics, as well as the surgical approach, of patients less than 18 years-old with rheumatic fever. Materials and methods: A descriptive, retrospective study was conducted that included patients less than 18 years-old diagnosed with rheumatic fever between the years 2006 to 2016. Results: The study included a total of 22 children with ages between 10 and 14 years, of whom 59% were female. It was recorded that 13.6% (3/22) were from Arauca Department (Colombia), and 18.1% (4/22) were indigenous. Carditis was diagnosed in 63.6% (14/22), followed by polyarthralgia and fever in 59% (13/22) and 54% (12/22), respectively. Sydenham's chorea and marginal erythema was also detected in 4.5% (1/22). The severity of mitral valve involvement was determined in 72.7% (16/22). Of the 15 patients that had surgery, 13.3% (2/15) had a mitral valve replacement, mitral and tricuspid repair in 40% (6/15), mitral, tricuspid, and aortic repair in 6.66%, respectively (1/15), and aortic replacement in 5.2% (1/15). One patient required extracorporeal membrane oxygenation. There was no mortality. Conclusions: It was observed that rheumatic fever led to serious cardiac compromise that required a high rate of surgical interventions. Rural and indigenous population would notably benefit from basic, primary, and secondary prevention, as well as a timely diagnosis and treatment.

Double Coronary Artery Fistula from Left Circumflex Artery draining to the left Atrium in a Rheumatic Heart Disease patient – A case report

Introduction@#Coronary artery fistula (CAF) is a connection between one or more of the coronary arteries and a cardiac chamber or great vessel. This is a rare defect and occurs in only 0.2% of the population. Most of the cases are congenital but acquired causes are also reported. A CAF may occur as an isolated cardiac defect or with other cardiac diseases such as rheumatic heart disease (RHD). Only a few cases of coexisting CAF and RHD have been reported. Local data reports only 0.69% CAFs associated with congenital malformations of the heart. Only 61 patients among all patients who underwent coronary arteriography in 34 years were reported to have a CAFs. We report a case of severe mitral stenosis (MS) with a double CAF from the left circumflex (LCx) artery draining into the left atrium. @*Case@#A 46-year old female with RHD with severe MS came in due to progressive dyspnea. The coronary angiogram revealed two fistulous tracts originating from the LCx draining into the left atrium. She underwent mitral valve replacement (MVR) surgery, left atrial plication, and closure of the fistula drainage the left atrium. The postoperative course was uneventful. @*Discussion@#A CAF is often asymptomatic until the second decade of life. Untreated, this may progress and cause ischemic and heart failure signs and symptoms. The presence of MS caused elevated left atrial pressure which might have prevented the increase in the volume of blood draining from the LCx artery to the left atrium through the fistulas. Hence, the MS might have prevented the dilatation of the two fistulas. Surgical correction is also indicated in the fistulas since resolution of the mitral stenosis with MVR will decrease the LA pressure which might result to dilatation and increased drainage of the fistulas causing complications later.

Predictors of Unfavourable Outcomes in Children and Adolescents Submitted to Surgical Mitral Valvuloplasty Secondary to Chronic Rheumatic Heart Disease / Preditores de Desfecho Desfavorável em Crianças e Adolescentes Submetidos à Valvoplastia Mitral Cirúrgica Secundária a Cardiopatia Reumática Crônica

Abstract Background: Mitral valve repair in paediatric patients with chronic rheumatic heart disease is superior to valve replacement and has been used with good results. Objective: To identify predictors of unfavourable outcomes in children and adolescents submitted to surgical mitral valvuloplasty secondary to rheumatic heart disease. Methods: Retrospective study of 54 patients under the age of 16 operated at a tertiary paediatric hospital between March 2011 and January 2017. The predictors of risk for unfavourable outcomes were: age, ejection fraction, degree of mitral insufficiency, degree of pulmonary hypertension, presence of tricuspid insufficiency, left chamber dilation, preoperative functional classification, duration of cardiopulmonary bypass, duration of anoxia, presence of atrial fibrillation, and duration of vasoactive drug use. The outcomes evaluated were: death, congestive heart failure, reoperation, residual mitral regurgitation, residual mitral stenosis, stroke, bleeding and valve replacement. For all analyzes a value of p < 0.05 was established as significant. Results: Of the patients evaluated, 29 (53.7%) were female, with an average of 10.5 ± 3.2 years. The functional classification of 13 patients (25%) was 4. There was no death in the sample studied. The average duration of extracorporeal circulation was 62.7±17.8 min, and anoxia 50 ± 15.7 min. The duration of use of vasoactive drug in the immediate postoperative period has an average of 1 day (interquartile interval 1-2 days). The logistic regression model was used to evaluate the predictive variables for each unfavourable outcome. The duration of use of vasoactive drug was the only independent predictor for the outcomes studied (p = 0.007). Residual mitral insufficiency was associated with reoperation (p = 0.044), whereas tricuspid insufficiency (p = 0.012) and pulmonary hypertension (p = 0.012) were associated with the presence of unfavourable outcomes. Conclusion: The duration of vasoactive drug use is an independent predictor for unfavourable outcomes in the immediate and late postoperative period, while residual mitral regurgitation was associated with reoperation, and both tricuspid regurgitation and pulmonary hypertension were associated with unfavourable outcomes.

Resumo Fundamento: A plastia da valva mitral, em pacientes pediátricos com cardiopatia reumática crônica, é superior à troca valvar e vem sendo utilizada com bons resultados. Objetivo: Identificar variáveis preditoras de desfecho desfavorável em crianças e adolescentes submetidos à valvoplastia mitral cirúrgica secundária à cardiopatia reumática. Métodos: Estudo retrospectivo em 54 pacientes menores de 16 anos, operados em um hospital pediátrico terciário entre março de 2011 e janeiro de 2017. As variáveis preditoras de risco para desfecho desfavorável foram: idade, fração de ejeção, grau de insuficiência mitral, grau de hipertensão pulmonar, presença de insuficiência tricúspide, dilatação de câmaras esquerdas, classe funcional no pré-operatório, tempo de circulação extracorpórea, tempo de anóxia, presença de fibrilação atrial e tempo de uso de droga vasoativa. Os desfechos avaliados foram: morte, insuficiência cardíaca congestiva, reoperação, insuficiência mitral residual, estenose mitral residual, acidente vascular cerebral, sangramento e troca valvar. Para todas as análises foi estabelecido valor de p < 0,05 como significante. Resultados: Dos pacientes avaliados, 29 (53,7%) eram do sexo feminino, com média de idade de 10,5 ± 3,2 anos. A classe funcional de 13 pacientes (25%) foi 4. Não houve morte na amostra estudada. O tempo médio de circulação extracorpórea foi de 62,7 ± 17,8 minutos e de anóxia 50 ± 15,7 minutos. O tempo de uso de droga vasoativa no pós-operatório imediato teve mediana de 1 dia (intervalo interquartil 1-2 dias). O modelo de regressão logística foi utilizado para avaliar as variáveis preditoras para o desfecho desfavorável. O tempo de uso de droga vasoativa foi o único preditor independente para os desfechos estudados (p = 0,007). A insuficiência mitral residual foi associada à reoperação (p = 0,044), enquanto a insuficiência tricúspide (p = 0,012) e a hipertensão pulmonar (p = 0,012) se associaram à presença de desfechos desfavoráveis. Conclusão: O tempo de uso de droga vasoativa é um preditor independente para desfechos desfavoráveis no pós-operatório imediato e tardio, enquanto insuficiência mitral residual se associou à reoperação e tanto a insuficiência tricúspide quanto a hipertensão pulmonar foram associadas a desfechos desfavoráveis.

Rheumatic Fever: A Disease without Color / Febre Reumática: Uma Doença sem Cor

Abstract Background: Brazil has approximately 30.000 cases of Acute Rheumatic Fever (ARF) annually. A third of cardiovascular surgeries performed in the country are due to the sequelae of rheumatic heart disease (RHD), which is an important public health problem. Objectives: to analyze the historical series of mortality rates and disease costs, projecting future trends to offer new data that may justify the need to implement a public health program for RF. Methods: we performed a cross-sectional study with a time series analysis based on data from the Hospital Information System of Brazil from 1998 to 2016. Simple linear regression models and Holt's Exponential Smoothing Method were used to model the behavior of the series and to do forecasts. The results of the tests with a value of p < 0.05 were considered statistically significant. Results: each year, the number of deaths due to RHD increased by an average of 16.94 units and the mortality rate from ARF increased by 215%. There was a 264% increase in hospitalization expenses for RHD and RHD mortality rates increased 42.5% (p-value < 0.05). The estimated mortality rates for ARF and RHD were, respectively, 2.68 and 8.53 for 2019. The estimated cost for RHD in 2019 was US$ 26.715.897,70. Conclusions: according to the Brazilian reality, the 1-year RHD expenses would be sufficient for secondary prophylaxis (considering a Benzatin Penicillin G dose every 3 weeks) in 22.574 people for 10 years. This study corroborates the need for public health policies aimed at RHD.

Resumo Fundamento: O Brasil tem aproximadamente 30.000 casos de febre reumática aguda (FRA) por ano. Um terço das cirurgias cardiovasculares realizadas no país se deve às sequelas da doença reumática cardíaca (DRC), a qual é um importante problema de saúde pública. Objetivos: Analisar as séries históricas de taxas de mortalidade e custos das doenças, projetando tendências futuras para oferecer novos dados que possam justificar a necessidade de implementação de um programa de saúde pública para FR. Métodos: Foi realizado um estudo transversal com análise de séries temporais a partir de dados do Sistema de Informações Hospitalares do Brasil, de 1998 a 2016. Modelos de regressão linear simples e o método de suavização exponencial de Holt foram utilizados para modelar o comportamento das séries e fazer previsões. Os resultados dos testes com um valor de p <0,05 foram considerados estatisticamente significantes. Resultados: A cada ano, o número de mortes por DRC aumentou em média 16,94 unidades, e a taxa de mortalidade por FRA aumentou em 215%. Houve um aumento de 264% nas despesas de hospitalização por DRC, e as taxas de mortalidade por DRC aumentaram 42,5% (p-valor < 0,05). As taxas de mortalidade estimadas para FRA e DRC foram, respectivamente, 2,68 e 8,53 para 2019. O custo estimado para a DRC em 2019 foi de US$ 26.715.897,70. Conclusões: De acordo com a realidade brasileira, o gasto relativo a 1 ano de DRC seria suficiente para a profilaxia secundária (considerando uma dose de penicilina G benzatina a cada 3 semanas) em 22.574 pessoas por 10 anos. Este estudo corrobora a necessidade de políticas públicas de saúde direcionadas à DRC.