Research progress of Parkin protein regulating mitochondrial homeostasis through ubiquitination in cardiovascular diseases / 中国药理学通报

In addition to providing energy for cells, mitochondria also participate in calcium homeostasis, cell information transfer, cell apoptosis, cell growth and differentiation. Therefore, maintaining mitochondrial homeostasis is very crucial for the body to carry out normal life activities. Ubiquitination, a post-translational modification of proteins, is involved in various physiological and pathological processes of cells by regulating mitochondrial homeostasis. However, the mechanism by which ubiquitination regulates mitochondrial homeostasis has not been summarized, especially the effect of Parkin protein on cardiovascular diseases. In this paper, the specific mechanism of mitochondrial homeostasis regulated by ubiquitination of Parkin protein is discussed, and the influence of mitochondrial homeostasis imbalance on cardiovascular diseases is reviewed, with a view to providing potential therapeutic strategies for the clinical treatment of cardiovascular diseases.

Síndrome de barlow: causa olvidada de miocardiopatía dilatada y muerte súbita / Barlow syndrome: A rare etiology of sudden death

Resumen Se presenta el caso de una mujer de 60 años, con an tecedente de prolapso de la válvula mitral, que consultó por disnea y palpitaciones de 2 semanas de evolución hasta clase funcional IV. En el electrocardiograma de ingreso se evidenció ritmo de fibrilación auricular de moderada respuesta con extrasístoles ventriculares fre cuentes. Se realizó ecocardiograma transtorácico donde se observó prolapso de la válvula mitral con deterioro grave de la función ventricular. Se diagnosticó síndrome de Barlow. La paciente intercurrió durante la internación con tres episodios de paro cardiorrespiratorio que revir tieron con maniobras de reanimación cardiopulmonar avanzada. Durante la internación se realizó balance negativo, se revirtió a ritmo sinusal y se colocó cardio desfibrilador implantable en prevención secundaria. En el seguimiento persiste con deterioro grave de la función ventricular. Destacamos el síndrome de Barlow como una causa poco frecuente de muerte súbita y su asociación con miocardiopatía dilatada.

Abstract We present the case of a 60-year-old woman, with a history of mitral valve prolapse, who consulted for dyspnea and palpitations of 2 weeks of evolution up to functional class IV. The admission electrocardio gram showed a moderately responsive atrial fibrilla tion rhythm with frequent ventricular extra systoles. A transthoracic echocardiogram was performed which showed mitral valve prolapse with severe impairment of ventricular function. Barlow syndrome was diagnosed. During hospitalization, the patient presented three epi sodes of cardiorespiratory arrest that were reversed with advanced cardiopulmonary resuscitation maneuvers. During admission, a negative balance was performed, sinus rhythm was reverted and an implantable auto matic defibrillator was placed in secondary prevention. During follow-up, severe deterioration of ventricular function persisted. We highlight Barlow syndrome as a rare cause of sudden death and its association with dilated cardiomyopathy.

Advancements in pharmacological therapy for transthyretin cardiac amyloidosis and its comorbidities / 中国药房

Transthyretin cardiac amyloidosis myocardiopathy （ATTR-CM） is an infiltrative cardiomyopathy characterized by the deposition of amyloidogenic material in the myocardial interstitium due to the misfolding of monomers following the dissociation of unstable transthyretin （TTR） tetramers. Previous treatments for ATTR-CM lacked specificity，primarily targeting symptomatic management of heart failure and arrhythmias. In recent years，researchers have developed two major classes of drugs addressing the pathogenesis of ATTR-CM. The first class stabilizes TTR tetramer structure （such as tafamidis and acoramidis）， while the second class interferes with TTR synthesis （such as patisiran）. Among these，tafamidis has been confirmed as the only currently effective treatment for ATTR-CM，while other drugs are still in clinical trial stages with limited clinical evidence. Concerning the management of comorbidities in ATTR-CM，treatment mainly focuses on common cardiac comorbidities （such as heart failure and arrhythmias）. Traditional drugs used to improve heart failure prognosis （such as β-blockers and renin-angiotensin- receptor blocker），have not demonstrated prognosis improvement in ATTR-CM patients and may even lead to adverse reactions. For ATTR-CM patients with concurrent atrial fibrillation，anticoagulation therapy is recommended to prevent thrombus formation，and amiodarone can be used for rhythm control. Despite significant advancements in pharmaceutical treatments for ATTR-CM，the overall prognosis remains poor，necessitating further research into the pathogenesis and target development to enhance the prognosis of ATTR-CM patients.

Paciente adulto con alteraciones cardiacas secundarias a enfermedad de Fabry / An adult patient with heart abnormalities secondary to Fabry disease

Resumen Introducción: La enfermedad de Fabry es una entidad crónica, progresiva, poco frecuente, de origen genético y patrón de herencia recesivo ligado al cromosoma X. Se caracteriza por déficit enzimático de alfa-galactosidasa causado por mutaciones en el gen GLA, lo que produce almacenamiento anormal de esfingolípidos celulares y tisulares Caso clínico: Se describe el caso de un paciente de 53 años, con antecedente familiar y compromiso cardíaco predominante, dado por hipertrofia ventricular izquierda, arritmias auriculares e insuficiencia cardiaca congestiva secundaria, quien, adicionalmente, tiene manifestaciones multisistémicas que han evolucionado desde la infancia. Entre los pilares de tratamiento requirió implantación definitiva de marcapasos y terapia de reemplazo enzimático. Conclusiones: La enfermedad de Fabry es una entidad de compromiso sistémico y progresivo, de baja prevalencia, cuya importancia se debe reflejar en el entrenamiento del personal de salud para el adecuado diagnóstico, con miras a mejorar la calidad de vida de los pacientes.

Abstract Introduction: Fabry’s disease is a chronic, progressive and a multisystemic disease of genetic origin, with a recessive pattern of inheritance tied to the X chromosome, characterized by the lisosomal deposit of globotriaosylceramide as a consequence of a deficiency in the activity of the alpha-galactosidase A enzyme. Clinical case: We present a clinical case of a 53-year old male patient carrying this disease with family history of Fabry’s disease, who suffers cardiac compromise as the main clinical manifestation. He is a patient who required the implantation of a permanent pacemaker and enzyme replacement therapy. Conclusions: Fabry´s disease is a systemic and progressive disease, low fre-quency, and not well known by the health personnel, which implies a late diagnosis, being the cardiac compromise the second in frequency after renal compromise, which can lead to the patient to a hypertrophic cardiomyopathy and a rhythm and cardiac conduction disorder.

Translational research in Chagas disease: perspectives in nutritional therapy emerging from selenium supplementation studies as a complementary treatment

Translational research (TR) is an interdisciplinary branch of the biomedical field that seeks to connect its three supporting pillars: basic research on the bench, the hospital beds and other health system services, and the delivery of products for the well-being and health of the community. Here, we review the five transition stages of the TR spectrum, registering the lessons learned during > 20 years leading to the first clinical trial designed and performed in Brazil for testing a complementary treatment for Chagas disease (CD): the selenium trial (STCC). Lessons learned were: (1) to consider all the TR spectrum since the beginning of the project; (2) to start simultaneously animal studies and translation to humans; (3) to ensure a harmonious interaction between clinical and basic research teams; (4) to include MSc and PhD students only in pre-clinical and basic studies (TR0) or vertical clinical studies using retrospective samples and data (TR1); (5) to identify potential suppliers in the national commercial market for a future final treatment since the pre-clinical stage; (6) to keep an international network of experts as permanent advisers on the project. In the whole process, some perspectives were created: a complementary clinical trial for the opened questions and the construction of a Brazilian clinical CD platform.

Patologías del niño inmigrante en la Unidad de Cuidados Intensivos: actualización / Pathologies of the immigrant child in the Intensive Care Unit: update

Resumen: Cada vez es más frecuente la atención médica en la Unidad de Cuidados Intensivos (UCI) de niños o adolescentes inmigrantes como también de aquellos nacidos en nuestro país con padres en tal condición. Esto ha ocasionado, en la actualidad, que el equipo de salud se deba enfrentar con problemas diagnósticos derivados del escaso conocimiento de condiciones genéticas propias de esta población y/o el desarrollo de diversas patologías infrecuentes en nuestro país, algunas resultantes de su condi ción sanitaria. En esta revisión se abordan diversos aspectos de la patología hematológica, infecciosa, parasitaria, respiratoria y cardiovascular, todos tópicos relevantes de conocer durante su estadía en la UCI. Es un deber del equipo de salud actualizarse sobre patologías de baja prevalencia en nuestro país, algunas de ellas muy poco conocidas hasta hace una década, pero que, actualmente, están cada vez más presentes en las UCI del sistema de salud público chileno.

Abstract: It is increasingly common to provide medical care in the Intensive Care Unit (ICU) for immigrant children and adolescents as well as those born in Chile with parents in such condition. Currently, this has caused that the health team has to face diverse infrequent pathologies in our country and/ or diagnostic problems derive from the poor knowledge of genetic conditions of this population, some resulting from their health conditions. This review addresses several aspects of hematological, infectious, parasitic, respiratory, and cardiovascular pathologies, all relevant topics to know during their stay in the ICU. It is a duty of the health team to be updated on pathologies of low prevalence in our country, some of them very little known until a decade ago, but which are currently increasingly present in the ICUs of the Chilean public health system.

Miocardiopatía de y movimiento sistólico anterior de válvula mitral / Myocardiopaty and sistolic anterior movement of mitral valve

Tako tsubo cardiomyopathy associated with left ventricular outflow tract obstruction (LVOT) is a rare cause of persistent perioperative hypotension. One of the causes of this association is the systolic anterior motion (SAM) of the mitral valve. We report a case of a 67-year-old woman who, after undergoing liver segmentectomy because of metastasis, presents post-operative hypotension that is difficult to manage. Upon evaluation with a transthoracic echocardiogram, the diagnostic suspicion of Tako tsubo syndrome associated with LVOT obstruction secondary to SAM was raised. As initial therapy, a volume expander, in association with propanolol and phenylephrine were administred, achieving partial hemodynamic response. Later, she evolved with signs of heart failure and was transferred to the intensive care unit for management. In this unit, non-invasive ventilatory support, diuretic and vasopressor therapy were required, achieving favorable results on the second post-operative day. Echocardiography was essential to make the differential diagnosis against a persistent post-operative hypotension

La cardiomiopatía de Tako tsubo asociada a obstrucción del tracto de salida del ventrículo izquierdo (TSVI) es una causa poco frecuente de hipotensión persistente en el perioperatorio. Uno de los motivos de esta asociación es el movimiento anormal sistólico (SAM) de la válvula mitral. A continuación, presentamos el caso de una mujer de 67 años que, tras ser sometida a segmentectomía hepática por metástasis, presenta cuadro de hipotensión postoperatoria de difícil manejo. Al ser evaluada con ecocardiograma transtorácico se plantea la sospecha diagnóstica de síndrome de Tako tsubo asociado a obstrucción del TSVI secundario a SAM. Como terapia inicial se realizó aporte de volumen, en asociación con propanolol y fenilefrina, logrando parcial respuesta hemodinámica. Posteriormente, evolucionó con signos de insuficiencia cardiaca y fue trasladada a unidad de cuidados intensivos para su manejo. En esta unidad requirió apoyo ventilatorio no invasivo, terapia diurética y vasopresora, logrando resultados favorables al segundo día postoperatorio. La ecocardiografía fue fundamental para realizar el diagnóstico diferencial frente a un cuadro de hipotensión postoperatoria persistente.

Cardiomiopatía por citomegalovirus en el feto: Reporte de un cas / Fetal cardiomyopathy associated with cytomegalovirus infection: Case report

Fetal cytomegalovirus infection may be detected by ultrasonographic signs in various organs. Though uncommon, cardiac compromise may take various presentations, from isolated pericardial effusion to cardiomyopathy with calcifications. We report a case of fetal cytomegalovirus infection with cardiac involvement.

La infección fetal por citomegalovirus puede ser detectado por signos ultrasonográficos en diversos órganos. El compromiso del corazón es informado con poca frecuencia, pero puede abarcar desde efusión pericárdica aislada hasta miocardiopatía con calcificaciones. Comunicamos un caso de citomegalovirus fetal con afectación cardiaca.

Research Progress and Forensic Identification of Alcoholic Cardiomyopathy / 法医学杂志

With the rapid development of the social economy in China, the incidence of diseases caused by excessive drinking is gradually increasing as well. Alcoholic cardiomyopathy refers to long-term high intake of ethanol, and has typical dilated cardiomyopathy characteristics, such as, hemodynamic changes, symptoms, signs, and morphological features. It is a kind of cardiomyopathy that excludes other causes of dilated cardiomyopathy. Due to the lack of specific pathological changes, the forensic pathological identification of alcoholic cardiomyopathy can only be based on the patient's medical history and by ruling out other causes of cardiomyopathy. This paper reviews the pathogenesis and forensic identification of alcoholic cardiomyopathy in order to provide reference for forensic pathologists and clinicians.

Rastreo de familiares de una paciente con miocardiopatía hipertrófica obstructiva / Family Tracing of a Patient with Hypertrophic Obstructive Miocardiomyopathy

Resumen Introducción: La miocardiopatía hipertrófica familiar es la modalidad hereditaria autosómica dominante de la miocardiopatía hipertrófica, de penetrancia incompleta y expresión variable. Se exponen los estudios realizados a los familiares de un caso índice de miocardiopatía hipertrófica obstructiva, dado un 50% de probabilidad de padecer la enfermedad. Material y métodos: A partir de un caso índice se reclutaron familiares (hijos y hermanos) del caso índice, quienes fueron invitados a realizarse electrocardiograma, ecocardiograma transtorácico, prueba de esfuerzo y Holter cardíaco de manera voluntaria. Se aplicó una encuesta semiestructurada para obtener datos sociodemográficos, antecedentes personales, familiares y hábito de práctica de actividad física. Para el diagnóstico se siguieron los criterios utilizados por la guía de la Sociedad española de cardiología. Resultados: En los 11 casos evaluados se identificaron 4 miembros de la familia con criterios diagnósticos de miocardiopatía hipertrófica y 5 con hipertensión arterial. Conclusión: Con el estudio de los familiares de la paciente índice se logró descubrir la afectación de esta enfermedad en 2 familiares de primer grado y en 2 familiares de segundo grado, con predominancia para el sexo masculino (3:1); en la paciente femenina se encontró, además, hipertensión arterial y miocardiopatía hipertrófica asociadas a fibrilación auricular. Ninguno de ellos tuvo historia personal de síncope o resucitación de muerte súbita.

Abstract Introduction: Familial hypertrophic myocardiopathy is a hereditary autosomal dominant trait of hypertrophic myocardiopathy, of incomplete penetrance and variable expression. Studies carried out on the family of an index case of hypertrophic obstructive myocardiopathy gave a 50% probability of suffering from the disease. Material and methods: Family members (children, brothers and sisters) of an index case were recruited and were invited to volunteer to have a transthoracic electrocardiogram, stress test, and Holter test performed. A semi-structured questionnaire was completed in order to obtain sociodemographic data, personal and family history, and physical activity habits. The criteria used in the Spanish Cardiology Society guidelines were followed to make the diagnosis. Results: In the 11 cases evaluated, 4 members of the family were identified with diagnostic criteria of hypertrophic myocardiopathy, and 5 with arterial hypertension. Conclusions: With the study of the family members of the index patient, it was discovered that this disease affected 2 first degree and 2 second degree family members, with a predominance of male gender (3:1). The female case was also found to have arterial hypertension and hypertrophic cardiomyopathy associated with atrial fibrillation. None of them had a personal history of syncope or sudden death resuscitation.

Miocarditis aguda por citomegalovirus / Myocarditis due to cytomegalovirus

Resumen La miocarditis es una enfermedad inflamatoria del tejido miocárdico, la poca incidencia y la variabilidad del cuadro clínico hacen que el diagnóstico sea de exclusión. La causa es principalmente viral, aunque en los últimos años ha habido una transición en el virus causante. El tratamiento es controvertido y el pronóstico en los pacientes con miocardiopatía dilatada es ominoso. Comunicamos el caso clínico de una paciente que cursó con miocarditis secundaria a citomegalovirus en el servicio de Cardiología del Hospital Regional Puebla. Se realiza una revisión bibliográfica del tema para ayudar a establecer la causa, el tratamiento y posteriormente el pronóstico al alta hospitalaria.

Abstract Myocarditis is an inflammatory disease of the myocardium, due to the low incidence and the variability of the clinical course, the diagnosis is an exclusion one. The etiology is mainly viral, although a shift of the specific agent in recent years has taken place. Treatment is controversial and the prognosis related to dilated myocardiopathy is generally bad. We present the clinical case of a patient with myocarditis due to cytomegalovirus in the cardiology department of our Hospital. We conducted a bibliographic review of the topic to help to determine the etiology, the treatment and to be able to establish a prognosis at the discharge.

Pulse contour analysis calibrated by Trans-pulmonar thermodilution (Picco Plus ® ) for the perioperative management of a caesarean section in a patient with severe cardiomyopathy / Análise do contorno do pulso calibrado por termodiluição transpulmonar (Picco Plus ® ) para o manejo perioperatório de cesariana em paciente com miocardiopatia grave

ABSTRACT BACKGROUND: The delivery of cardiac patients is a challenge for the anaesthesiologist, to whom the welfare of both the mother and the foetus is a main issue. In case of caesarean section, advanced monitoring allows to optimize haemodynamic condition and to improve morbidity and mortality. OBJECTIVE: To describe the use of pulse contour analysis calibrated by Trans-pulmonar thermodilution (Picco Plus® for the perioperative management of a caesarean section in a patient with severe cardiomyopathy. CASE REPORT: We describe the case of a 28-year-old woman with a congenital heart disease who was submitted to a caesarean section under general anaesthesia for maternal pathology and foetal breech presentation. Intra- and post-operative management was optimized by advanced haemodynamic monitorization obtained by pulse contour wave analysis and thermodilution calibration (Picco Plus® monitor). The information about preload, myocardial contractility and postcharge was useful in guiding the fluid therapy and the use of vasoactive drugs. CONCLUSION: This case report illustrates the importance of advanced haemodynamic monitoring with an acceptably invasive device in obstetric patients with high cardiac risk. The increasing experience in advanced haemodynamic management will probably permit to decrease morbidity and mortality of obstetric patients in the future.

RESUMO JUSTIFICATIVA: O parto em pacientes cardíacas é um desafio para o anestesiologista, para o qual o bem-estar tanto da mãe quanto do feto é a questão principal. Em caso de cesariana, o monitoramento avançado permite melhorar a condição hemodinâmica e diminuir a morbidade e mortalidade. OBJETIVO: Descrever o uso da análise do contorno do pulso calibrado por termodiluição transpulmonar (Picco Plus®) para o manejo perioperatório de cesariana em paciente com miocardiopatia grave. RELATO DE CASO: Descrevemos o caso de uma paciente de 28 anos com uma doença cardíaca congênita, submetida a uma cesariana sob anestesia geral devido a afecção materna e apresentação fetal pélvica. O manejo nos períodos intraoperatório e pós-operatório foi aprimorado por monitoração hemodinâmica avançada obtida pela análise do contorno da onda de pulso e calibração por termodiluição (monitor Picco Plus®). As informações sobre pré-carga, pós-carga e contratilidade miocárdica foram úteis para orientar a reposição hídrica e o uso de medicamentos vasoativos. CONCLUSÃO: Este relato de caso ilustra a importância da monitoração hemodinâmica avançada com dispositivo aceitavelmente invasivo em pacientes obstétricas com alto risco cardíaco. O aumento do conhecimento no manejo hemodinâmico avançado provavelmente possibilitará a redução da morbidade e mortalidade de pacientes obstétricas no futuro.

Hallazgos ecocardiográficos en niños y adolescentes con diabetes mellitus tipo 1 / Echocardiographic findings in children and adolescents with type 1 diabetes mellitus

Introducción: las complicaciones cardiovasculares en los pacientes con diabetes mellitus tipo 1 pueden iniciarse desde edades tempranas. Objetivo: identificar las alteraciones de función ventricular en niños y adolescentes con diabetes mellitus 1. Métodos: se realizó un estudio de corte transversal en 52 niños y adolescentes (28 pacientes del sexo masculino y 24 del sexo femenino) con diagnóstico de diabetes mellitus tipo 1, con edades entre 2 y 18 años, así como 44 sujetos no diabéticos (22 pacientes del sexo masculino y 22 del sexo femenino), con edad similar al grupo de estudio, atendidos en el Servicio de Endocrinología del Hospital Pediátrico Docente William Soler, entre febrero de 2010 y agosto de 2011. A todos los pacientes se les realizó estudio ecocardiográfico utilizando un equipo Aloka 5500 y transductores de 3,5 y 5 MHz. Resultados: se encontraron diferencias significativas en la función diastólica de ambos ventrículos, así como en la función sistólica del ventrículo derecho en los pacientes con diabetes mellitus tipo 1 con respecto a los controles, y también una relación inversamente significativa con la variable de función diastólica del ventrículo derecho, el tiempo de evolución y la edad del diagnóstico de la diabetes mellitus tipo 1. Conclusiones: las alteraciones de la función diastólica y sistólica compatibles con miocardiopatía diabética se hallan presentes en niños y adolescentes con diabetes mellitus tipo 1, significativamente con mayor frecuencia que en los controles, lo que pudiera estar relacionado por el incremento de las hormonas durante la pubertad y la asociación con neuropatía autonómica cardiovascular(AU)

Introduction: cardiovascular complications can emerge at early ages in patients with type 1 diabetes mellitus. Objective: to identify alterations in ventricle function of children and adolescents with type 1 diabetes mellitus. Methods: a cross-sectional study was conducted in 52 children and adolescents (28 males and 24 females) diagnosed with type 1 diabetes mellitus, aged 2 to 8 years and in 44 non-diabetic subjects (22 males and 22 females) with ages similar to those of the study group. They had been all seen at the endocrinology service of William Soler pediatric teaching hospital February 2010 to August 2011 and they were all performed echocardiographic exams using Aloka 5500 equipment and 3,5 and 5 MHz transducers. Results: there were significant differences in the diastolic function of both ventricles as well as in the systolic function of the right ventricle of patients with type 1 diabetes mellitus in comparison with the controls. Likewise, there was a reversely significant relation with the diastolic function variable of the right ventricle, the length of progression and the ages at diagnosis of type 1 diabetes mellitus. Conclusions: the alterations in the diastolic and systolic functions compatible with diabetic myocardiopathy are present in children and adolescents with type 1 diabetes mellitus more frequently than in the control group, all of which could be related to the increase in the number of hormones in puberty and the association with autosomal cardiovascular neuropathy(AU)

Miocardiopatia periparto após cesariana: studo de casos / Peripartum cardiomyopathy after caesarean section: case studies

Miocardiopatia periparto é condição rara e indefinida,que afeta predominantemente mulheres dedescendência africana, multíparas e com idade acimados 30 anos. A principal característica consisteem insuficiência cardíaca materna no último mêsde gestação ou em até cinco meses após o parto. Adisfunção ventricular ocorre mesmo sem antecedentede cardiopatia e em mulheres previamentesaudáveis. Relatam-se dois casos de miocardiopatiaocorrendo após a realização de cesarianas, em umamultípara e em uma primigesta, sem antecedentesde cardiopatia. O diagnóstico foi estabelecido combase em exames complementares, incluindo-se radiografiade tórax, eletrocardiograma e ecocardiograma.O tratamento visa a melhorar o inotropismocardíaco e reduzir as manifestações de congestãopulmonar associados. Pesquisas são necessárias paraesclarecer e estabelecer a melhor opção terapêuticapara essa desafiante entidade clínica.

Peripartum cardiomyopathy is a rare condition of unknownetiology which predominantly affects women ofAfrican descent, multiparous, and over 30 years of age.The main characteristic of this condition is maternalheart failure in the last month of pregnancy or up to fivemonths postpartum. Ventricular dysfunction occurseven without history of heart disease, and in previouslyhealthy women. We report two cases of cardiomyopathyfollowing caesarean sections in a primigravida and in a multipara without previous heart disease. Diagnosis wasestablished based on complementary exams, includingchest X-ray, electrocardiogram, and echocardiography.Treatment seeks to improve cardiac inotropism and reducethe associated symptoms of pulmonary congestion.Further research studies are required to better clarifythe etiology of the disease, as well as to establish the besttreatment option for this challenging clinical entity.

Doença de Chagas congênita com manifestações pleomórficas: relato de caso / Congenital Chagas disease in adolescent with pleomorphic manifestations: case report

JUSTIFICATIVA E OBJETIVOS: A doença de Chagas é uma infecção parasitária, endêmica, em várias regiões do Brasil. Nas últimas décadas, a via de transmissão predominante foi modificada de vetorial para congênita, devido ao êxodo rural e aos métodos mais eficazes de combate aos vetores. Alguns pacientes jovens apresentam manifestações crônicas e intensas, consequentes da transmissão congênita. O objetivo deste estudo foi relatar um caso de uma paciente com doença de Chagas de transmissão vertical com manifestações variadas da doença. Ressalta-se a importância de se conhecer diversas formas de transmissão e, ademais, enfatiza-se a importância de realizar adequado pré-natal em áreas endêmicas para possível diagnóstico, tratamento precoce e acompanhamento clínico. RELATO DO CASO: Paciente do sexo feminino, 20 anos, solteira, apresentando há 8 meses dispneia aos mínimos esforços, precordialgia, epigastralgia e tosse seca, evoluindo com disfagia, confirmando-se o diagnóstico de insuficiência cardíaca com exames complementares. A investigação prosseguiu com provas sorológicas, eletrocardiograma, radiografia do coração e vasos da base (RCVB), Ecodopplercardiograma, confirmando-se: IgG positivo para Chagas em dois testes sorológicos (hemaglutinação indireta e imunofluorescência para T. Cruzi), alterações eletrocardiográficas difusas, megaesôfago grau II, cardiomegalia grau III. Foi realizado tratamento etiológico com benzonidazol (Rochagan®) para doença de Chagas crônica de início recente. Apesar da dificuldade na obtenção dos exames complementares,objetivando maior acurácia no diagnóstico e melhor acompanhamento do tratamento, concluiu-se que era um caso de doença de Chagas congênita, uma vez que a história familiar era positiva e a paciente não teve contato com triatomídeos (morou sempre na área urbana) e a transmissão vetorial está diminuindo devido às campanhas de erradicação dos vetores...

BACKGROUND AND OBJECTIVES: Chagas disease is a parasitic infection, endemic in several regions of Brazil. In recent decades the predominant route of transmission changed from vectorial to congenital, due to rural exodus and more effective methods for fighting the vectors. Some young patients have chronic and severe manifestations due to congenital transmission. The aim of this study was to report a case of a young adult patient with Chagas disease vertical transmission, with varied clinical manifestations of the disease. The importance of knowing the various forms of transmission was emphasized and also of the importance of prenatal assistance in endemic areas for possible early diagnosis, treatment and clinical follow up. CASE REPORT: Female patient, 20 years old, single, has presented experiencing, for the previous 8 months, dyspnea on exertion, constrictive chest pain, epigastric pain, and dry cough associated with progressive dysphagia; the diagnosis of heart failure was confirmed by complementary tests. The investigation progressed with serological tests, electrocardiogram, chest teleradiograph, echodopplercardiogram, confirming: IgG positive for Chagas disease, in two different serological tests (indirect hemagglutination assay and immunofluorescence for T cruzi), diffuse electrocardiographic changes, grade II megaesophagus, grade III cardiomegaly. Etiological treatment was conducted with benzonidazol (Rochagan®) for chronic Chagas disease of recent onset. Despite the difficulty in obtaining additional tests, aiming greater accuracy in the diagnosis and better monitoring of the treatment, it was concluded that it was a case of congenital Chagas disease, since family history is positive and the patient denied having contact with triatomids (hematophagus bugs) as she has always lived in urban areas, and because vectorial transmission is declining due to campaigns for eradication of the vectors...

Miocardiopatía Chagásica con taquicardia ventricular: Hospital Obrero Nº 2 "Caja Nacional de Salud" / Chagas Cardiomyopathy with ventricular tachycardia: Hospital Obrero Nº 2 "Caja Nacional de Salud"

La miocardiopatía chagásica es la inflamación del músculo cardiaco producida por el Trypanosoma cruzi. Los parásitos que se encuentran en los excrementos de la vinchuca (Tripanosoma infestans) son transmitidos al organismo luego de la alimentación del vector. Afecta varias áreas del organismo humano como son los nervios periféricos y las paredes del intestino grueso entre otros, sin embargo el corazón es uno de los órganos que con mayor frecuencia es afectado una vez producida la infección. Otros mecanismos de infección son a través de transfusiones de sangre contaminada y por medio de la placenta al feto. Se describe el caso de un paciente de 53 años transferido del policlínico N° 32 de la ciudad de Cochabamba con el diagnóstico de Insuficiencia Cardiaca Congestiva y bradicardia sinusal: El diagnóstico de bradicardia sinusal se realizó hace 2 años, donde se le propuso al paciente un implante de marcapasos, que este no realizó. Ingresó a salas de unidad de terapia intensiva con el diagnóstico de admisión de síndrome de taquicardia - bradicardia con antecedente de miocardiopatía chagásica. Al examen físico paciente presenta disnea, mareos y palpitaciones. Durante su internación el paciente presenta episodios de taquicardia sinusal de hasta 150 latidos por minuto y bradicardias sistólicas hasta 30 latidos por minuto, taquicardia ventricular y fibrilación auricular por lo que se considera como diagnóstico: miocardiopatía chagásica con taquicardia ventricular. Se decide implantar un marcapaso temporal y luego definitivo, se decide descontinuar la dosis de amiodarona, a la auscultación se presenta ritmo regular sin ruidos sobre agregados.

Chagasic miocardyopathy is a cardiac muscle imflamation caused by the Trypanosoma cruzi. Parasites can be found on vinchucas dregs.They are transmitted to human organism after vectors feeding. It affects several areas of the human body such as the peripheral nerves and colon walls however heart is one of the organs with a higher affection frequency. Other mechanisms of infection are dare contaminated blood transfusions and through the placenta to the fetus. Describing a case of a 53 years old patient whose transferred from the N° 32 policlinic of Cochabamba city diagnosed with congestive heart failure and sinusal bradycardia.The history of diagnosed sinusal bradicardia was made two years ago where he proposed to the patient; a pacemaker implantation was not performed. It Enter at the intensive care unit room with the admition diagnostic of bradycardia tachycardia syndrome and the chagasic cardiomyopathy antecedent. Physic exam revels, dyspnea, dizziness and palpitations. During its internation patient presents sinusal tachycardia up to 150 rates per minute and a systolic bradycardia of 30 rates per minute, ventricular tachycardia and auricular fibrillation so it is considered as a diagnosis: Chagas cardiomyopathy with ventricular tachycardia. So it is decided to implement a temporary pacemaker and then final is decided to discontinue the dose of amiodarone on auscultation regular rhythm occurs without noise on aggregate.

Miocarditis viral en el adulto / Viral myocarditis in the adult

La miocarditis viral es resultado de una inflamación del miocardio provocada por diferentes virus. Esta enfermedad cardíaca constituye sin duda una urgencia cardiovascular en el adulto por las complicaciones que ocasiona. El cuadro clínico se caracteriza por arritmias e insuficiencia cardíaca que pueden conducir a la muerte. La secuela más frecuente de la miocarditis viral a largo plazo es la miocardiopatía dilatada. En el presente trabajo se revisa etiología, fisiopatología, sintomatología, diagnóstico y tratamiento de la miocarditis viral en el adulto

Viral myocarditis is the result of a myocardial inflammation provoked by different viruses. This cardiac disease is doubtless a cardiovascular urgency in the adult due to its complications. The clinical picture is characterized by arrhythmias and cardiac failure that may to lead to death. The more frequent long-term myocarditis sequela is the dilated myocardiopathy. In present paper etiology, pathophysiology, symptomatology, diagnosis and treatment of viral myocarditis in the adult are reviewed

A lesão apical em cardiopatas chagásicos crônicos: estudo necroscópico / Apical lesions in Chagas' heart disease patients: an autopsy study

INTRODUÇÃO: A lesão apical ventricular é típica da cardiopatia chagásica e sua presença representa risco de fenômenos tromboembólicos. O objetivo deste trabalho é avaliar a frequência de LA à necropsia de portadores de cardiopatia chagásica crônica. MÉTODOS: Análise retrospectiva de necropsias de chagásicos maiores que 17 anos. Efetuada análise estatística comparativa das variáveis clínicas e dos achados necroscópicos entre o grupo A (com lesão apical) e o grupo B (ausência de lesão apical). RESULTADOS: Estudados 51 casos: 25 no grupo A (idade média de 53 anos, 64 por cento do sexo masculino)e 26 no B.. A LA localizava-se no ventrículo esquerdo em 80 por cento casos. No grupo B, a média de idade foi de 56 anos e 46,1 por cento eram do sexo masculino. A forma clínica prevalente nos dois grupos foi a miopática, mas arritmia cardíaca também esteve presente em ambos (57,9 por cento no grupo A e 32,1 por cento no B). Foi constatada a presença de trombos em 60 por cento dos casos do grupo A (53,3 por cento localizados na LA ) e 30,7 por cento no B; CONCLUSÕES: Houve predomínio da forma miopática nos casos com LA, com média de peso cardíaco maior em relação ao B. Em ambos os grupos observamos relação diretamente proporcional entre maior peso cardíaco e presença de tromboses. Houve predomínio do número de tromboses no grupo A, mais de 50 por cento eram localizadas na lesão, cujo diferencial clínico principal consistiu na presença maior de arritmias. A miopatia (com aumento de peso acima de 500g) foi primordial para aparecimento de tromboses.

INTRODUCTION: The presence of an apical ventricular lesion increases the risk of intracardiac thrombosis and thromboembolic phenomena. The study evaluated the incidence of apical lesions and intracardiac thrombosis in Chagas' heart disease patients at autopsy. METHODS: A retrospective review of autopsies of Chagas' heart disease patients was conducted. Statistical analysis included comparison of clinical variables and autopsy findings between two groups: group A (apical lesions) and group B (no apical lesions). RESULTS: A total of 51 cases of Chagas' disease patients were studied: 25 in group A (mean age 53 years-old; 64 percent male) and 26 in group B. Apical lesions were verified in the left ventricle in 80 percent of cases. The prevalent clinical subtype in both groups was myopathic, but significant cardiac arrhythmia was present in 57.9 percent of patients in group A, while 76.9 percent in group B did not present arrhythmias. Mean heart weight was 500.9g in group A and 408.4g in group B. The presence of thrombosis occurred in 60 percent of group A with 8 (53.3 percent) thrombi occurring in the apical lesion. CONCLUSIONS: The myopathic subtype was the most common clinical form in group A and the mean heart weight was statistically higher in this group. Clear prevalence of thrombosis was verified in group A, with 50 percent located in the apical lesion, whose main differential factor was a greater incidence of arrhythmias. Myopathy (heart weight above 500g) was primordial for the presence of thrombosis in both groups.

Anomalía de Ebstein en edad adulta / Ebstein anomaly in adulthood

La anomalía de Ebstein es una malformación congénita poco frecuente caracterizada por el adosamiento de los elementos de la válvula tricúspide en la cavidad del ventrículo derecho. Este adosamiento produce un desplazamiento del orificio fisiológico de la válvula tricúspide hacia la cavidad del ventrículo derecho. Generalmente se asocia a alteraciones propias de la válvula, como insuficiencia o estenosis tricuspídea, así con el desarrollo de insuficiencia cardiaca y la presencia de arritmias y síndrome de preexcitación. Aunque es un diagnóstico propio del neonato o del niño, se han descrito pacientes diagnosticados en la edad adulta. En estos últimos, la forma de presentación varía, y puede ir desde encontrarse asintomático hasta comenzar con arritmias. Se presenta el caso de una paciente de 70 años de edad que ingresó con el diagnóstico de flutter auricular, se decide cardioversión eléctrica retornando a ritmo sinusal. Al realizarle el ecocardiograma se diagnostica la anomalía de Ebstein. Se revisan las características clínicas, exámenes complementarios y tratamientos de esta entidad, así como la anatomía y embriología del aparato valvular tricuspídeo. El ecocardiograma continúa siendo el examen complementario de elección en esta entidad.

Ebstein anomaly is a non-frequent congenital malformation characterized by enclosing of tricuspid valve elements in right ventricle cavity. This enclosing produces a move of physiologic orifice of tricuspid valve within the right ventricle valve. Generally, it is associated with alterations typical of this valve, as insufficiency or tricuspid stenosis, as well as the development of heart insufficiency and the presence of arrhythmias and pre-excitation syndrome. Although it is a diagnosis characteristic of neonate or the children, has described patients diagnosed in adulthood. In past years, its presentation varies, and may be asymptomatic of start with arrhythmias. This is a female case presentation aged 70 admitted with a diagnosis of atrial flutter, thus the electric cardioversion to return to sinusal rhythm. In echocardiogram it is diagnosed an Ebstein anomaly. Clinical features, complementary examinations and treatment of this entity, as well as the anatomy and embryology of tricuspid valvular apparatus. Echocardiogram is the choice complementary examination in this entity.

Efeito de anestésicos locais com e sem vasoconstritor em pacientes com arritmias ventriculares / Effect of local anesthetics with and without vasoconstrictor agent in patients with ventricular arrhythmias

FUNDAMENTO: A utilização de anestésicos locais associados a vasoconstritores para tratamento odontológico de rotina de pacientes cardiopatas ainda gera controvérsia, em razão do risco de efeitos cardiovasculares adversos. OBJETIVO: Avaliar e comparar os efeitos hemodinâmicos do uso de anestésico local com vasoconstritor não-adrenérgico em pacientes portadores de arritmias ventriculares, em relação ao uso de anestésico sem vasoconstritor. MÉTODOS: Um estudo prospectivo randomizado avaliou 33 pacientes com sorologia positiva para doença de Chagas' e 32 pacientes com doença arterial coronariana, portadores de arritmia ventricular complexa ao Holter (>10 EV/h e TVNS), 21 do sexo feminino, idade de 54,73 + 7,94 anos, submetidos a tratamento odontológico de rotina com anestesia pterigomandibular. Esses pacientes foram divididos em dois grupos: no grupo I, utilizou-se prilocaína a 3 por cento associada a felipressina 0,03 UI/ml, e no grupo II, lidocaína a 2 por cento sem vasoconstritor. Avaliaram-se o número e a complexidade de extra-sístoles, a freqüência cardíaca e a pressão arterial sistêmica dos pacientes no dia anterior, uma hora antes, durante o procedimento odontológico e uma hora após. RESULTADOS: Não foram observadas alterações hemodinâmicas, nem aumento do número e da complexidade da arritmia ventricular, relacionados ao anestésico utilizado, em ambos os grupos. CONCLUSÃO: Os resultados sugerem que prilocaína a 3 por cento associada a felipressina 0,03 UI/ml pode ser utilizada com segurança em pacientes chagásicos e coronarianos, com arritmia ventricular complexa.

BACKGROUND: The routine use of local anesthetics associated to vasoconstrictors for the dental treatment of patients with cardiopathies is still controversial, due to the risk of adverse cardiovascular effects. OBJECTIVE: To evaluate and compare the hemodynamic effects of the use of local anesthetics with a non-adrenergic vasoconstrictor in patients with ventricular arrhythmia, when compared to the use of anesthetics without vasoconstrictor. METHODS: A prospective randomized study evaluated 33 patients with positive serology for Chagas' disease and 32 patients with coronary artery disease that presented complex ventricular arrhythmia at Holter monitoring (>10 EV/h and NSVT), of which 21 were females, aged 54.73 + 7.94 years, submitted to routine dental treatment with pterygomandibular anesthesia. These patients were divided in two groups: group I received prilocaine 3 percent associated with felypressin 0.03 IU/ml and group II received lidocaine 2 percent without vasoconstrictor. The number and complexity of extrasystoles were analyzed, as well as the heart rate and systemic arterial pressure of the patients on the day before, one hour before, during the procedure and one hour after the dental procedure. RESULTS: No hemodynamic alterations or increase in the number and complexity of the ventricular arrhythmia related to the anesthetic used in the dental procedure were observed in either group. CONCLUSION: The results suggest that prilocaine 3 percent associated to a felypressin 0.03 IU/ml can be safely used in patients with Chagas' disease or coronariopathy with complex ventricular arrhythmia.