Rehabilitación cardíaca integral en un caso de paciente con miocardiopatía por SARS-COV-2 / Comprehensive Cardiac Rehabilitation in a Case of a Patient with SARS-CoV-2 Cardiomyopathy

Introducción: Una complicación reportada en la infección por SARS-CoV-2 es la miocardiopatía. Se ha descrito que los pacientes pueden presentar un deterioro significativo de la clase funcional, que limita el desempeño en las actividades de la vida diaria, sociales, familiares y laborales. La rehabilitación cardíaca como componente integral es una alternativa de tratamiento no farmacológica en el manejo de secuelas cardiovasculares ocasionadas por SARS-CoV-2. Objetivo: Describir el impacto de la rehabilitación cardíaca integral en un caso de paciente con miocardiopatía por SARS-CoV-2. Caso clínico: Se presenta el caso de un hombre de 56 años de edad con miocardiopatía por SARS-CoV-2 quien realizó rehabilitación cardíaca integral. Conclusiones: La rehabilitación cardíaca con un componente de intervención integral multidisciplinar permitió al paciente mejorar la capacidad aeróbica, sintomatología, calidad de vida relacionada con la salud y reincorporarse a las actividades sociales, familiares y laborales(AU)

Introduction: A reported complication of SARS-CoV-2 infection is cardiomyopathy. It has been described that patients may present with significant impairment of functional class, limiting performance in activities of daily living, social, family and work. Cardiac rehabilitation as an integral component is a non-pharmacological treatment alternative in the management of cardiovascular sequelae caused by SARS-CoV-2. Objective: To describe the impact of comprehensive cardiac rehabilitation in a case of a patient with cardiomyopathy due to SARS-CoV-2. Case report: We present the case of a 56-year-old patient with SARS-CoV-2 cardiomyopathy who underwent comprehensive cardiac rehabilitation. Conclusions: Cardiac rehabilitation with a comprehensive multidisciplinary intervention component allowed the patient to improve aerobic capacity, symptomatology, health-related quality of life and reincorporation to social, family and work activities(AU)

Repeated stellate ganglion blockade for the treatment of ventricular tachycardia storm in patients with nonischemic cardiomyopathy: a new therapeutic option for patients with malignant arrhythmias / 中华心血管病杂志

Objectives: This study sought to describe our institutional experience of repeated percutaneous stellate ganglion blockade (R-SGB) as a treatment option for drug-refractory electrical storm in patients with nonischemic cardiomyopathy (NICM). Methods: This prospective observational study included 8 consecutive NICM patients who had drug-refractory electrical storm and underwent R-SGB between June 1, 2021 and January 31, 2022. Lidocaine (5 ml, 1%) was injected in the vicinity of the left stellate ganglion under the guidance of ultrasound, once per day for 7 days. Data including clinical characteristics, immediate and long-term outcomes, and procedure related complications were collected. Results: The mean age was (51.5±13.6) years. All patients were male. 5 patients were diagnosed as dilated cardiomyopathy, 2 patients as arrhythmogenic right ventricular cardiomyopathy and 1 patient as hypertrophic cardiomyopathy. The left ventricular ejection fraction was 37.8%±6.6%. After the treatment of R-SGB, 6 (75%) patients were free of electrical storm. 24 hours Holter monitoring showed significant reduction in ventricular tachycardia (VT) episodes from 43.0 (13.3, 276.3) to 1.0 (0.3, 34.0) on the first day following R-SGB (P<0.05) and 0.5 (0.0, 19.3) after whole R-SGB process (P<0.05). There were no procedure-related major complications. The mean follow-up was (4.8±1.1) months, and the median time of recurrent VT was 2 months. Conclusion: Minimally invasive R-SGB is a safe and effective method to treat electrical storm in patients with NICM.

Função atrial esquerda pelo método de speckle tracking: além da avaliação volumétrica / Left atrial function using speckle tracking: beyond volumetric evaluation

Atualmente, a avaliação da função atrial esquerda é um método emergente que pode ter relação com o prognóstico dos pacientes. Classicamente, as medidas estáticas de diâmetro, área e volume são as mais usadas com esta finalidade. A técnica conhecida como speckle tracking é capaz de fornecer informações dinâmicas do átrio esquerdo ao longo do ciclo cardíaco, assim como detectar alterações na função atrial esquerda em fases subclínicas, antes de ocorrerem aumentos volumétricos ou disfunções diastólicas. Valores de normalidade para o speckle tracking estão sendo propostos, mas as diferenças metodológicas e de técnicas empregadas dificultam sua padronização. Esta revisão da literatura se propõe a discutir os avanços na análise da função atrial esquerda, em especial via speckle tracking

Prevalencia y espectro de las enfermedades que predisponen a la muerte súbita cardiaca en niños mexicanos: una muestra obtenida del Hospital Infantil de México Federico Gómez / Prevalence and spectrum of diseases that predispose to sudden cardiac death in Mexican children: a sample obtained from The Federico Gomez Children's Hospital of Mexico

Resumen Objetivo: Determinar la prevalencia y espectro de las enfermedades que predisponen la muerte súbita cardiaca en niños mexicanos e identificar los principales signos y síntomas tempranos que pueden permitir al personal de salud sospechar acerca de estas enfermedades y referir a los pacientes a un hospital de tercer nivel de manera temprana. Métodos: La incidencia, prevalencia y prevalencia de periodo, así como los primeros síntomas, los datos clínicos y el seguimiento, se describen en todos los niños con enfermedades que predisponen a la muerte súbita cardiaca en el Hospital Infantil de México. Resultados: Cincuenta y nueve pacientes de 8 ± 5 años, 40 con miocardiopatías y 19 con enfermedades arritmogénicas hereditarias. La prevalencia del periodo fue de 9.5/1,000 pacientes/año. Los primeros síntomas más comunes fueron disnea, palpitaciones y síncope. En 9 casos se encontró un patrón de herencia mendeliana. Tres pacientes fallecieron de muerte súbita cardiaca durante el periodo de estudio. Conclusión: Las enfermedades que predisponen a la muerte súbita cardiaca en los niños no son muy conocidas por la comunidad médica y general. Todo niño con disnea, palpitaciones y/o síncope debe referirse para la búsqueda intensiva de estas enfermedades. Una evaluación cardiológica completa en todos los miembros de la familia está indicada.

Abstract Objective: To determine the prevalence and spectrum of diseases that predispose to sudden cardiac death in Mexican children, and to identify the main early signs and symptoms that can enable the health personnel to suspect these diseases and to refer the patients to a tertiary hospital in a timely manner. Methods: Incidence, prevalence, and period prevalence, as well as early symptoms, clinical data, and follow-up were recorded on all children found with diseases that predispose to sudden cardiac death in The Children's Hospital of Mexico. Results: The study included 59 patients, with a mean age of 8 ± 5 years old, with 40 cardiomyopathies, and 19 with inherited arrhythmogenic diseases. The period prevalence was 9.5/1,000 patients/year. The most common early symptoms were dyspnoea, palpitations, and syncope. A Mendelian inheritance pattern was found in 9 cases. Three patients died of sudden cardiac death during the period of the study. Conclusion: Diseases that predispose to sudden cardiac death in children are not very well known by the general medical community. Every child with dyspnoea, palpitations and/or syncope, should be referred for the intensive search of these diseases. A complete cardiological evaluation in all members of the family is indicated.

Brazilian consensus for diagnosis, management and treatment of transthyretin familial amyloid polyneuropathy / Consenso Brasileiro para o diagnóstico, manejo e tratamento da Polineuropatia Amiloidótica Familiar associada à Transtirretina

ABSTRACT Transthyretin familial amyloid polyneuropathy is an autosomal dominant inherited sensorimotor and autonomic polyneuropathy, which if untreated, leads to death in approximately 10 years. In Brazil, liver transplant and tafamidis are the only disease-modifying treatments available. This review consists of a consensus for the diagnosis, management and treatment for transthyretin familial amyloid polyneuropathy from the Peripheral Neuropathy Scientific Department of the Brazilian Academy of Neurology. The first and last authors produced a draft summarizing the main views on the subject and emailed the text to 10 other specialists. Relevant literature on this subject was reviewed by each participant and used for the individual review of the whole text. Each participant was expected to review the text and send a feedback review by e-mail. Thereafter, the 12 panelists got together at the city of Fortaleza, discussed the controversial points, and reached a consensus for the final text.

RESUMO Polineuropatia amiloidótica familiar é uma polineuropatia sensitivo-motora e autonômica de herança autossômica dominante, que caso não seja tratada leva a morte em aproximadamente 10 anos. O transplante de fígado e o tafamidis são os únicos tratamentos disponíveis no Brasil. Essa revisão consiste em um consenso do Departamento Científico de Neuropatias Periféricas da Academia Brasileira de Neurologia. O primeiro e último autores produziram um texto resumindo os principais aspectos sobre o tema e enviaram para os outros 10 especialistas por email. A literatura relevante sobre o assunto foi revisada por cada participante e utilizada para revisão individual do texto. Foi esperado que cada participante revisasse o texto e enviasse suas sugestões por e-mail. Finalmente, os 12 panelistas se encontraram na cidade de Fortaleza para discutir os pontos controversos e chegar a um consenso sobre texto final.

Heart Transplantation for Peripartum Cardiomyopathy: A Single-Center Experience / Transplante Cardíaco por Cardiomiopatia Periparto: Uma Experiência de Centro Único

Abstract Background: Peripartum cardiomyopathy is an idiopathic disorder defined by the occurrence of acute heart failure during late pregnancy or post-partum period in the absence of any other definable cause. Its clinical course is variable and severe cases might require heart transplantation. Objective: To investigate long-term outcomes after heart transplantation (HT) for peripartum cardiomyopathy (PPCM). Methods: Out of a single-center series of 1938 HT, 14 HT were performed for PPCM. We evaluated clinical characteristics, transplant-related complications, and long-term outcomes, in comparison with 28 sex-matched controls. Primary endpoint was death from any cause; secondary endpoints were transplant-related complications (rejection, infection, cardiac allograft vasculopathy). A value of p < 0.05 was considered of statistical significance. Results: PPCM patients and matched controls were comparable for most variables (all p values > 0.05), except for a higher use of inotropes at the time of HT in PPCM group (p = 0.03). During a median follow-up of 7.7 years, 16 patients died, 3 (21.5%) in PPCM group and 13 (46.5%) in control group. Mortality was significantly lower in PPCM group (p = 0.03). No significant difference was found in terms of transplant-related complications (p > 0.05). Conclusions: Long-term outcomes following HT for PPCM are favorable. Heart transplantation is a valuable option for PPCM patients who did not recover significantly under medical treatment.

Resumo Fundamento: A cardiomiopatia periparto é uma doença idiopática definida pela ocorrência de insuficiência cardíaca aguda durante a gravidez tardia ou pós-parto na ausência de qualquer outra causa definível. Seu curso clínico é variável e casos graves podem exigir transplante. cardíaco. Objetivo: Pesquisar os resultados a longo prazo após transplante cardíaco (TC) por cardiomiopatia periparto (CMPP). Métodos: De uma única série central de 1938 TC, 14 TC foram realizados por CMPP. Foram avaliadas características clínicas, complicações relacionadas ao transplante e resultados a longo prazo, em comparação com 28 controles pareados por gênero. O principal critério de avaliação foi a morte por qualquer causa; os critérios secundários foram complicações relacionadas ao transplante (rejeição, infecção, vasculopatia do aloenxerto cardíaco). Um valor de p < 0,05 foi considerado estatisticamente significante. Resultados: As pacientes com CMPP e controles pareados foram comparáveis ​​para a maioria das variáveis (todos os valores de p > 0,05), exceto para um maior uso de inotrópicos no momento do TC no grupo CMPP (p = 0,03). Durante um seguimento médio de 7,7 anos, 16 pacientes morreram, 3 (21,5%) no grupo CMPP e 13 (46,5%) no grupo controle. A mortalidade foi significativamente menor no grupo CMPP (p = 0,03). Não foram encontradas diferenças significativas em termos de complicações relacionadas ao transplante (p> 0,05). Conclusões: Os resultados a longo prazo após TC para CMPP são favoráveis. O transplante cardíaco é uma opção valiosa para pacientes com CMPP que não se recuperaram significativamente sob tratamento médico.

Structural heart disease as the cause of syncope

We described the clinical evolution of patients with structural heart disease presenting at the emergency room with syncope. Patients were stratified according to their syncope etiology and available scores for syncope prognostication. Cox proportional hazard models were used to investigate the relationship between etiology of the syncope and event-free survival. Of the 82,678 emergency visits during the study period, 160 (0.16%) patients were there due to syncope, having a previous diagnosis of structural heart disease. During the median follow-up of 33.8±13.8 months, mean age at the qualifying syncope event was 68.3 years and 40.6% of patients were male. Syncope was vasovagal in 32%, cardiogenic in 57%, orthostatic hypotension in 6%, and of unknown causes in 5% of patients. The primary composite endpoint death, readmission, and emergency visit in 30 days was 39.4% in vasovagal syncope and 60.6% cardiogenic syncope (P<0.001). Primary endpoint-free survival was lower for patients with cardiogenic syncope (HR=2.97, 95%CI=1.94-4.55; P<0.001). The scores were analyzed for diagnostic performance with area under the curve (AUC) and did not help differentiate patients with an increased risk of adverse events. The differential diagnosis of syncope causes in patients with structural heart disease is important, because vasovagal and postural hypotension have better survival and less probability of emergency room or hospital readmission. The available scores are not reliable tools for prognosis in this specific patient population.

Pacientes cirróticos com escore Child-Pugh C apresentam intervalos QT mais longo / Cirrhotic patients with Child-Pugh C have longer QT intervals

Fundamento e objetivos: A cardiomiopatia cirrótica tem sido usada para descrever a disfunção cardíaca crônica em pacientes cirróticos sem doença cardíaca estrutural prévia. Além disso, o prolongamento do intervalo QT é uma das alterações cardíacas mais importantes relacionadas à cirrose. Estudos prévios sugerem que o prolongamento QT está associado com uma taxa de mortalidade mais alta em pacientes cirróticos. O objetivo deste estudo foi analisar intervalos QTs segundo a gravidade da cirrose, medida pela classificação Child-Plugh. Materiais e métodos: Em um estudo transversal, um total de 67 pacientes com cirrose não alcoólica submeteu-se à avaliação clínica e eletrocardiográfica. A gravidade da cirrose foi classificada de acordo com o escore Child-Pugh. O intervalo QT foi medido por um eletrocardiograma de 12 derivações. Resultados: Os intervalos QTs foram mais longos em pacientes no grupo Child-Plugh C que nos grupos Child-Pugh A e B (459 ± 33 vs 436 ± 25 e 428 ± 34 ms, respectivamente, p = 0,004). Houve uma correlação positiva entre o intervalo QT e o escore Child-Pugh em indivíduos com escore Child-Pugh ≥ 7 (r = 0,50; p < 0,05) e intervalos QT ≥ 440 ms (r = 0,46, p < 0,05). Conclusão: O presente estudo mostrou que pacientes com cirrose Child-Plugh C apresentam intervalos QTs mais longos, o que reforçou a relação entre a gravidade da cirrose e achados eletrocardiográficos da cardiomiopatia cirrótica. Além disso, esse resultado foi encontrado em pacientes sem sintomas cardíacos, o que destacou a importância de um método simples e não invasivo, como o eletrocardiograma, para identificar pacientes cirróticos com cardiomiopatia

Background and aims: Cirrhotic cardiomyopathy has been used to describe chronic cardiac dysfunction in cirrhotic patients with no previous structural heart disease. Additionally, QT prolongation is one of the most important cardiac alterations related to cirrhosis. Previous studies suggest that QT prolongation is associated with a higher mortality rate among cirrhotic patients. The aim of this study was to analyze QT intervals according to cirrhosis severity as measured by the Child-Pugh classification. Materials and methods: In a cross-sectional study, a total of 67 patients with nonalcoholic cirrhosis underwent clinical and electrocardiographic evaluation. Cirrhosis severity was classified according to the Child-Pugh score. The QT interval was measured by a 12-lead electrocardiogram. Results: The QT intervals were longer in patients in the Child-Pugh C group than those in the Child-Pugh A and B groups (459 ± 33 vs 436 ± 25 and 428 ± 34 ms, respectively, p=0.004). There was a positive correlation between the QT interval and the Child-Pugh score in individuals with Child-Pugh scores ≥ 7 (r=0.50, p<0.05) and QT intervals ≥ 440 ms (r=0.46, p<0.05). Conclusion: The present study showed longer QT intervals in patients with Child-Pugh C cirrhosis, which reinforced the relationship between the severity of cirrhosis and electrocardiographic findings of cirrhotic cardiomyopathy. Moreover, this finding emerged in patients with no cardiac symptoms, which highlighted the importance of a simple and noninvasive method (ECG) to identify cirrhotic patients with cardiomyopathy

Identification of the difference in the pathogenesis in heart failure arising from different etiologies using a microarray dataset

OBJECTIVES: Clinically, patients with chronic heart failure arising from different etiologies receive the same treatment. However, the prognoses of these patients differ. The purpose of this study was to elucidate whether the pathogenesis of heart failure arising from different etiologies differs. METHODS: Heart failure-related dataset GSE1145 was obtained from the Gene Expression Omnibus database. Differentially expressed genes were identified using R. A protein-protein interaction network of the differentially expressed genes was constructed using Search Tool for the Retrieval of Interacting Genes. The modules in each network were analyzed by Molecular Complex Detection of Cytoscape. The Database for Annotation, Visualization and Integrated Discovery was used to obtain the functions of the modules. RESULTS: Samples contained in GSE1145 were myocardial tissues from patients with dilated cardiomyopathy, familial cardiomyopathy, hypertrophic cardiomyopathy, ischemic cardiomyopathy, and post-partum cardiomyopathy. The differentially expressed genes, modules, and functions of the modules associated with different etiologies varied. Abnormal formation of extracellular matrix was overlapping among five etiologies. The change in cytoskeleton organization was specifically detected in dilated cardiomyopathy. The activation of the Wnt receptor signaling pathway was limited to hypertrophic cardiomyopathy. The change in nucleosome and chromatin assembly was associated with only familial cardiomyopathy. Germ cell migration and disrupted cellular calcium ion homeostasis were solely detected in ischemic cardiomyopathy. The change in the metabolic process of glucose and triglyceride was detected in only post-partum cardiomyopathy. CONCLUSION: These results indicate that the pathogenesis of heart failure arising from different etiologies varies, which may provide molecular evidence supporting etiology-based treatment for heart failure patients.

Miocardiopatía periparto / Peripartum cardiomyopathy

Resumen Dentro de las indicaciones para practicarse una autopsia médico-legal en Costa Rica, se encuentran las muertes súbitas o causas judiciales para investigación por mala práctica médica, donde en ocasiones hay casos de mujeres en su embarazo tardío, así como en periodo post-parto. Este artículo busca conocer aspectos acerca de un diagnóstico específico que debe tomarse en cuenta durante el análisis forense en una autopsia de mujeres en periodo comprendido entre el último mes de embarazo y los 5 posteriores al parto: la miocardiopatía periparto. Esta entidad patológica se determina por su establecimiento en el lapso del tiempo anteriormente expuesto y trata de una forma de cardiomiopatía dilatada no tan habitual, pero con una morbimortalidad elevada y por tanto, en el ámbito de la patología forense es de carácter trascendental conocerlo. Este artículo busca conocer su fisiopatología, así como sus manifestaciones clínicas y diagnóstico dentro del enfoque patológico forense.

Abstract Some of the indications to perform a legal autopsy in Costa Rica, are the sudden deaths as well as judicial inquiries to investigate malpractices causes; in many cases these autopsies are of women in their last pregnancy month or in their post-natal period, specially in the first 5 months. This article search to learn aspects of a specific diagnosis that has to be thought during the forensic analysis of an autopsy of women in the period between the last month of gestation and the first 5 months postpartum: the peripartum cardiomyopathy. This pathology is an unusual kind of dilated cardiomyopathy with a high morbidity and mortality, and so is transcendental to know about it. The goal of this article is to learn about its pathophysiology as well as its clinical manifestations and diagnosis inside the forensic approach.

Estudo clínico e de fatores de risco associados às alterações cardiovasculares em cães / Clinical study and associated risk factors of cardiovascular alterations in dogs

O conhecimento clínico das alterações cardíacas em cães é de fundamental importância na formulação de diagnósticos diferenciais e instituição terapêutica na rotina da Clínica Médica de Pequenos Animais. Objetivaram-se com este estudo descrever os aspectos clínicos, epidemiológicos e radiográficos inerentes as alterações cardiovasculares de cães cardiopatas atendidos no Hospital Veterinário de Patos-PB (HV). Para isso foram avaliados os prontuários clínicos de todos os animais atendidos no período de Janeiro de 2007 a Dezembro de 2012, selecionando-se 131 cães (GCARD) que apresentavam histórico, alterações clínicas e radiográficas compatíveis com cardiopatia. Para fins comparativos, um grupo controle sem cardiopatia de igual número foi criado (GCON). Para análise estatística foram utilizados o teste de qui-quadrado ou teste exato de Fisher e regressão logística múltipla, com nível de significância de 5%, utilizando-se o programa SPSS 20.0 for Windows. Edema pulmonar e alterações morfológicas cardíacas foram os achados radiográficos mais frequentes. Não houve diferença quanto ao sexo e prevaleceram os animais mais velhos e da raça poodle. Tosse, cansaço e tristeza foram as queixas mais comuns. Sopro, tártaro e alterações oculares foram os sinais clínicos de maior ocorrência. As variáveis relacionadas ao engasgo, tontura/fraqueza, creptação pulmonar, ascite, obesidade, tumor e tártaro foram identificadas como associadas às cardiopatias. O modelo final apresentou ajuste satisfatório (teste de Hosmer e Lemeshow: χ2=0,221; P=0,994; R2 = 0,46). Mesmo que não seja possível determinar o diagnóstico etiológico das cardiopatias, faz-se necessário que um amplo conhecimento clínico e epidemiológico destes seja cada vez mais consolidado, de modo que as consequências fisiopatológicas dos distúrbios cardíacos sejam rapidamente identificados, trazendo qualidade de vida a todos os pacientes cardiopatas.(AU)

The clinical knowledge of cardiac disorders in dogs is fundamental to diagnose and apply appropriate therapy in the routine of a Small Animal Clinic. The objective of this study was to describe the clinical, epidemiological and radiographic aspects inherent to cardiovascular changes of cardiopathic dogs examined at the Veterinary Hospital of Patos/PB, Brazil. Clinical records of all animals treated between January 2007 and December 2012 were evaluated. The 131 dogs (GCARD) selected for the study had clinical and radiographic changes compatible with heart disease. For comparison, a control group (GCON) with the same number of dogs with no history of heart disease was formed. Pulmonary edema and cardiac morphological alterations were the most frequent radiographic finding. The results showed that gender was not a factor; however, older Poodles were more commonly affected by cardiac disorders. Cough, tiredness and sadness were the most frequent complaints. Cardiac murmur, dental plaque and ocular abnormalities were the most frequent clinical signs. Variables related to choking (OR=14.59), dizziness/weakness (OR=8.6), pulmonary crepitation (OR=37.34), ascites (OR=18.32), obesity (OR=12.28), tumor (OR=4.25) and dental plaque (OR=3.75) were identified as associated with heart disease. Even if it is not possible to determine the etiologic diagnosis of heart diseases, a broad clinical and epidemiological knowledge of these diseases is necessary, so that the pathophysiological consequences of heart disease are quickly identified, bringing quality of life to all cardiopathic patients.(AU)

Cardiomiopatía periparto / Peripartum Cardiomyopathy

La evolución normal del embarazo, parto o puerperio, puede verse afectada ocasionalmente por la aparición de insuficiencia cardiaca. La mayoría de las veces el origen del síndrome es la presencia de enfermedad cardiovascular previa (conocida o no), y la iatrogenia. El objetivo del trabajo fue presentar una revisión actualizada del concepto, epidemiología, mecanismos fisiopatológicos y estrategias de tratamiento de la cardiomiopatía periparto. Se realizó una revisión actualizada de la literatura internacional sobre el tema. Esta entidad se caracteriza por un síndrome de insuficiencia cardiaca por disfunción sistólica ventricular relacionada con el embarazo. Frecuentemente hace su aparición en el periparto, siendo el especialista en Obstetricia, u otro personal que asiste a la mujer gestante o puérpera quien a menudo tiene el primer contacto con la paciente. Aunque la etiología de la enfermedad es desconocida, nuevas teorías que involucran la predisposición genética, y su relación compleja con la hipófisis, placenta, cardiomiocito y células endoteliales, generan avances en el manejo específico de la enfermedad(AU)

The normal course of pregnancy, childbirth or postpartum, may occasionally be affected by the occurrence of heart failure. Most of the time the origin of this syndrome is the presence of previous cardiovascular disease (known or not), and iatrogenic. The objective of this paper is presenting an updated revision of the concept, its epidemiology, physiopathologic mechanisms and peripartum strategies of treatment of the cardiomyopathy. This entity is characterized by a syndrome of heart failure for systolic ventricular dysfunction related with pregnancy. Frequently it appears in peripartum. The specialist in obstetrics, or other personnel attending to the pregnant or puerperal women, is who often notices of the manifestations of this entity. Although the etiology of this disease is unknown, there are new theories involving genetic predisposition, and complex relationship with the pituitary, placenta, cardiomyocytes and endothelial cells, and generate improvements in this specific disease management(AU)

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.

Aortic Counterpulsation Therapy in Patients with Advanced Heart Failure: Analysis of the TBRIDGE Registry / Terapia de Contrapulsação Aórtica em Pacientes com Insuficiência Cardíaca Avançada: Análise do Registro TBRIDGE

Abstract Background: The use of aortic counterpulsation therapy in advanced heart failure is controversial. Objectives: To evaluate the hemodynamic and metabolic effects of intra-aortic balloon pump (IABP) and its impact on 30-day mortality in patients with heart failure. Methods: Historical prospective, unicentric study to evaluate all patients treated with IABP betwen August/2008 and July/2013, included in an institutional registry named TBRIDGE (The Brazilian Registry of Intra-aortic balloon pump in Decompensated heart failure - Global Evaluation). We analyzed changes in oxygen central venous saturation (ScvO2), arterial lactate, and use of vasoactive drugs at 48 hours after IABP insertion. The 30-day mortality was estimated by the Kaplan-Meier method and diferences in subgroups were evaluated by the Log-rank test. Results: A total of 223 patients (mean age 49 ± 14 years) were included. Mean left ventricle ejection fraction was 24 ± 10%, and 30% of patients had Chagas disease. Compared with pre-IABP insertion, we observed an increase in ScvO2 (50.5% vs. 65.5%, p < 0.001) and use of nitroprusside (33.6% vs. 47.5%, p < 0.001), and a decrease in lactate levels (31.4 vs. 16.7 mg/dL, p < 0.001) and use of vasopressors (36.3% vs. 25.6%, p = 0.003) after IABP insertion. Thirty-day survival was 69%, with lower mortality in Chagas disease patients compared without the disease (p = 0.008). Conclusion: After 48 hours of use, IABP promoted changes in the use of vasoactive drugs, improved tissue perfusion. Chagas etiology was associated with lower 30-day mortality. Aortic counterpulsation therapy is an effective method of circulatory support for patients waiting for heart transplantation.

Resumo Fundamento: A utilização da terapia de contrapulsação aórtica na insuficiência cardíaca avançada é controversa. Objetivos: Avaliar o efeito hemodinâmico e metabólico do balão intra-aórtico (BIA) e seu impacto sobre a mortalidade em 30 dias em pacientes com insuficiência cardíaca. Métodos: Estudo prospectivo histórico, unicêntrico, avaliando todos os pacientes tratados com BIA entre agosto/2008 e julho/2013, incluídos em registro institucional denominado TBRIDGE (The Brazilian Registry of Intra-aortic balloon pump in Decompensated heart failure - Global Evaluation). Analisaram-se variações na saturação venosa central de oxigênio (SVO2), lactato arterial e uso de fármacos vasoativos 48 horas após instalação do dispositivo. A mortalidade em 30 dias foi estimada pelo método de Kaplan-Meier e diferenças entre subgrupos foram avaliadas pelo teste de Log-rank. Resultados: Foram incluídos 223 pacientes com idade média de 49 ± 14 anos, fração de ejeção do ventrículo esquerdo média de 24 ± 10%, sendo 30% acometidos por Doença de Chagas. Em comparação à pré-instalação do BIA, após a instalação, houve aumento da SVO2 (51% vs. 66%, p < 0,001) e no uso de nitroprussiato (34% vs. 48%, p < 0,001), além de redução do lactato (31 vs. 17 mg/dL, p < 0,001) e no uso de vasopressores (36% vs. 26%, p = 0,003). A sobrevida em 30 dias foi de 69%, com menor mortalidade nos pacientes chagásicos comparativamente aos não chagásicos (p = 0,008). Conclusão: Nas primeiras 48 horas de utilização, o BIA promoveu mudança no uso de fármacos vasoativos e melhora da perfusão tecidual. A etiologia chagásica associou-se a menor mortalidade em 30 dias. A terapia de contrapulsação aórtica mostrou-se opção eficaz de suporte circulatório em pacientes candidatos a transplante cardíaco.

Multiorgan disorder syndrome (MODS) in an octagenarian suggests mitochondrial disorder / Síndrome mitocondrial no sindrómico con fallas de múltiples sistemas. Presentación de un caso

Non-syndromic, multi-organ mitochondrial disorders (MIDs) are frequently missed if treating physicians are not aware of them. We report a 85 years old Caucasian male, referred for tonic-clonic seizures, presenting with a plethora of abnormalities, including neurodermitis, atopic dermatitis, diabetes, hypertension, renal insufficiency, non-specific colitis, urine bladder lithiasis, bilateral cataracts, atrial fibrillation, diverticulosis, polyneuropathy, vitamin-D-deficiency, renal cysts, left anterior hemi-block, right bundle branch block, pulmonary artery hypertension, and heart failure. Neurological investigations revealed ptosis, quadriparesis, fasciculations, dysarthria, dysdiadochokinesia, tremor, hyperkinesia, ataxia, leukoencephalopathy, and basal ganglia calcification. Based upon this combination of abnormalities a non-syndromic mitochondrial multi-organ disorder syndrome (MIMODS, encephalo-myo-cardiomyopathy) was diagnosed.

Las alteraciones mitocondriales no sindrómicas y mutisistémicas pueden ser pasadas por alto si no se está consciente de su existencia. Presentamos un hombre de 85 años, referido por convulsiones tónico clónicas, que presentaba una plétora de anomalías tales como neurodermatitis, dermatitis atópica, diabetes, hipertensión, insuficiencia renal, colitis no específica, litiasis vesical, cataratas bilaterales, fibrilación auricular, diverticulosis, polineuropatía, deficiencia de vitamina D, quistes renales, hemibloqueo izquierdo anterior y bloqueo de rama derecha, hipertensión pulmonar e insuficiencia cardíaca. El estudio neurológico reveló la presencia de ptosis, cuadriparesia, fasciculaciones, disartria, disdiadocoquinesia, temblor, hiperquinesia, ataxia, leucoencefalopatía y calcificación de ganglios basales. Basados en estos hallazgos, se diagnosticó un síndrome mitocondrial no sindrómico con fallas de múltiples sistemas.

Transplante cardíaco em crianças: visão atual e perspectivas futuras / Heart transplant in children: current view and future perspectives

O transplante cardíaco em crianças tem sido opção nas cardiopatias congênitas complexas e nas cardiomiopatias refratárias a terapêutica convencional. A investigação diagnóstica da etiologia das cardiopatias tem avançado na última década como, por exemplo, a sequência de exons. A assistência circulatória tem sido utilizada como ponte para transplante e no tratamento de rejeições agudas e crônicas. Do ponto de vista imunológico, a prova cruzada virtual no momento do transplante principalmente para os pacientes sensibilizados tem sido útil e fundamental. Novas drogas imunossupressoras como tacrolimo, sirolimo e rituximabe apresentam-se como promissoras na prevenção e tratamento da rejeição. O transplante com sitema ABO incompativel é uma possibilidade para recém-nascidos pela escassez de doadores.

Heart transplant in children has been an option in complex congenital heart disease and cardiomyopathies that do not respond to conventional treatment. The diagnostic investigatio nof the etiology of heart disease has advanced in the last decade, an example of this being exon sequencing. Circulatory support has been used as a bridge to transplantation, and in the treatment of acute and chronic rejections. From an immunological point of view,the virtual crossmatch test at the time of transplant, particularly for sensitized patients, has been useful and essential. New immunosuppressant drugs such as tacrolimus, sirolimusand rituximab have shown promise in the prevention and treatment of rejection. ABO--incompatible transplantation is a possibility for newborn infants, due to the lack of donos.

O coração na obesidade: Mecanismos e consequências / Heart in obesity: Mechanism and consequences

A obesidade está associada a risco aumentado de doença cardiovascular, tanto pela associação a múltiplos fatores de risco que frequentemente a acompanham como pelos efeitos diretos do excesso de peso sobre a estrutura e a dinâmica cardíacas. A patogênese da disfunção miocárdica na obesidade é complexa e multifatorial. Alterações hemodinâmicas, neuro-humorais e no metabolismo dos substratos energéticos estão implicadas no desenvolvimento da miocardiopatia da obesidade. Acredita-se que as mudanças estruturais e funcionais no miócito cardíaco representem uma “má adaptação” às alterações metabólicas descritas na obesidade,levando à disfunção miocárdica progressiva e, finalmente, à insuficiência cardíaca. A perda de peso induz significativas mudanças tanto na estrutura miocárdica quanto na disfunção diastólica relacionada à obesidade. Abordagens farmacológicas que atuem sobre o remodelamento cardíaco, bloqueando a fibrogênese, tais como TGF-β1, espécies reativas de oxigênio ou endotelina-1, têm apresentado resultados promissores em estudos experimentais.

Obesity is associated with increased risk of cardiovascular disease, both by the presence of multiple well documente drisk factors that often accompany this condition, and the direct effects of excess of weight on cardiac structure and dynamics. The pathogenesis of myocardial dysfunction in obesity is complex and multifactorial. Neurohumoral, hemodynamic, and metabolism changes of energy substrates are implicated in the development of the cardiomyopathy of obesity. It is believed that the structural and functional changes in cardiac myocytes represent a “maladaptive”metabolic alteration described in obesity, leading to progressive heart failure and ultimately to myocardial dysfunction. Weight loss can induce significant changes inboth myocardial structure and diastolic dysfunction relatedto obesity. Pharmacological approaches that act on cardiac remodeling blocking fibrogenesis, such as TGF-β1, reactive oxygen species and endothelin-1, have shown promising results in experimental research.