Prevalence, risk factors and survival in dogs with myxomatous valve degeneration / [Prevalência, fatores de risco e sobrevivência em cães com degeneração mixomatosa valvar]

The objective of this study was to describe the prevalence, survival time, and risk factors of myxomatous mitral valve disease (MMVD) in dogs by a retrospective observational cohort study design of a Brazilian veterinary center, admitted from 2012 to 2018 (2.585 days). Considering the 105 dog files confirmed as MMVD, the prevalence of the disease was higher in small-breed dogs with ages from 10 to 19 years (30.3%). The survival time of the dogs at B1 stage (1.854 ± 145 days) was higher than the B2 (1.508+209) and C/D (930 ± 209). Higher risks of death (hazard ratio) were detected in the presence of syncope (2.5), pulmonary crackling (2.0), dyspnea (1.9), and higher values of vertebral heart scale (1.7), radiographic left atrium dimension (1.8), the ACVIM staging (1.7), and the duration of QRS complex (1.05). The results achieved demonstrated higher prevalence of MMVD in old small-breed dogs, associated with longer survival time in the B1 stage of the disease, and higher risk of death related to the presence of clinical signs, such as dyspnea, syncope, and pulmonary crackling, beyond some radiographic, echocardiographic, and electrocardiographic variables of heart or atrial enlargement.(AU)

O objetivo deste estudo foi descrever a prevalência, o tempo de sobrevivência e os fatores de risco da doença mixomatosa valvar mitral (DMVM) em cães, por meio de estudo de coorte observacional retrospectivo, os quais foram atendidos em um centro veterinário de 2012 a 2018 (2.585 dias). Considerando os 105 arquivos de cães com confirmação da DMVM, maior prevalência foi observada em raças pequenas com idades entre 10 e 19 anos (30,3%). O tempo de sobrevivência de cães em estágio B1 (1.854+145 dias) foi superior ao B2 (1.508+209) e ao C/D (930+209). Maior risco de morte (hazard ratio) foi observado na presença de síncope (2,5), crepitação pulmonar (2,0), dispneia (1,9), bem como de valores superiores de VHS (1,7), dimensão radiográfica do átrio esquerdo (1,8), estadiamento ACVIM (1,7) e duração do complexo QRS (1,05). Os resultados obtidos demonstraram maior prevalência de DMVM em cães idosos de raças pequenas, associada a maior tempo de sobrevida no estágio B1 da doença e a maior risco de morte relacionado à presença de sinais clínicos como dispneia, síncope e crepitação pulmonar, além de algumas variáveis radiográficas, ecocardiográficas e eletrocardiográficas de cardiomegalia ou aumento atrial esquerdo.(AU)

Echocardiographic features of pre- and postcapillary pulmonary hypertension with a focus on the right ventricle in dogs / Características ecocardiográficas da hipertensão pulmonar pré e pós-capilar em cães com foco no ventrículo direito

Pulmonary hypertension (PH) in dogs is a syndrome that can occur secondary to several causes, including left heart disease (postcapillary) and chronic respiratory disease (precapillary). This study evaluates morphological and functional consequences in the right ventricle (RV) of dogs with pre- and postcapillary PH through echocardiography, and also considers the severity of PH (mild, moderate or severe). Echocardiography was performed on 66 dogs of various breeds and weights (age >3 years old) which were assigned to three groups: postcapillary PH, which included mitral valve disease/endocardiosis, precapillary PH, which included chronic respiratory diseases (bronchitis, collapse of the trachea and primary lung cancer or metastasis), and finally, a healthy group of controls. The parameters for RV morphology were RV1, RV2, and RV3 for systole and diastole. The following measurements were used to assess RV systolic function: tricuspid annular plane systolic excursion (TAPSE), TAPSE:Ao (aorta), maximum velocity of the tricuspid systolic wave obtained by tissue Doppler (S'), S':Ao, right ventricle end-diastolic area (RVEDA); RVEDA:BSA (body surface area); flow velocity integral (FVI) and FVI:Ao. The variables were assessed using ANOVA. The results showed that RV3d, RV1s, S':Ao, S', and FVI were able to distinguish cases of pre- and postcapillary PH in this study. Remodeling of the RV of dogs with PH was observed, which can be influenced by the pre- or postcapillary origin of the PH, with dilation in dogs with postcapillary PH and severe PH. The results for RV systolic function were similar, with FVI and FVI:Ao showing that RV ejection function is reduced in dogs with postcapillary PH and with severe PH.(AU)

A hipertensão pulmonar (HP) em cães é uma síndrome que pode ocorrer secundária às diversas causas, dentre elas, a doença cardíaca esquerda (pós-capilar) e a doença respiratória crônica (pré-capilar). Essa é uma condição importante que motivou os objetivos do estudo: avaliar ecocardiograficamente as consequências morfológicas e funcionais no ventrículo direito (VD) dos cães acometidos com HP pré- e pós-capilar, considerando também o estágio de severidade da HP (leve, moderada ou severa). A ecocardiografia foi realizada em 66 cães de diversas raças e pesos, com pelo menos quatro anos de idade, que compuseram um grupo com doença valvar mitral, ou endocardiose, outro com doença respiratória crônica (bronquite, colapso de traqueia e neoplasia pulmonar primária ou metástase), e por último, um grupo saudável. Os parâmetros para a morfologia do VD foram RV1, RV2 e RV3 na sístole e na diástole. Para a avaliação da função sistólica do VD foram mensurados: excursão sistólica do plano anular tricúspide (TAPSE), TAPSE:Ao (aorta), velocidade máxima da onda sistólica da tricúspide obtida pelo doppler tecidual (S'), S':Ao, área do ventrículo direito no final da diástole (RVEDA); RVEDA:BSA (body superficie area); integral tempo velocidade (FVI) e FVI:Ao. As variáveis foram avaliadas por meio da ANOVA. Os resultados mostraram que RV3d, RV1s, S':Ao, S' e FVI foram capazes de distinguir casos de HP pré e pós-capilar neste estudo. Observou-se que há remodelamento do VD de cães com HP e este pode ser influenciado pela origem pré ou pós-capilar da HP, encontrando-se dilatado em cães com HP pós capilar e com HP severa. Os resultados para a função sistólica do VD foram similares, uma vez que o FVI e FVI:Ao mostraram que a capacidade de ejeção do VD está reduzida nos cães com HP pós-capilar e com HP severa.(AU)

La repolarización auricular y sus implicaciones clínicas / Atrial repolarisation and its clinical implications

Resumen La onda P en el electrocardiograma representa exclusivamente la despolarización de las aurículas puesto que la repolarización auricular queda oculta en el QRS. Sin embargo, cuando la repolarizacón auricular se hace evidente en el electrocardiograma se convierte en un elemento clave para el diagnóstico de algunas enfermedades. Diferente sucede en la repolarización ventricular; la repolarización auricular adopta el mismo sentido de la despolarización ya que sigue un sentido cráneo-caudal y se da desde el epicardio hacia el endocardio.

Abstract The P wave on the electrocardiogram exclusively represents atrial depolarisation, since atrial repolarisation is hidden in the QRS complex. However, when atrial repolarisation becomes evident in the electrocardiogram, it is a key element for the diagnosis of some diseases. Unlike what happens in ventricular repolarisation, atrial repolarisation adopts the same direction as depolarisation since it follows a craniocaudal course and it goes from the epicardium towards the endocardium.

Relato de endomiocardiofibrose de ventrículo direito em paciente nonagenária / Case history of right ventricular endomyocardial fibrosis in a nonagenarian patient

A endomiocardiofibrose é uma cardiopatia restritiva, pouco comum, com descrição recente. Foi descrita pela primeira vez em 1938, por Williams e estudada do ponto de vista anatomopatológico pelo patologista Davies, na África do Sul, recebendo o epônimo Doença de Davies em 1948. Considerando a história natural dessa doença, há maior incidência de diagnósticos nas terceiras e quartas décadas da vida, com média de 32 anos. Queremos chamar a atenção da comunidade científica, relatando o caso de uma senhora com diagnóstico inicial de endomiocardiofibrose aos 90 anos e indagar sobre a incidência e o subdiagnóstico dessa doença, bem como sua evolução

Endomyocardial fibrosis is a rare, recently-described, restrictive cardiopathy. It was first described in 1938 by Williams and studied from an anatomopathological perspective by pathologist Davies in South Africa, receiving the eponym Davies Disease in 1948. In terms of the natural history of this disease, there is a higher incidence of diagnoses in the third and fourth decades of life, at a mean age of 32 years. We want to raise the awareness of the scientific community by reporting the case of a woman who was first diagnosed with endomyocardial fibrosis at 90 years of age and pose questions about the incidence and subdiagnosis of this disease, as well as about its evolution

Anti-serum validation for use in immunohistochemistry for Trypanosoma cruzi detection

Abstract INTRODUCTION: The detection of Trypanosoma cruzi in tissue samples is important in many situations, such as testing of the reactivation of the infection. The detection of T. cruzi nests in endomyocardial biopsies (EMB) may be useful to evaluate graft rejection. Given their scarcity, such nests are not routinely identified. To increase the diagnosis sensitivity, immunohistochemistry (IHC) may serve as a promising strategy. Here, we validate an antiserum for the detection of T. cruzi infection by IHC. METHODS: We used 1) positive controls (PCs) - 13 EMB, 12 skin biopsies, and 1 heart with T. cruzi nests as sections stained with hematoxylin and eosin (HE); 2) negative controls - a) 10 explant hearts and 10 EMB with no amastigote nests or clinical/laboratory signs of chagasic infection; and b) eight samples with leishmaniasis, toxoplasmosis, or histoplasmosis; and 3) Cases - 31 EMB of chagasic patients with no parasite nests in HE sections but detected positive for T. cruzi DNA by polymerase chain reaction. As a primary antibody, a hyperimmune serum from T. cruzi-infected rabbits was used. RESULTS: IHC results were positive for 21 of 26 PCs (80.8%) and one case of cutaneous leishmaniasis. In 4 of 31 cases, IHC revealed nests (12.9%), which were undetected by conventional histological examination. CONCLUSIONS: This study shows that IHC with the tested antiserum increases the sensitivity of the diagnosis and may be recommended for routine use in EMB analyses of cardiac transplant patients with Chagas disease.

Characterization of Cardiopulmonary Exercise Testing Variables in Patients with Endomyocardial Fibrosis after Endocardial Resection / Caracterização das Variáveis do Teste de Esforço Cardiopulmonar em Pacientes com Endomiocardiofibrose após Cirurgia de Ressecção Endocárdica

Abstract Background: Endomyocardial fibrosis (EMF) is a rare disease, characterized by diastolic dysfunction which leads to reduced peak oxygen consumption (VO2). Cardiopulmonary exercise testing (CPET) has been proved to be a fundamental tool to identify central and peripheral alterations. However, most studies prioritize peak VO2 as the main variable, leaving aside other important CPET variables that can specify the severity of the disease and guide the clinical treatment. Objective: The aim of this study was to evaluate central and peripheral limitations in symptomatic patients with EMF by different CPET variables. Methods: Twenty-six EMF patients (functional class III, NYHA) were compared with 15 healthy subjects (HS). Functional capacity was evaluated using CPET and diastolic and systolic functions were evaluated by echocardiography. Results: Age and gender were similar between EMF patients and HS. Left ventricular ejection fraction was normal in EMF patients, but decreased compared to HS. Peak heart rate, peak workload, peak VO2, peak oxygen (O2) pulse and peak pulmonary ventilation (VE) were decreased in EMF compared to HS. Also, EMF patients showed increased Δ heart rate /Δ oxygen uptake and Δ oxygen uptake /Δ work rate compared to HS. Conclusion: Determination of the aerobic capacity by noninvasive respiratory gas exchange during incremental exercise provides additional information about the exercise tolerance in patients with EMF. The analysis of different CPET variables is necessary to help us understand more about the central and peripheral alterations cause by both diastolic dysfunction and restrictive pattern.

Resumo Fundamento: A endomiocardiofibrose (EMF) é uma doença rara, caracterizada por disfunção diastólica que leva à redução consumo de oxigênio (VO2) pico. O teste de esforço cardiopulmonar (TECP) tem se mostrado uma ferramenta fundamental na identificação de alterações centrais e periféricas. No entanto, a maioria dos estudos prioriza o VO2 pico como a variável principal, em detrimento de outras importantes variáveis do TECP que poderiam identificar a gravidade da doença e direcionar o tratamento clínico. Objetivo: O objetivo deste estudo foi avaliar limitações centrais e periféricas em pacientes com EMF sintomáticos por meio de variáveis do TECP. Métodos: Vinte e seis pacientes com EMF (classe funcional III, NYHA) foram comparados com 15 indivíduos controle saudáveis (CS). A capacidade funcional foi avaliada por TECP e funções sistólicas e diastólicas por ecocardiografia. Resultados: A idade e o gênero foram similares entre pacientes com EMF e CS. A fração de ejeção do ventrículo esquerdo foi normal em pacientes com EMF, porém diminuída em comparação aos CS. Os picos de frequência cardíaca, carga de trabalho, VO2, pulso de oxigênio (O2) e da ventilação pulmonar (VE) estavam diminuídos em pacientes com EMF em comparação aos CS. Ainda, os pacientes com EMF apresentaram Δ frequência cardíaca /Δ consumo de oxigênio e Δ consumo de oxigênio /Δ taxa de trabalho aumentados em comparação aos CS. Conclusão: A determinação da capacidade aeróbica por troca respiratória não invasiva durante exercício progressivo fornece informações sobre a tolerância ao exercício em pacientes com EMF. É necessária uma análise das diferentes variáveis do TECP para nos ajudar a compreender mais acerca das alterações centrais e periféricas causadas tanto pela disfunção diastólica como pelo padrão restritivo.

Exercise training of dogs with myxomatous valve disease / Treinamento físico de cães com doença mixomatosa valvar

The present study has the objective of evaluating the effects of exercise training, using moderate intensity walking (60 to 80% of maximum heart rate), from 30 to 50 minutes, three times a week, in alternate days, during eight weeks, on heart rate variability in dogs with myxomatous valve disease (MVD). For that, 20 dogs in stages B1 (1), B2 (14) and C2 (5) of MVD (ACVIM classification) were divided into untrained control group (CG, n=9) and training group (TG, n=11), and assessed at baseline (T0), after four (T1) and eight weeks (T2). Only one B1 and five B2 dogs completed the training program. In the time domain, the rMSSD was greater in TG in T1 (155,5+42,07) and T2 (199,8+83,54) than CG (T1:91,17+35,79 and T2:88,17+57,51). In the frequency domain, the variable High Frequency (HF) increased in TG in T1 (30950+25810) and T2 (40300+33870) when compared to the CG (T1:19090+23210 and T2:18810+22200) and within the group TG in T2 in relation to T0 (29340+20950). The proposed walking protocol is concluded to have increased the rMSSD and HF variables in TG, representing an increase of the parasympathetic tonus, justifying the indication of this therapy in B1 and B2 stages of MVD.(AU)

O presente estudo objetivou avaliar o efeito do treinamento físico, utilizando-se a caminhada de moderada intensidade (60 a 80% da frequência cardíaca máxima), por 30 a 50 minutos, três vezes por semana, em dias alternados, durante oito semanas, sobre a variabilidade da frequência cardíaca em cães com degeneração mixomatosa valvar (DMV). Para tanto, 20 cães nos estágios B1 (1), B2 (14) e C2 (5) da DMV (classificação ACVIM) foram divididos em grupo controle não treinado (GC, n=9) e grupo treinamento (GT, n=11) e avaliados no início do estudo (T0), após quatro (T1) e oito semanas (T2). Apenas um cão B1 e cinco B2 completaram o programa de treinamento. No domínio do tempo, a variável rMSSD foi maior no GT em T1 (155,5+42,07) e T2 (199,8+83,54) que o GC (T1:91,17+35,79 e T2:88,17+57,51). No domínio da frequência, a variável High Frequency (HF) aumentou no GT em T1 (30950+25810) e T2 (40300+33870) comparada à do GC (T1:19090+23210 e T2:18810+22200), e dentro do GT no T2 em relação ao T0 (29340+20950). Dessa forma, conclui-se que o protocolo de treinamento proposto aumentou as variáveis rMSSD e HF no GT, representando aumento do tônus parassimpático, o que fundamenta a indicação dessa terapia nos estágios B1 e B2 da DMV.(AU)

Fibrosis endomiocárdica / Endomyocardial fibrosis

Resumen La fibrosis endomiocárdica o endocarditis de Löffler es una patología de causa todavía desconocida, esta puede presentarse durante la evolución de diversas enfermedades de causa infecciosa, tumoral, autoinmune, medicamentos, etc. En muchos casos el presentar eosinofilia moderada (más de 1500 eosinófilos/microlitro) por largos períodos de tiempo puede producir toxicidad en diferentes órganos, entre ellos el corazón, produciendo disfunción del mismo por infiltración directa lo cual daña el tejido y también por las proteínas encontradas en los gránulos, principalmente la proteína catiónica eosinofílica y la proteína básica mayor que tienen predilección por el tejido endocárdico, llevando a su destrucción celular, lo que se traducirá en engrosamiento y fibrosis del subendocardio. Estas alteraciones conllevan a la cardiomiopatía restrictiva, siendo la fibrosis endomiocárdica su principal causa. Se presenta el caso de un paciente masculino de 30 a˜nos de edad, que ingresa al hospital por un cuadro de falla cardiaca aguda con evidencia en el ecocardiograma de ingreso de un componente restrictivo biventricular, el cual en diferentes series se presenta hasta en el 51% de los casos. El paciente presentaba una enfermedad hematológica de base, donde la eosinofilia era persistente durante más de 6 meses.

Abstract Endomyocardial fibrosis or Löffler endocarditis is a condition whose cause still remains unknown. It can develop during the progress of multiple infectious or tumour diseases, medication, etc. In many cases, showing moderate eosinophilia (more than 1500 eosinophils/ microliter) for long periods of time can cause organ toxicity, among them the heart. This produces a dysfunction of the heart due to direct infiltration, which damages the tissue, and also due to the proteins found in the granules, mostly eosinophil cationic protein and major basic protein, which have a predilection for endocardial tissue, leading to their cell destruction, which will translate into a subcardial enlargement and fibrosis. These alterations result in restrictive cardiomyopathy, endomyocardial fibrosis being their main cause. We present the case of a 30 year-old male patient who is admitted at the hospital due to acute heart failure with an admission echocardiogram that evidenced a restrictive biventricular component, present in up to 51% of the cases in different series. The patient had a base hematological disorder, where eosinophilia had been persistent for more than 6 months.

Factors associated with subendocardial ischemia risk in patients on hemodialysis / Fatores associados ao risco de isquemia subendocárdica em pacientes em hemodiálise

Abstract Introduction: Bone metabolism disorder (BMD) and vascular dysfunction contribute to excess cardiovascular mortality observed in hemodialysis patients. Vascular dysfunction, a new marker of atherosclerosis, can play a role in this risk. Even though associated with higher mortality in the general population, such vascular evaluation in patients on hemodialysis has not been extensively studied. Methods: In this cross-sectional study, hemodialysis patients were submitted to flow-mediated dilation, subendocardial viability ratio (SEVR) and ejection duration index assessment, in order to estimate the impact of BMD markers on vascular dysfunction. Results: A matched cohort of patients with (n = 16) and without (n = 11) severe secondary hyperparathyroidism (SHPT) was studied. Additionally, time spent under severe SHPT was also evaluated. Patients with severe SHPT had lower SEVR and higher ejection duration index, indicating higher cardiovascular risk. Lower SEVR was also associated to diastolic blood pressure (r = 0.435, p = 0.049), serum 25-Vitamin-D levels (r = 0.479, p = 0.028) and to more time spent under severe secondary hyperparathyroidism (SHPT), defined as time from PTH > 500pg/ml until parathyroidectomy surgery or end of the study (r = -0.642, p = 0.027). In stepwise multiple regression analysis between SEVR and independent variables, lower SEVR was independently associated to lower serum 25-Vitamin-D levels (p = 0.005), female sex (p = 0.012) and more time spent under severe SHPT (p = 0.001) in a model adjusted for age, serum cholesterol, and blood pressure (adjusted r² = 0.545, p = 0.001). Conclusion: Subendocardial perfusion was lower in patients with BMD, reflecting higher cardiovascular risk in this population. Whether early parathyroidectomy in the course of kidney disease could modify such results still deserves further investigation.

Resumo Introdução: Distúrbios do metabolismo ósseo (DMO) e alterações da função vascular contribuem para a elevada mortalidade de pacientes em hemodiálise. A disfunção vascular, um novo marcador de aterosclerose, pode contribuir para este risco. Apesar de associada a aumento de mortalidade na população geral, a avaliação de tal disfunção ainda não foi realizada de modo amplo em pacientes em hemodiálise. Métodos: Neste estudo transversal, pacientes em hemodiálise foram submetidos à avaliação da vasodilatação mediada por fluxo, razão de viabilidade subendocárdica (RVSE) e índice de duração de ejeção, como estimativas de avaliação dos marcadores de DMO sobre disfunção vascular. Resultados: Uma coorte pareada com (n = 16) e sem (n = 11) hiperparatireoidismo secundário (HPTS) grave foi estudada. Adicionalmente, o tempo transcorrido do diagnóstico de HPTS grave também foi avaliado. Pacientes com HPTS grave apresentaram menores valores de RVSE e maiores valores de índice de duração de ejeção, apontando maior risco cardiovascular. Baixa RVSE também foi associada à pressão arterial diastólica (r = 0,435, p = 0,049), níveis séricos de 25-Vitamina D (r = 0,479, p = 0,028) e maior tempo transcorrido desde diagnóstico de HPTS grave, definido como tempo em que o paciente permaneceu com valores de paratormônio superiores a 500 pg/ml até realização de cirurgia de paratireoidectomia ou término do estudo (r = -0,642, p = 0,027). Em regressão logística stepwise entre RVSE e variáveis independentes, menor RVSE foi independentemente associado a menores valores de 25-Vitamina D (p = 0,005), sexo feminino (p = 0,012) e maior tempo transcorrido desde diagnóstico de HPTS grave (p = 0,001) em um modelo ajustado para idade, colesterol sérico e pressão arterial (r2 ajustado = 0,545, p = 0,001). Conclusão: A perfusão subendocárdica foi menor em pacientes com DMO, refletindo o maior risco cardiovascular nesta população. Investigações adicionais são necessárias para definir se a paratireoidectomia precoce no curso da doença renal crônica poderia interferir neste risco.

Evaluation of the Differences of Myocardial Fibers between Acute and Chronic Myocardial Infarction: Application of Diffusion Tensor Magnetic Resonance Imaging in a Rhesus Monkey Model

OBJECTIVE: To understand microstructural changes after myocardial infarction (MI), we evaluated myocardial fibers of rhesus monkeys during acute or chronic MI, and identified the differences of myocardial fibers between acute and chronic MI. MATERIALS AND METHODS: Six fixed hearts of rhesus monkeys with left anterior descending coronary artery ligation for 1 hour or 84 days were scanned by diffusion tensor magnetic resonance imaging (MRI) to measure apparent diffusion coefficient (ADC), fractional anisotropy (FA) and helix angle (HA). RESULTS: Comparing with acute MI monkeys (FA: 0.59 ± 0.02; ADC: 5.0 ± 0.6 × 10(-4) mm2/s; HA: 94.5 ± 4.4°), chronic MI monkeys showed remarkably decreased FA value (0.26 ± 0.03), increased ADC value (7.8 ± 0.8 × 10(-4) mm2/s), decreased HA transmural range (49.5 ± 4.6°) and serious defects on endocardium in infarcted regions. The HA in infarcted regions shifted to more components of negative left-handed helix in chronic MI monkeys (-38.3 ± 5.0°-11.2 ± 4.3°) than in acute MI monkeys (-41.4 ± 5.1°-53.1 ± 3.7°), but the HA in remote regions shifted to more components of positive right-handed helix in chronic MI monkeys (-43.8 ± 2.7°-66.5 ± 4.9°) than in acute MI monkeys (-59.5 ± 3.4°-64.9 ± 4.3°). CONCLUSION: Diffusion tensor MRI method helps to quantify differences of mechanical microstructure and water diffusion of myocardial fibers between acute and chronic MI monkey's models.

Heart Disease in Disorders of Muscle, Neuromuscular Transmission, and the Nerves

Little is known regarding cardiac involvement (CI) by neuromuscular disorders (NMDs). The purpose of this review is to summarise and discuss the major findings concerning the types, frequency, and severity of cardiac disorders in NMDs as well as their diagnosis, treatment, and overall outcome. CI in NMDs is characterized by pathologic involvement of the myocardium or cardiac conduction system. Less commonly, additional critical anatomic structures, such as the valves, coronary arteries, endocardium, pericardium, and even the aortic root may be involved. Involvement of the myocardium manifests most frequently as hypertrophic or dilated cardiomyopathy and less frequently as restrictive cardiomyopathy, non-compaction, arrhythmogenic right-ventricular dysplasia, or Takotsubo-syndrome. Cardiac conduction defects and supraventricular and ventricular arrhythmias are common cardiac manifestations of NMDs. Arrhythmias may evolve into life-threatening ventricular tachycardias, asystole, or even sudden cardiac death. CI is common and carries great prognostic significance on the outcome of dystrophinopathies, laminopathies, desminopathies, nemaline myopathy, myotonias, metabolic myopathies, Danon disease, and Barth-syndrome. The diagnosis and treatment of CI in NMDs follows established guidelines for the management of cardiac disease, but cardiotoxic medications should be avoided. CI in NMDs is relatively common and requires complete work-up following the establishment of a neurological diagnosis. Appropriate cardiac treatment significantly improves the overall long-term outcome of NMDs.

Sindrome de inmunodeficiencia adquirida: compromiso del corazón y de los vasos / AIDS: cardiovascular involvement

Las complicaciones cardiovasculares del SIDA, son a menudo asintomáticas, aunque en ocasiones pueden constituir la causa directa de muerte. Puede comprometerse el pericardio, miocardio, endocardio y los vasos, ya sea como manifestación de la enfermedad de base o como resultado de la terapia antirretroviral y su efecto sobre los factores de riesgo, en el contexto de una patología que es actualmente de evolución crónica y con mejores expectativas de sobrevida gracias a las nuevas drogas empleadas para su control y tratamiento. El objetivo de este artículo es presentar una revisión de los aspectos más relevantes del SIDA que comprometen al corazón y los vasos.

The cardiovascular complications of AIDS, are often asymptomatic, although some may be direct cause of death. Pericardium, myocardium, endocardium, and vessels may be involved as a result of illness or the adverse effects of antiretroviral therapy on risk factors, Today has become a chronic condition with improved life expectancy thanks to the development of new drugs for its treatment and control The aim of this article is to present a review of the most relevant aspects of AIDS involving the heart and vessels.

Association between clinical features and histopathological findings in patients with left ventricular non-compaction cardiomyopathy / 中华心血管病杂志

<p><b>OBJECTIVE</b>To investigate the association between clinical and histopathological features in patients with left ventricular non-compaction cardiomyopathy (LVNC).</p><p><b>METHODS</b>Histopathological examinations were made on 11 LVNC recipient hearts from June 2004 to June 2014 in Fuwai Hospital, myocardial ultrastructure changes were detected using transmission electron microscopy. Association between clinical and pathological features were analyzed.</p><p><b>RESULTS</b>Patients were (24 ± 11) years old. There were 6 patients with mucus matrix LVNC, 3 patients with fibrous fatty infiltration, and 2 patients with cardiomyocytes proliferation. The gross morphological changes of LVNC hearts were characterized by numerous and prominent trabeculations with deep intratrabecular recesses in left ventricular myocardium. Ratios of the thicker noncompacted endocardial layer (N) and thin epicardial compacted layer (C) (N/C ratio) were ≥ 2.0, and the most serious lesions were located in the left ventricular apex, and followed by the left ventricular free wall. Histological microscopic examinations evidenced numerous matrix-like material and immature cardiomyocytes on endocardial tissue. Transmission electron microscopy revealed mitochondrial abnormalities on morphology, number, and distribution, underdeveloped cardiomyocytes and anomalies of intercalated disc structure, increased deposition of extracellular matrix-like substance and perinuclear glycogen. Pathological changes on cytoplasmic matrix and intercalated disc were present in all three tissue types of LVNC in this cohort and mitochondria hyperplasia was detected in patients with fibrous fatty infiltration. Heart weight ≥ 350 g is often associated with increased number of mitochondria. Increased cytoplasmic matrix was often detected in patients with LVEF ≥ 30% while intercalated disc anomalies were often detected in patients with LVEF < 30%.</p><p><b>CONCLUSION</b>Histological changes were closely related clinical features in patients with LVNC.</p>

Usefulness of ultrasound contrast media for cardiac output measurement with echocardiography / 대한수의학회지

The purpose of this study was to determine if contrast media would enhance visualization of the endocardium for assessment of left ventricle (LV) function. In addition, differences between pre- and post-contrast evaluation for the cardiac output measurements including the modified Simpson's method and automated contour tracking (ACT) method were examined. Ten clinically healthy adult beagle dogs (three males and seven females) between 2~3 years old and weighing 6.6~10.8 kg were used. Echocardiographic examinations were performed to compare pre- and postcontrast LV endocardium visualization using a segmental scoring method. Two different methods for measuring cardiac output were also compared. LV visualization was significantly enhanced in post-contrast echocardiography (p < 0.01). Significant differences between pre- and post-contrast measurements for the modified Simpson's method (p < 0.05) were also observed. No significant difference was found for the ACT method. Contrast echocardiography provides better LV chamber opacification and significantly improves wall segment visualization. Furthermore, contrast echocardiography for measuring cardiac output is helpful for the modified Simpson's method.

Sincronia e função ventricular esquerda em pacientes pediátricos portadores de marca-passo definitivo / Left ventricular synchrony and function in pediatric patients with definitive pacemakers

FUNDAMENTO: A estimulação crônica do ventrículo direito (EVD) induz um padrão de contração dessincronizado, produzindo assincronia interventricular e intraventricular. Muitos estudos têm mostrado a relação entre EVD e a forma e função ventricular esquerda (VE) comprometida. OBJETIVO: O objetivo deste estudo foi avaliar a sincronia e a função do VE em pacientes pediátricos que receberam EVD em comparação com aqueles que receberam estimulação do VE (EVE). MÉTODOS: As funções sistólica e diastólica e a sincronia do VE foram avaliadas em 80 pacientes pediátricos com bloqueio atrioventricular total não cirúrgico ou pós-cirúrgico, com estimulação a partir do endocárdio do ventrículo direito (VD) (n = 40) ou do epicárdio do VE (n = 40). Foram analisados dados ecocardiográficos obtidos antes da implantação do marca-passo, imediatamente após a implantação, e no final de um acompanhamento médio de 6,8 anos. RESULTADOS: A função diastólica do VE não se alterou em nenhum dos pacientes durante o acompanhamento. A função sistólica ventricular esquerda foi preservada nos pacientes que receberam EVE. No entanto, a fração de encurtamento e a fração de ejeção diminuíram de medianas de 41% ± 2,6% e 70% ± 6,9% antes da implantação para 32% ± 4,2% e 64% ± 2,5% (p < 0,0001 e p < 0,0001), respectivamente, no acompanhamento final. O atraso mecânico interventricular foi significativamente maior com a EVD (66 ± 13 ms) do que com a EVE (20 ± 8 ms). Da mesma forma, nos dois grupos houve uma diferença significativa entre os seguintes parâmetros: atraso mecânico do VE (EVD: 69 ± 6 ms, EVE: 30 ± 11 ms, p < 0,0001); atraso septo-lateral (RVP: 75 ± 19 ms, LVP: 42 ± 10 ms, p < 0,0001) e atraso septo-posterior (EVD: 127 ± 33 ms, EVE: 58 ± 17 ms, p < 0,0001). CONCLUSÃO: Quando comparado ao endocárdio do VD, o epicárdio do VE é um local ideal para a estimulação para preservar a sincronia e a função cardíaca.

BACKGROUND: Chronic right ventricular pacing (RVP) induces a dyssynchronous contraction pattern,producing interventricular and intraventricular asynchrony. Many studies have shown the relationship of RVP with impaired left ventricular (LV) form and function. OBJECTIVE: The aim of this study was to evaluate LV synchrony and function in pediatric patients receiving RVP in comparison with those receiving LV pacing (LVP). METHODS:LV systolic and diastolic function and synchrony were evaluated in 80 pediatric patients with either nonsurgical or postsurgical complete atrioventricular block, with pacing from either the RV endocardium (n = 40) or the LV epicardium (n = 40). Echocardiographic data obtained before pacemaker implantation, immediately after it, and at the end of a mean follow-up of 6.8 years were analyzed. RESULTS: LV diastolic function did not change in any patient during follow-up. LV systolic function was preserved in patients with LVP. However, in children with RVP the shortening fraction and ejection fraction decreased from medians of 41% ± 2.6% and 70% ± 6.9% before implantation to 32% ± 4.2% and 64% ± 2.5% (p < 0.0001 and p < 0.0001), respectively, at final follow-up. Interventricular mechanical delay was significantly larger with RVP (66 ± 13 ms) than with LVP (20 ± 8 ms). Similarly, the following parameters were significantly different in the two groups: LV mechanical delay (RVP: 69 ± 6 ms, LVP: 30 ± 11 ms, p < 0.0001); septal to lateral wall motion delay (RVP: 75 ± 19 ms, LVP: 42 ± 10 ms, p < 0.0001); and, septal to posterior wall motion delay (RVP: 127 ± 33 ms, LVP: 58 ± 17 ms, p < 0.0001). CONCLUSION: Compared with RV endocardium, LV epicardium is an optimal site for pacing to preserve cardiac synchrony and function.

KN-93, A CaMKII inhibitor, suppresses ventricular arrhythmia induced by LQT2 without decreasing TDR / 华中科技大学学报（医学）（英德文版）

Abnormal enhanced transmural dispersion of repolarization (TDR) plays an important role in the maintaining of the severe ventricular arrhythmias such as torsades de pointes (TDP) which can be induced in long-QT (LQT) syndrome. Taking advantage of an in vitro rabbit model of LQT2, we detected the effects of KN-93, a CaM-dependent kinase (CaMK) II inhibitor on repolarization heterogeneity of ventricular myocardium. Using the monophasic action potential recording technique, the action potentials of epicardium and endocardium were recorded in rabbit cardiac wedge infused with hypokalemic, hypomagnesaemic Tyrode's solution. At a basic length (BCL) of 2000 ms, LQT2 model was successfully mimicked with the perfusion of 0.5 μmol/L E-4031, QT intervals and the interval from the peak of T wave to the end of T wave (Tp-e) were prolonged, and Tp-e/QT increased. Besides, TDR was increased and the occurrence rate of arrhythmias like EAD, R-on-T extrasystole, and TDP increased under the above condition. Pretreatment with KN-93 (0.5 μmol/L) could inhibit EAD, R-on-T extrasystole, and TDP induced by E-4031 without affecting QT interval, Tp-e, and Tp-e/QT. This study demonstrated KN-93, a CaMKII inhibitor, can inhibit EADs which are the triggers of TDP, resulting in the suppression of TDP induced by LQT2 without affecting TDR.

Complication Rate of Transfemoral Endomyocardial Biopsy with Fluoroscopic and Two-dimensional Echocardiographic Guidance: A 10-Year Experience of 228 Consecutive Procedures

Endomyocardial biopsy (EMB) is one of the reliable methods for the diagnosis of various cardiac diseases. However, EMB can cause various complications. The purpose of this study is to evaluate the complication of transfemoral EMB with both fluoroscopic and two-dimensional (2-D) echocardiographic guidance. A total of 228 patients (148 men; 46.0+/-14.6 yr-old) who underwent EMB at Kyungpook National University Hospital from January 2002 to June 2012 were included. EMB was performed via the right femoral approach with the guidance of both echocardiography and fluoroscopy. Overall, EMB-related complications occurred in 21 patients (9.2%) including one case (0.4%) with cardiac tamponade requiring emergent pericardiocentesis, four cases (1.8%) with small pericardial effusion without pericardiocentesis, two cases (0.9%) with hemodynamically unstable ventricular tachycardia (VT), one case (0.4%) with nonsustained VT, one case (0.4%) with tricuspid regurgitation, twelve cases (5.3%) with right bundle branch block. There was no occurrence of either EMB-related death or cardiac surgery. Left ventricular ejection fraction was significantly lower (32.0+/-18.7% vs 42.0+/-19.1%, P=0.023) and left ventricular end-diastolic dimension was larger (60.0+/-10.0 mm vs 54.2+/-10.2 mm, P=0.013) in patients with EMB related complications than in those without. It is concluded that transfemoral EMB with fluoroscopic and 2-D echocardiographic guidance is a safe procedure with low complication rate.