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Emergency medical system transportation has been shown to reduce treatment times in ST-segment elevation myocardial infarction. The authors studied the Portuguese National Registry of Acute Coronary Syndromes to determine the nationwide impact of the emergency medical system transportation in the treatment of ST-segment elevation myocardial infarction.A multicentric, nationwide, retrospective study of ST-segment elevation myocardial infarction patients inserted in the National Registry from 2010 to 2017 was performed. The patients were divided into: Group I, composed of patients transported by emergency medical system, and Group II, patients arriving to the Emergency department by other means.Of the 5702 patients studied, 25.9% were transported via emergency medical system. Rates of emergency medical system activation increased by 17% in the last 7 years. The emergency medical system provided a higher rate of transport to a percutaneous coronary intervention capable centre, of Emergency department bypass, of on-site fibrinolysis, and ensured a 59-minute reduction of the median reperfusion time (p0.001). There was no difference in in-hospital mortality.In this nationwide cohort, emergency medical system transportation is associated with a reduction in reperfusion times. It provides a higher amount of salvaged myocardium and reduces the incidence of acute heart failure. However, emergency medical system use did not result in lower in-hospital mortality, probably due to confounding factors of higher disease severity and comorbidity.The benefits associated with emergency medical system based transportation of patients with ST-segment elevation myocardial infarction do not translate into lower in-hospital mortality.Introdução: O transporte através de sistemas de emergência médica reduz os tempos de tratamento no enfarte agudo do miocárdio com elevação do segmento ST. Os autores estudaram o Registo Nacional de Síndromes Coronários Agudos para avaliar o impacto nacional do transporte através de sistema de emergência médica no tratamento do enfarte agudo do miocárdio com elevação do segmento ST. Material e Métodos: Foi realizado um estudo retrospetivo, multicêntrico de doentes com enfarte agudo do miocárdio com elevação do segmento ST inseridos no Registo Nacional desde 2010 até 2017. Os doentes foram divididos em Grupo I, representando doentes transportados por viaturas de emergência médica e Grupo II, doentes que chegaram ao Serviço de Urgência por outros meios. Resultados: Do total de 5702 doentes, 25,6% foram transportados por viaturas de emergência médica. Registou-se um aumento no uso de viaturas de emergência médica de 17% nos últimos sete anos. Os sistemas de emergência médica garantiram uma maior taxa de transporte para centros capazes de realizar intervenção coronária percutânea, de bypass do Serviço de Urgência e de fibrinólise no local. O transporte através de viaturas de emergência médica conseguiu uma redução da mediana do atraso para a reperfusão de 59 minutos (p0,001). Não houve diferença na mortalidade intra-hospitalar. Discussão: Nesta amostra nacional, é evidente que os sistemas de emergência médica reduziram significativamente os tempos de reperfusão, associando-se a uma menor incidência de insuficiência cardíaca aguda pós-enfarte. No entanto, esse benefício não resultou numa menor mortalidade intra-hospitalar, provavelmente devido ao facto dessa população representar um subgrupo de doentes com doença mais grave e mais comorbilidades. Conclusão: Os benefícios associados ao uso de sistemas de emergência médica no transporte de doentes com enfarte agudo do miocárdio com elevação do segmento ST não se traduziram numa menor mortalidade intra-hospitalar.

Case report A 35-year-old man was admitted following a recovered cardiorespiratory arrest. He was attacked by another man with multiples blunts by a wood stick in the chest and head. An eyewitness statement indicated that the victim had lost consciousness and collapsed after being hit in the chest. He was in cardiac arrest, and a nearby healthcare professional provided first aid with cardiopulmonary resuscitation. The first electrocardiogram (ECG) rhythm strip, fifteen minutes later, identified ventricular fibrillation (VF) (). Sixteen electrical [...]

Funding Acknowledgements Type of funding sources: None. INTRODUCTION Implantable loop recorders (ILR) are a powerful diagnostic tool for heart rhythm diseases, and particularly useful when symptoms are infrequent or when long-term data are required. The main indication is for study of syncope/presyncope due to suspected cardioinhibitory etiology. OBJECTIVE To evaluate the diagnostic profitability of ILR in patient (P) with syncope/ presyncope and to evaluate the effect of symptons on follow-up. METHODS Included P undergoing ILR implantation in 6 consecutive years, to study syncope/presyncope. Information was collected on P characteristics, indication, diagnostic outcome and subsequent management and complications. A follow-up (FU) of 1 year and 3 years were done. Diagnostic outcome was based in symptom-rhythm correlation. Symptons and correlation to ECG during the FU and management after the diagnosis were assessed. RESULTS 99 P were selected. Evaluation of syncope in 91.9% (n = 91) and presyncope in 8.1% (n = 8). 54.5% female, median age 59.4 ± 17.4 years. 55.8% (n = 53) completed the 3 years FU and 84.2% (n = 80) completed the 1 year FU. Death occurred in 4% (n = 4) during the FU. ILR results led to device implantation in 35% of the P (22 pacemakers and 1 ICD) in 1 year FU, with a median time to implantation of 11.6 months after ILR, and the majority of ILR motivated by syncope (87%). If we consider only P that finished the 3 years of FU, in 47.2% P were implanted a device. The most common arrhythmic finding were AV block (47.8%), followed by sinus pauses / asystole (43.5%), AF with slow ventricular rate (4.3%) and VT (4.3%). 4.9% of P experienced a complication related to the device (2 infection and 3 non-infectious pain), that resulted in explantation. 60.9% of the P were symptomatic during the FU, with 24.1% achieving symptom-rhythm correlation and 36.8% who did not. Device implantation was associated with the presence of symptons in FU (82.6% vs 17.4%, p = 0.012) and symptom-rhythm correlation (95% vs 5%,p = 0.001). 38.7% (n = 27) of the P finished the 3 years of FU without a diagnostic outcome or detectable event, with 44.4% being assymptomatic and 55.6% presenting symptoms without ECG correlation. Compared to the P that implanted a device, this type of P was frequently of female sex (57.6% vs 30.4%, p = 0.045), younger age (54.8 ± 18.1 vs 65.8 ± 12.8 years, p = 0.014), with less cardiovascular risk factors like dyslipidemia (37.0% vs 78.3%, p = 0.003) and arterial hypertension (48.1% vs 73.9%, p = 0.05), AF (0% vs 19%, p = 0.025), previous history of myocardial infarction and percutaneous coronary intervention (3.2% vs 17.4%, p = 0.05; 3.0% vs 21.7%, p = 0.028) and more frequently with history of depression (51.9% vs 22.7%, p = 0.037). CONCLUSION In this study, ILR monitoring led to a device implantation in 47.2% of the P that finished the 3 years FU. The presence of symptons and symptom-rhythm correlation during FU was associated with device implantation.

Introduction and Objectives: The aim of this study was to assess the impact of a conservative strategy in non-ST-segment elevation myocardial infarction in patients in the Portuguese Registry of Acute Coronary Syndromes. Methods: The 3780 patients included in the study over a three-year period were divided into three groups: group 1, patients treated by a conservative strategy during hospitalization; group 2, patients who underwent coronary angiography without percutaneous coronary intervention (PCI); and group 3, patients who underwent PCI. Clinical and procedural data and in-hospital complications were compared. The primary endpoint was defined as in-hospital or one-year mortality and the secondary endpoint as the presence of at least one of the following in-hospital complications: major bleeding according to the GUSTO criteria, need for blood transfusion, invasive ventilation, heart failure or reinfarction. Results: Of the patients analyzed, 16.5% were treated by a conservative strategy. Patients in this group were older, more often women, and had more high-risk factors. A conservative strategy was associated with a higher rate of the primary endpoint – in-hospital mortality (10.6% vs. 1.1% vs. 0.6% in groups 1, 2 and 3, respectively, p

Resumo: Os autores apresentam um raro caso de miocardiopatia hipertrófica (MCH) associada a ventrículo esquerdo não compactado (VENC) e fístulas das artérias coronárias para o ventrículo esquerdo (VE) numa doente de 42 anos, que se apresenta com enfarte agudo do miocárdio sem elevação do segmento ST (EAMSEST). A coronariografia, a ecocardiografia transtorácica (ETT) e ressonância magnética cardíaca (RMC) revelaram as alterações estruturais do VE e da árvore coronária. Abstract: The authors present a rare case of hypertrophic cardiomyopathy associated with left ventricular noncompaction cardiomyopathy and coronary artery-left ventricular fistulae in a 42-year-old woman presenting with non-ST-elevation myocardial infarction. Coronary angiography, transthoracic echocardiography and cardiac magnetic resonance revealed the structural abnormalities of the left ventricle and the coronary tree. Palavras-chave: Miocardiopatia hipertrófica, Ventrículo esquerdo não compactado, Fístula coronário-ventricular, Enfarte do miocárdio embólico, Recesso intramiocárdico, Mutação genética, Keywords: Hypertrophic cardiomyopathy, Left ventricular noncompaction cardiomyopathy, Coronary artery-left ventricular fistulae, Embolic myocardial infarction, Intramyocardial recess, Genetic mutation

The authors present a rare case of hypertrophic cardiomyopathy associated with left ventricular noncompaction cardiomyopathy and coronary artery-left ventricular fistulae in a 42-year-old woman presenting with non-ST-elevation myocardial infarction. Coronary angiography, transthoracic echocardiography and cardiac magnetic resonance revealed the structural abnormalities of the left ventricle and the coronary tree. Resumo: Os autores apresentam um raro caso de miocardiopatia hipertrófica (MCH) associada a ventrículo esquerdo não compactado (VENC) e fístulas das artérias coronárias para o ventrículo esquerdo (VE) numa doente de 42 anos, que se apresenta com enfarte agudo do miocárdio sem elevação do segmento ST (EAMSEST). A coronariografia, a ecocardiografia transtorácica (ETT) e ressonância magnética cardíaca (RMC) revelaram as alterações estruturais do VE e da árvore coronária. Keywords: Hypertrophic cardiomyopathy, Left ventricular noncompaction cardiomyopathy, Coronary artery-left ventricular fistulae, Embolic myocardial infarction, Intramyocardial recess, Genetic mutation, Palavras-chave: Miocardiopatia hipertrófica, Ventrículo esquerdo não compactado, Fístula coronário-ventricular, Enfarte do miocárdio embólico, Recesso intramiocárdico, Mutação genética

Resumo: Os autores apresentam dois casos de pericardite purulenta secundária a pneumonia por pneumococos, um deles num doente sem antecedentes patológicos conhecidos. É feita uma sistematização da abordagem diagnóstica ao derrame pericárdico de moderadas dimensões e uma revisão da pericardite purulenta, uma entidade muito rara na era da antibioterapia. A constatação de derrame pericárdico com quadro de febre persistente, com ou sem origem conhecida, fundamentalmente no doente com compromisso imune, mas também no aparentemente saudável, deve levantar-se sempre a possibilidade de pericardite purulenta. Abstract: The authors present two cases of purulent pericarditis secondary to pneumococcus pneumonia, a rare entity in the antibiotic era, one of them in an apparently healthy person. A systematized diagnostic approach to moderate pericardial effusion is presented, together with a review of purulent pericarditis. The presence of pericardial effusion with persistent fever with or without known etiology, particularly in the immunocompromised but also in the apparently healthy patient, should always raise the possibility of purulent pericarditis. Palavras-chave: Pericardite bacteriana, Pericardite purulenta, Derrame pericárdico, Tamponamento, Pneumonia, Keywords: Bacterial pericarditis, Purulent pericarditis, Pericardial effusion, Tamponade, Pneumonia

Left ventricular noncompaction (LVNC) is now recognized as a distinct form of cardiomyopathy with a clinical presentation and natural history of its own. Common manifestations of LVNC include heart failure, ventricular arrhythmias and embolic events, but serious atrioventricular conduction disturbances are rarely reported in the literature. The authors describe the case of a 40-year-old woman who went to the emergency department due to syncope. The ECG revealed left bundle branch block (LBBB) and 2:1 atrioventricular block (AVB) and the patient was admitted for pacemaker implantation. During hospitalization she developed torsade de pointes and complete AVB with increased QTc. The echocardiogram showed images compatible with LVNC. This case provides additional evidence that LVNC may be complicated by 2:1 (or complete) AVB, intraventricular conduction disturbances (LBBB) and repolarization abnormalities (long QT). This combination of electrocardiographic changes has rarely been reported in the literature. We describe a series of affected patients, focusing on electrocardiographic characteristics. Resumo: O ventrículo esquerdo não compactado (VENC) tem sido reconhecido como uma forma distinta de miocardiopatia com uma apresentação clínica e história natural próprias. As manifestações comuns de VENC são insuficiência cardíaca, arritmias ventriculares e eventos embólicos. A presença de perturbações da condução auriculoventricular de maior gravidade encontra-se raramente descrita na literatura. Os autores descrevem o caso clínico de uma paciente com 40 anos de idade, que recorreu ao Serviço de Urgência por síncope. O ECG revelou bloqueio completo de ramo esquerdo do feixe de His e bloqueio auriculoventricular (BAV) 2:1, tendo sido internada para implantação de pacemaker. Durante o internamento, desenvolve fenómenos de torsade de pointes e de BAV completo com aumento do intervalo QTc. O ecocardiograma revelou imagens compatíveis com VENC. Com este caso, os autores fornecem evidência adicional de que a presença de VENC pode ser complicada por BAV 2:1 (ou completo), perturbações da condução intraventricular (BCRE) e da repolarização (QT longo), alterações eletrocardiográficas concomitantes pouco descritas na literatura. Descreve-se uma série de pacientes afetados, com foco nas características eletrocardiográficas. Keywords: Left ventricular noncompaction, Atrioventricular block, Torsade de pointes, Palavras-chave: Ventrículo esquerdo não compactado, Bloqueio auriculoventricular, Torsade de pointes

Background: Heart failure (HF) is a highly prevalent syndrome. Although the long-term prognostic factors have been identified in chronic HF, this information is scarcer with respect to patients with acute HF. despite available data in the literature on long-term prognostic factors in chronic HF, data on acute HF patients are more scarce. Objectives: To develop a predictor of unfavorable prognostic events in patients hospitalized for acute HF syndromes, and to characterize a group at higher risk regarding their clinical characteristics, treatment and outcomes. Methods: cohort study of 600 patients admitted for acute HF, defined according to the European Society of Cardiology criteria. Primary endpoint for score derivation was defined as all-cause mortality and / or rehospitalization for HF at 12 months. For score validation, the following endpoints were used: all-cause mortality and / or readmission for HF at 6, 12 and 24 months. The exclusion criteria were: high output HF; patients with acute myocardial infraction, acute myocarditis, infectious endocarditis, pulmonary infection, pulmonary artery hypertension and severe mitral stenosis. Results: 505 patients were included, and prognostic predicting factors at 12 months were identified. One or two points were assigned according to the odds ratio (OR) obtained (p < 0.05). After the total score value was determined, a 4-point cut-off was determined for each ROC curve at 12 months. Two groups were formed according to the number of points, group A < 4 points, and group B = 4 points. Group B was composed of older patients, with higher number of comorbidities and predictors of the combined endpoint at 6, 12 and 24 months, as linearly represented in the survival curves (Log rank). Conclusions: This risk score enabled the identification of a group with worse prognosis at 12 months. Resumo Fundamento: A insuficiência cardíaca (IC) é uma síndrome de elevada prevalência. Apesar de existir na literatura informação relativa aos fatores prognósticos a longo prazo na IC crônica, esta é mais escassa no que diz respeito aos pacientes com IC aguda. Objetivos: Desenvolver um score preditor de eventos prognósticos desfavoráveis em doentes admitidos com síndromes de IC aguda e caracterizar um grupo de maior risco quanto às suas características clínicas, terapêutica e resultados. Métodos: Estudo de coorte de 600 doentes internados com IC aguda, definida de acordo com os critérios da Sociedade Europeia de Cardiologia. O endpoint primário para a derivação do score foi definido como mortalidade de qualquer causa e/ou reinternação por IC aos 12 meses. Para a validação do score, foram utilizados como endpoints: mortalidade de qualquer causa e/ou reinternação por IC aos 6, 12 e 24 meses. Os critérios de exclusão foram: IC de alto débito, pacientes com infarto agudo do miocárdio, miocardite aguda, endocardite infeciosa, infeção pulmonar, hipertensão arterial pulmonar e estenose mitral grave. Resultados: Foram incluídos 505 doentes e identificados preditores prognósticos aos 12 meses. Atribuíram-se 1 ou 2 pontos (p.) de acordo com os odds ratio (OR) obtidos (p < 0,05). Após a determinação do valor de score total, foi estabelecido um cut-off de 4 pontos por curva ROC. Constituíram-se 2 grupos de acordo com a pontuação, grupo A < 4 p. versus grupo B = 4 p. O grupo B era constituído por idosos, com maior número de comorbidades e preditor de endpoint combinado aos 6, 12 e 24 meses traduzido linearmente nas curvas de sobrevida (Log rank). Conclusões: Este score de risco permitiu identificar um grupo com pior prognóstico aos 12 meses.

Introduction: Pulmonary angiography by computed tomography (CT) is the method of choice for the detection of acute pulmonary embolism (PE). Studies have shown that the severity of PE can be estimated by clot burden scores. Objective: To evaluate the correlation between an angiographic clot burden score (Qanadli score–QS) and parameters of right ventricular dysfunction (RVD) in patients admitted for PE. Methods: We performed a retrospective study of 107 patients (60% female) admitted to an intensive care unit for PE (intermediate/high risk) between January 1, 2007 and September 30, 2011. Images from 16-slice multidetector CT angiography were reviewed in 102 patients and the QS calculated. Based on a cut-off of 18 points established by ROC curve analysis, two groups were formed (A 18 was an independent predictor of RVD (RV/LV ratio >1) (OR: 10.85; p 18 points proved to be an independent predictor of RVD in PE, and correlated linearly with variables associated with higher morbidity and mortality. Resumo: Introdução: A angio-TC pulmonar é o método de escolha para o diagnóstico de tromboembolismo pulmonar (TEP). Estudos têm demonstrado que a gravidade do TEP poderá ser estimada com sistemas de quantificação de carga embólica. Objetivo: Avaliar a correlação entre um score de carga embólica angiográfica (Qanadli score – QS), com os parâmetros de disfunção ventricular direita (DVD), em pacientes com TEP. Métodos: Estudo retrospetivo de 107 pacientes (feminino – 60%), admitidos por TEP (intermédio/elevado risco) numa UCIC (1/1/2007-30/9/2011). Revistas as imagens de angio-TC de 102 pacientes (TCMD-16C) e quantificado o QS. Estabelecido cut-off de 18 pontos por curva ROC. Constituídos 2 grupos (G) (A 18 preditor independente de DVD (VD/VE> 1) (OR:10,85;p 18 pontos revelou-se preditor independente de DVD no TEP, correlacionando-se linearmente com multivariáveis associadas a morbi/mortalidade mais elevada. Keywords: Pulmonary embolism, Clot burden score, Right ventricular dysfunction, Palavras-chave: Tromboembolismo pulmonar, Score de carga embólica, Disfunção ventricular direita

Resumo: Introdução: A angio-TC pulmonar é o método de escolha para o diagnóstico de tromboembolismo pulmonar (TEP). Estudos têm demonstrado que a gravidade do TEP poderá ser estimada com sistemas de quantificação de carga embólica. Objetivo: Avaliar a correlação entre um score de carga embólica angiográfica (Qanadli score – QS), com os parâmetros de disfunção ventricular direita (DVD), em pacientes com TEP. Métodos: Estudo retrospetivo de 107 pacientes (feminino – 60%), admitidos por TEP (intermédio/elevado risco) numa UCIC (1/1/2007-30/9/2011). Revistas as imagens de angio-TC de 102 pacientes (TCMD-16C) e quantificado o QS. Estabelecido cut-off de 18 pontos por curva ROC. Constituídos 2 grupos (G) (A 18 preditor independente de DVD (VD/VE > 1) (OR:10,85; p 18 pontos revelou-se preditor independente de DVD no TEP, correlacionando-se linearmente com multivariáveis associadas a morbi/mortalidade mais elevada. Abstract: Introduction: Pulmonary angiography by computed tomography (CT) is the method of choice for the detection of acute pulmonary embolism (PE). Studies have shown that the severity of PE can be estimated by clot burden scores. Objective: To evaluate the correlation between an angiographic clot burden score (Qanadli score - QS) and parameters of right ventricular dysfunction (RVD) in patients admitted for PE. Methods: We performed a retrospective study of 107 patients (60% female) admitted to an intensive care unit for PE (intermediate/high risk) between January 1, 2007 and September 30, 2011. Images from 16-slice multidetector CT angiography were reviewed in 102 patients and the QS calculated. Based on a cut-off of 18 points established by ROC curve analysis, two groups were formed (A 18 was an independent predictor of RVD (RV/LV ratio > 1) (OR: 10.85; p 18 points proved to be an independent predictor of RVD in PE, and correlated linearly with variables associated with higher morbidity and mortality. Palavras-chave: Tromboembolismo pulmonar, Score de carga embólica, Disfunção ventricular direita, Keywords: Pulmonary embolism, Clot burden score, Right ventricular dysfunction

Aims Brugada syndrome (BrS) is a life-threatening arrhythmia disorder associated with autosomal-dominant mutations in the SCN5A gene. We aimed to characterize the diagnostic challenges and clinical manifestations of a novel SCN5A mutation associated with BrS. Methods and results From a novel SCN5A mutation (c.664C>T; p.Arg222X) identified in a proband with the characteristic electrocardiographic pattern and the history of sudden collapse, 122 family members were studied including 40 carriers of the mutation. The electrocardiographic diagnosis of BrS requires type 1 Brugada electrocardiogram (ECG) pattern in >1 right precordial lead (V1–V3), but recently an isolated lead with coved-type ECG was proposed to be enough for the diagnosis. In this family, these proposed criteria (PC) were more sensitive in detecting mutation carriers than the conventional criteria without repercussion on the specificity. Carriers had, on average, longer P-wave duration, PR, and QRS intervals and higher transmural dispersion of repolarization. The prevalence of late potentials was higher in carriers, and individual signal average ECG (SAECG) parameters (QRSf, LAS, and RMS40) also were related to SCN5A gene mutation. Three non-carriers were found to be affected by BrS, two with a spontaneous type 1 ECG with alternative placement of the precordial electrodes, and one only after the pharmacological provocative test, suggesting that other genes may play a role in the pathophysiology of this disease. Conclusion The PC for BrS diagnosis should be implemented. Some parameters from the spontaneous ECG and the SAECG are more effective tools than the characteristic repolarization pattern to discriminate between carriers of SCN5A mutations.

Resumo: O movimento anterior sistólico (SAM) é uma complicação pós cirúrgica da valvuloplastia mitral, sendo a sua incidência de 5-10%. O reconhecimento precoce dos sinais e sintomas de SAM é imperativo no delinear de estratégia terapêutica nesses pacientes. Este artigo foca os principais mecanismos fisiopatológicos do SAM dinâmico e modalidades de tratamento descritas na literatura. Os autores descrevem um caso clínico e as imagens ecocardiográficas captadas ilustrando a relevância clínica do mecanismo envolvido, na tentativa de esclarecer uma questão suscitada: reintervenção cirúrgica necessária? Abstract: Systolic anterior motion (SAM) is a postoperative complication of mitral valve repair, with an incidence of 5 to 10%. Early recognition of the signs and symptoms of SAM is essential for the management of these patients. This article focuses on the pathophysiology and dynamics of SAM and the treatment strategies described in the literature. The authors present a case study and echocardiographic images illustrating the clinical relevance of the mechanism involved, in order to clarify whether surgical reintervention is necessary. Palavras-chave: Movimento anterior sistólico, Valvuloplastia mitral, Fisiopatologia, Keywords: Systolic anterior motion, Mitral valve repair, Pathophysiology

Resumo: A doença de Fabry é causada por acumulação intracelular de glicoesfingolipidos em vários tecidos, secundária a mutações no gene GLA (Xq22).Classicamente descrita como afectando homens hemizigotos sem actividade residual de alfa-galactosidase-A, sabe-se hoje afectar ambos os sexos com manifestações mais tardias e frustres no feminino. Associa-se a sintomas neurológicos, cutâneos (angioqueratomas), renais, cardiovasculares (hipertrofia ventricular esquerda, alterações do ritmo e espessamento valvular), cocleo-vestibulares e cerebrovasculares.Na ausência de tratamento ocorre dano progressivo de órgãos vitais: doença renal terminal e complicações cardiovasculares e cerebrovasculares potencialmente fatais, implicando redução da qualidade e esperança de vida.Descreve-se o caso de doente do sexo feminino, com antecedentes de acidente vascular cerebral (AVC) isquémico criptogênico com 38 anos e insuficiência renal crónica (IRC) com proteinúria, que se apresenta no serviço de urgência com fibrilhação auricular. O estudo ecocardiográfico revelou hipertrofia concêntrica do ventrículo esquerdo com disfunção diastólica e diminuição do strain longitudinal basal. Englobada num protocolo, de rastreio, foi-lhe diagnosticada doença de Fabry e identificada uma mutação não descrita previamente. Abstract: Fabry disease is caused by intracellular accumulation of glycosphingolipids in various tissues, secondary to mutations in the GLA gene (Xq22).Classically described as affecting hemizygous males with no residual alpha-galactosidase A activity, it is now known to affect both sexes, with later and less severe manifestations in females. The manifestations of this disease are systemic: neurological, cutaneous (angiokeratomas), renal, cardiovascular (left ventricular hypertrophy, valve thickening or rhythm disturbances), cochlear-vestibular, and cerebrovascular. In the absence of treatment there is progressive damage to vital organs with renal failure, stroke, heart failure or rhythm perturbations, leading to severe impairment of quality of life as well as reduced life expectancy.We describe the case of a female patient with a history of cryptogenic ischemic stroke at the age of 38 years and chronic renal failure with proteinuria, who presented to the emergency room with atrial fibrillation. The echocardiogram revealed concentric left ventricular hypertrophy, diastolic dysfunction and decreased longitudinal strain in the basal septum.In the context of a screening protocol, she was diagnosed with Fabry disease and a previously undescribed mutation was identified. Palavras-chave: Doença de Fabry, Miocardiopatias, Hipertrofia ventricular esquerda, 2D Strain, Genética, Keywords: Fabry disease, Cardiomyopathies, Left ventricular hypertrophy, Speckle tracking, Genetics

Fabry disease is caused by intracellular accumulation of glycosphingolipids in various tissues, secondary to mutations in the GLA gene (Xq22).Classically described as affecting hemizygous males with no residual alpha-galactosidase A activity, it is now known to affect both sexes, with later and less severe manifestations in females. The manifestations of this disease are systemic: neurological, cutaneous (angiokeratomas), renal, cardiovascular (left ventricular hypertrophy, valve thickening or rhythm disturbances), cochlear-vestibular, and cerebrovascular. In the absence of treatment there is progressive damage to vital organs with renal failure, stroke, heart failure or rhythm perturbations, leading to severe impairment of quality of life as well as reduced life expectancy.We describe the case of a female patient with a history of cryptogenic ischemic stroke at the age of 38 years, and chronic renal failure with proteinuria, who presented to the emergency room with atrial fibrillation. The echocardiogram revealed concentric left ventricular hypertrophy, diastolic dysfunction and decreased longitudinal strain in the basal septum.In the context of a screening protocol, she was diagnosed with Fabry disease and a previously undescribed mutation was identified. Resumo: A doença de Fabry é causada por acumulação intracelular de glicoesfingolipidos em vários tecidos, secundária a mutações no gene GLA (Xq22).Classicamente descrita como afectando homens hemizigotos sem actividade residual de alfa-galactosidase-A, sabe-se hoje afectar ambos os sexos com manifestações mais tardias e frustres no feminino. Associa-se a sintomas neurológicos, cutâneos (angioqueratomas), renais, cardiovasculares (hipertrofia ventricular esquerda, alterações do ritmo e espessamento valvular), cocleo-vestibulares, e cerebrovasculares.Na ausência de tratamento ocorre dano progressivo de órgãos vitais: doença renal terminal e complicações cardiovasculares e cerebrovasculares potencialmente fatais, implicando redução da qualidade e esperança de vida.Descreve-se o caso de doente do sexo feminino, com antecedentes de acidente vascular cerebral (AVC) isquémico criptogênico com 38 anos e insuficiência renal crónica (IRC) com proteinúria, que se apresenta no serviço de urgência com fibrilhação auricular. O estudo ecocardiográfico revelou hipertrofia concêntrica do ventrículo esquerdo com disfunção diastólica e diminuição do strain longitudinal basal. Englobada num protocolo, de rastreio, foi-lhe diagnosticada doença de Fabry e identificada uma mutação não descrita previamente. Keywords: Fabry disease, Cardiomyopathies, Left ventricular hypertrophy, Speckle tracking, Genetics, Palavras-chave: Doença de Fabry, Miocardiopatias, Hipertrofia ventricular esquerda, 2D Strain, Genética

Aims: Stratifying risk of sudden death is a major issue in the management of hypertrophic cardiomyopathy (HCM). Existing risk factors have low positive predictive value and new parameters are needed. Determination of myocardial deformation (strain) by 2D Speckle tracking is a new methodology for determining LV regional function and could correlate with myocite disarray and fibrosis. The aim of this study was to assess the relationship between strain analysis and nonsustained ventricular tachycardia (NSVT) in patients with HCM. Methods: Thirty-two consecutive patients with HCM (mean age 55, 17–78) were studied. All underwent standard echocardiographic and two-dimensional strain examination. Twenty-four-hour Holter monitoring was performed and echocardiographic parameters were correlated with NSVT. Results: Nine patients (28%) had one or more episodes of NSVT. Patients with NSVT had a higher value of maximal LV thickness (23.6 mm vs. 19.4 mm, P = 0.027). There were no significant associations between NSVT on Holter monitoring and LV outflow gradient left atrial diameter, E/Em or left ventricle ejection fraction. Patients with HCM and NSVT had significant reductions in mid septal, apical-septal, apical-lateral strain, and in mean longitudinal strain. Midseptal strain >–10.5% had a sensitivity of 89% and a specificity of 74% (area under the curve, 0.787; P < 0.0013) for predicting NSVT independently of age or maximum wall thickness. Conclusion: Lower end-systolic peak longitudinal strain obtained by 2D speckle tracking was a predictor of NSVT in HCM patients. This parameter could become a useful tool in stratifying SCD risk in this population. (Echocardiography 2011;28:709-714)

Os autores apresentam um caso de síndrome de Twiddler, uma complicação rara após implantação de pacemaker definitivo, primeiramente descrito em 1968, complementado com uma abordagem à etiologia e manifestações desta entidade.Palavras-chave: Síndrome de Twiddler; Pacemaker/efeitos adversos; Falha de Equipamento.

info:eu-repo/semantics/publishedVersion

Arrhythmogenic right ventricular cardiomyopathy, also known as arrhythmogenic right ventricular dysplasia, is a condition in which myocardium is replaced by fibrous or fibrofatty tissue, predominantly in the right ventricle. It is clinically characterized by potentially lethal ventricular arrhythmias, and is a leading cause of sudden cardiac death. Its prevalence is not known exactly but is estimated at approximately 1:5000 in the adult population. Diagnosis can be on the basis of structural and functional alterations of the right ventricle, electrocardiographic abnormalities (including depolarization and repolarization alterations and ventricular arrhythmias) and family history. Diagnostic criteria facilitate the recognition and interpretation of non-specific clinical features of this disease.The authors present a case in which the diagnosis of arrhythmogenic right ventricular cardiomyopathy was prompted by the suspicion of right ventricular disease on transthoracic echocardiography. This was confirmed by detection of epsilon waves on analysis of the ECG, which generally go unnoticed but in this case were the key to the diagnosis. Their presence was also shown by non-conventional ECG techniques such as modified Fontaine ECG.The course of the disease culminated in the occurrence of ventricular tachycardia, which prompted placement of an implantable cardioverter-defibrillator. Resumo: A miocardiopatia arritmogénica do ventrículo direito – também denominada de displasia arritmogénica do ventrículo direito – é uma patologia em que se verifica a substituição do miocárdio por tecido fibroso ou fibroadiposo predominantemente no ventrículo direito e que se caracteriza clinicamente por arritmias ventriculares potencialmente letais, sendo uma das causas mais relevantes de morte súbita cardíaca. A sua prevalência exata é desconhecida, no entanto, estima-se que seja de cerca de 1: 5.000 na população adulta. O diagnóstico pode ser efetuado mediante a constatação de alterações estruturais e funcionais do ventrículo direito, alterações eletrocardiográficas – da condução em ECG basal, arritmias ventriculares – e da história familiar. A existência de critérios de diagnóstico facilita o reconhecimento e interpretação das características clínicas não específicas desta entidade.Apresenta-se um caso clínico em que o diagnóstico de miocardiopatia arritmogénica do ventrículo direito foi desencadeado a partir da suspeita de patologia ventricular direita suscitada por ecocardiograma transtorácico. A análise do ECG serviu para o confirmar, tendo-se detetado ondas épsilon que em geral passam despercebidas, mas que neste caso foram a chave para o diagnóstico tendo-se também aferido a sua existência em técnicas de ECG não convencional como o ECG modificado de Fontaine.A evolução subsequente do quadro clínico culminou com a ocorrência de taquicardia ventricular o que motivou a implantação de cardiodesfibrilhador implantável (CDI). Keywords: Arrhythmogenic right ventricular cardiomyopathy, Modified Fontaine ECG, Epsilon waves, Palavras-chave: Miocardiopatia arritmogénica do ventrículo direito, Eletrocardiograma modificado de Fontaine, Ondas épsilon

Resumo: Introdução: Atualmente, o diagnóstico da Síndrome de Brugada (SB) obriga à documentação do padrão eletrocardiográfico de repolarização característico denominado tipo 1. Em 38% dos casos familiares desta entidade encontra-se uma mutação do gene SCN5A responsável pela síntese do canal de sódio NaV1.5. A disfunção desta corrente de sódio repercute-se no potencial de ação cardíaco de resposta rápida, fundamentalmente nos miócitos auriculares, nas fibras de Purkinje e nos miócitos ventriculares. Objetivos: Detetar portadores de mutação do SCN5A sem recorrer ao padrão de repolarização no ECG. Métodos: A partir de 3 famílias e de um total de 141 elementos, dos quais 55 portadores (P+) de duas mutações non-sense específicas do SCN5A causadoras de SB, foram estudados os maiores de 16 anos (113 elementos/42 P+). Foi medido no ECG o intervalo PR (PR) em DII e dispersão QT (dQT) entre V1 e V3. No ECG de alta resolução (ECGAR) determinado o QRS filtrado (QRSf), root-mean square (RMS40) e low-averaged signal (LAS). Para detetar P+ foi criado: 1) um teste de rastreio (TesteRas) constituído pelo intervalo PS (PR+QRSf) ≥ 250 (250 ms corresponde a 80% do valor teórico máximo deste intervalo em saudáveis) e 2) um teste diagnóstico (TesteDx) que resulta do cumprimento simultâneo de 4 condições: PS ≥ 250 e dQT > 10 e LAS ≥ 26 e RMS40 ≤ 29 (estes 2 últimos valores correspondem aproximadamente a 70% do valor teórico máximo em saudáveis). Resultados: Encontradas diferenças significativas entre os P+ versus não portadores (P-) do PR, da dQT, do QRSf, do LAS e da RMS40. O gene está associado a todas estas variáveis, sendo a associação mais forte com o PR. A 63 elementos (38 P+) foi aplicado o TesteRas e o TesteDx. O TesteRas foi positivo em 38 de 38 P+ com 8 falsos positivos em 27 P- (sensibilidade [S] =100% e especificidade [E] =66,67%). Na curva Roc para este teste o cut-off PS=252,5 tem S=100% e E=76% e o cut-off PS=260 tem S=94,7% e E=84%. O TesteDx foi positivo em 36 de 38 P+ com 3 falsos positivos: S=94,74% e E=88,89%. Por modelo logístico multivariado identifica-se um cut-off através da curva Roc com S=92% e E=92%. A S e E de diagnóstico do ECG espontâneo usando o padrão de repolarização tipo 1 no mesmo grupo de doentes foram de 52,4% e 97,2%, respetivamente (a diferença de S entre o TesteDx e o padrão de repolarização no ECG é estatisticamente significativa). Conclusões: O TesteRas e o TesteDx são uma ferramenta mais eficaz do que o padrão de repolarização típico para distinguir entre P+ e P- destas duas mutações non-sense do SCN5A. Sugerimos a sua utilização em familiares diretos de casos índice com SB, quando o resultado genético (ainda) não está disponível e num score de probabilidade de doença em indivíduos com ECG de Brugada idiopático ou em indivíduos com sintomas relacionados com arritmias e padrão de repolarização tipo 2 ou 3 de Brugada. Abstract: Introduction: Diagnosis of Brugada syndrome (BS) currently requires documentation of a characteristic repolarization pattern (type 1 Brugada ECG). Mutations in the SCN5A gene, which codes for sodium channel NaV 1.5, are found in 38% of familial cases of BS. Sodium current dysfunction negatively affects the cardiac fast response action potential, particularly in atrial and ventricular myocytes and in the fast-conducting Purkinje system. Objectives: To detect carriers of SCN5A mutations without using the characteristic repolarization pattern (type 1 Brugada ECG). Methods: Of a total of 141 members of three different families including 55 carriers of two nonsense SCN5A mutations causing BS, all those aged over 16 (113 individuals, 42 carriers) were studied. The PR interval (PR) and QT dispersion (QTd) between leads V1 and V3 were measured on conventional ECG. Using signal-averaged ECG the total duration of the filtered QRS complex (fQRS), the root-mean-square (RMS40) and the low-amplitude signal (LAS) were measured. The following procedures were developed to detect carriers/To detect carriers the following procedures were developed: (1) a screening test (ScreenTest) with PS (PR+fQRS) ≥250 (250 ms is 80% of the theoretical maximum in healthy individuals); and (2) a diagnostic test (DiagTest) for the simultaneous fulfillment of four conditions: PS ≥250 and QTd ≥10 and LAS >26 and RMS40 ≤29 (the latter two cut-offs are approximately 70% of the theoretical maximum in healthy carriers). Results: Significant differences in PR, QTd, QRSf, RMS40 and LAS were found between carriers and non-carriers. The SCN5A gene was associated with all these variables, the strongest association being with PR. Both tests were applied to 63 family members (38 carriers). The ScreenTest was positive in 38 of 38 carriers, with eight false positives in 27 non-carriers (sensitivity [SE]=100% and specificity [SP]=66.67%). From ROC curve analysis a cut-off of PS=252.5 shows SE=100% and SP=76% and a cut-off of PS=260 shows SE=94.7% and SP=84%. The DiagTest was positive in 36 of 38 carriers, with three false positives: SE=94.74% and SP=88.89%. From ROC curve analysis a multivariate logistic model identifies a cut-off with SE=92% and SP=92%. In the same group the SE and SP of the characteristic spontaneous repolarization pattern (type 1 Brugada ECG) to detect carriers were 52.4% and 97.2%, respectively, and the difference between the SE of the DiagTest and of the typical repolarization pattern is statistically significant. Conclusions: The ScreenTest and DiagTest are more effective tools than the characteristic repolarization pattern to discriminate between carriers and non-carriers of these two nonsense SCN5A mutations. We suggest their use in first-degree relatives of Brugada patients when the results of genetic testing are not available, in a score of disease probability in individuals with idiopathic Brugada ECG, and in patients with arrhythmias or other Brugada-related symptoms presenting type 2 or type 3 Brugada ECG. Palavras-chave: Síndrome de Brugada, Score diagnóstico, SCN5A, Keywords: Brugada syndrome, Diagnostic score, SCN5A gene

Systolic anterior motion (SAM) is a postoperative complication of mitral valve repair, with an incidence of 5–10%. Early recognition of the signs and symptoms of SAM is essential for the management of these patients. This article focuses on the pathophysiology and dynamics of SAM and the treatment strategies described in the literature. The authors present a case study and echocardiographic images illustrating the clinical relevance of the mechanism involved, in order to clarify whether surgical reintervention is necessary. Resumo: O movimento anterior sistólico (SAM) é uma complicação pós cirúrgica da valvuloplastia mitral, sendo a sua incidência de 5-10%. O reconhecimento precoce dos sinais e sintomas de SAM é imperativo no delinear de estratégia terapêutica nesses pacientes. Este artigo foca os principais mecanismos fisiopatológicos do SAM dinâmico e modalidades de tratamento descritas na literatura. Os autores descrevem um caso clínico e as imagens ecocardiográficas captadas ilustrando a relevância clínica do mecanismo envolvido, na tentativa de esclarecer uma questão suscitada: reintervenção cirúrgica necessária? Keywords: Systolic anterior motion, Mitral valve repair, Pathophysiology, Palavras-chave: Movimento anterior sistólico, Valvuloplastia mitral, Fisiopatologia

Reproducibility of the signal-averaged ECG in a family screened for Brugada syndrome {#article-title-2} Introduction This study represents a methodological evaluation of reproducibility of the Signal Averaged-ECG (SAECG) in Brugada syndrome (BS), and is a starting point for a project that aims to evaluate the usefulness of this technique in this particular clinical context. Methods Thirty-six subjects (55.6% women) of the same family, without structural heart disease, mean age 33.86 + 11.35 years, referred for screening of BS, underwent SAECG (with time domain analysis) on two occasions (6-month interval). Late potentials were defined when at least two of three criteria were found: fQRS >114 ms; RMS40 38 ms. All participants were genetically screened and 20 (55.6%) had the mutation (G+). Results The reliability of the SAECG was very good ( k = 0.88, P < 0.001), with an ICC = 0.89 ( P < 0.001). Fourteen subjects had positive SAECG (10 were G +), two of which changed diagnosis from one to the other time. Strong correlations were found for fQRS, RMS40, and LAD ( R = 0.79, 0.78, and 0.81, respectively). The RMS40 and LAS parameters were the most consistent in both moments ( k = 0.77 and 0.66, respectively, P < 0.001). Good indicators of reproducibility were also extracted in the Bland–Altman analysis. The mean discrepancy was 0.42 + 7.79 ms, −2.97 + 11.63, and 1.56 + 8.80 mV, respectively, for fQRS, RMS40, and LAS, without significant differences between G+ and G−. Conclusion Our data illustrate a high reproducibility of the SAECG, documented by excellent correlations and small discrepancies in the estimated parameters, particularly in patients with confirmed BS. # Cardiac event rate in Brugada patients {#article-title-3} Background ICD implantation can prevent sudden cardiac death (SCD) in Brugada patients. However, selecting patients to receive an ICD for primary prevention is challenging. ICD implantation also carries some risks, including inappropriate shock. The aims of this study were: Method Retrospective analysis from the registry of 29 ICD implantations for Brugada patients over the past 10 years in IJN, categorized into 3 groups of patients: 1. Asymptomatic (n = 6), 2. Syncope (n = 11), and 3. Those with documented VT/VF or survivors of resuscitated SCD (n = 12). Results During mean follow up time 53 months (min: 4 months, max: 161 months), none of group 1 developed cardiac event, 1 patient in group 2 (9%) developed VF and got appropriate shock, 10 patients in the documented VT/VF or resuscitated group (83.3%) had VF and appropriate shock. 1 patient in the asymptomatic group had inappropriate shock. Conclusion Although the number of patients is small (but with good duration of follow up), this study demonstrated that the risk of SCD is low in asymptomatic Brugada patients. This will help physicians to stratify Brugada patients better. # Value of C-reactive protein in patients with asymptomatic and symptomatic Brugada syndrome {#article-title-4} Background Brugada syndrome (BrS) is an arrhythmogenic disease with potential lethal outcomes. Inflammation in BrS and its clinical implication are unknown. We assessed the relationship between inflammation and arrhythmic events in BrS. Methods and results Fifty-four patients with BrS underwent work up. All had blood samples drawn for C-reactive protein levels at admission, prior to any invasive intervention. The decision to place an ICD was based on standard guidelines and was independent of C-reactive protein concentrations. Patients with conditions associated with increase of C-reactive protein levels were excluded. We divided our sample in two groups: asymptomatic and symptomatic (syncope or aborted sudden death). In a multivariable analysis, we adjusted for significant variables (age, history of VT, C-reactive protein, 2 mg/L). The mean age was 45 ± 13 years, and 91% was male. Twenty (37%) were symptomatic (17 syncope and 3 aborted SCD). Baseline characteristics were similar in both groups. Mean C-reactive protein level was 1.4 ± 0.9 mg/L in asymptomatic group and 2.4 ± 1.4 mg/L in symptomatic group ( P = 0.003). We hypothesized that C-reactive protein 0 ≥ 2 mg/L may discriminate individuals with and without symptoms. This hypothesis was confirmed by the finding that symptoms were more frequent in the subgroup with C-reactive protein 0 ≥ 2 mg/L ( P = 0.039, 95% CI: 1.07–18.79). In the multivariate model, C-reactive protein concentrations 0 ≥ 2 mg/L remained an independent marker for being symptomatic ( P = 0.018; 95% CI: 1.3–19.3). Conclusion C-reactive protein concentrations ≥2 mg/L appears to be associated with arrhythmic events in patients with BrS and can be considered as a new marker for risk stratification. # A 10-year single-centre prospective study on asymptomatic patients with spontaneous Cove type (Type I) Brugada ECG pattern {#article-title-5} Background Brugada syndrome is a genetic disease characterized by distinct electrocardiogram (ST-segment elevation in leads V1 through V3) finding with an increase in risk of sudden cardiac death. Various risk factors such as syncope, specific ECG pattern, and positive electrophysiology had been identified to predict further VF episodes. Although ICD is a well-proven effective measure for preventing sudden cardiac death in Brugada syndrome, it is often a difficult decision for physician on who is required and benefit. Objective To acquire demographic data, clinical characteristic, follow-up data and clinical outcome of asymptomatic patients with spontaneous Cove type (type I) Brugada ECG pattern. Methods This is a prospective single-centre study. Asymptomatic (without history of syncope, not sudden cardiac death survivor) patient with a coved-type Brugada ECG pattern is recruited since 1999. Demographic data, clinical characteristic, ECG data, investigation, treatment, and clinical outcome were collected. Results There are total 33 patients (31 males and 2 females) recruited since August 1999 to June 2010. The average age is 54.9 years old at diagnosis. Nineteen patients had electrophysiology test done; of which, 7 showed inducible VF. Four patients had ICD implanted eventually. During a total of 178 patient-years follow-up time, there are no documented VF episodes or confirmed sudden cardiac death. Three patients died because of unrelated disease. Conclusion Asymptomatic patients (without history of syncope) with spontaneously Cove type (Type I) Brugada ECG appears to be low risk subgroups in our cohort study. # Follow-up of five patients with Brugada syndrome treated with ICD {#article-title-6} Objective To investigate clinical symptoms, episodes of arrhythmias and its therapy in patients with Brugada syndrome treated with implantable cardioverter-defibrillator (ICD). Methods Five patients with concealed Brugada syndrome (all male, mean age 41.6 ± 10.14 years) were treated with single-chamber ICD and followed up every 3 months. The time of onset, type of arrhythmia, treatments, and its results of the episodes were investigated according to the datalogs of the ICD. Results The diagnosis of Brugada syndrome was made according to sodium-channel blocker provocation test in four patients (two by ajmaline, two by propafenone), and screen of new precordial leads system in another case. Episodes of syncope in all patients and ventricular fibrillation (Vf) in four cases were documented before ICD therapy. During electrophysiological study, ventricular fibrillation could be induced in three patients. During a follow-up of 22 ± 18 months, 75 episodes of Vf were documented, 61 were terminated by 86 shocks successfully, 14 stopped spontaneously. One patient still experienced four episodes of syncope because of his increased defibrillation threshold. One patient had 26 times inappropriate shocks due to atrial fibrillation, which disappeared after we adjusted the protocol of the ICD. Another one had two episodes of syncope though no event was recorded in his ICD. Because the tilt test reached a positive result, the diagnosis of vasovagal syncope was made. Conclusion ICD implantation is a necessary and effective therapy for high-risk patients with Brugada syndrome, and should be followed up regularly and programmed appropriately because of the increased defibrillation threshold or inappropriate shocks.

Is there a signal-averaged ECG phenotype in patients with Brugada syndrome? {#article-title-2} Background and objectives Electrocardiographic manifestations of Brugada syndrome (BS) have been attributed to one of the two basic mechanisms: conduction delay and premature repolarization. The possible contribution of signal-averaged ECG (SAECG) to BS diagnosis is indefinite, and this study tests the hypothesis that these patients have a distinct SAECG phenotype. Methods Seventy-two adults were included, 52 from the same family with Brugada [25 with positive genetic test (G+) and 27 with negative genetic test (G−)]. Twenty unrelated healthy individuals were the control group (CG). Late potentials (LPs) were defined when at least two of the following were present: (i) filtered QRS duration (fQRS) >114ms; (ii) root-mean square voltage of terminal 40 ms of QRS (RMS40) 38 ms. Results The prevalence of LPs was 15, 26, and 61%, respectively, in CG, G− , and G+ group ( P < 0.05). G+ presented a higher percentage of patients with abnormal parameters, particularly RMS40 (61.5 vs. 26.9% and 20%, respectively, in G− and CG, P = 0.005) and LAS (69.2 vs. 26% and 15%, respectively, in G− and CG, P < 0.001). Conclusion Brugada patients appear to have an SAECG characteristic phenotype, defined by fragmented activity within the terminal portion of the QRS (essentially LAS and RMS40) without a major change in its length (fQRS). # Critical role of microRNA miR-26 in atrial electrical remodelling in experimental atrial fibrillation {#article-title-3} Background Increase in inward rectifier K+ current I K1 is a hallmark of atrial fibrillation (AF)-related electrical remodelling. However, the underlying mechanism remains largely unknown. Recent studies have highlighted the important roles of microRNAs (miRNAs) in various heart diseases. Hence, this study aims to investigate the potential roles of miRNAs in AF by targeting ion channels, specifically I K1. Methods The canine AF model was induced by atrial tachypacing; the miRNA expression level was determined by Q-PCR; the effect of miR-26 on KCNJ2 was examined by luciferase assay and western blot; I K1 currents were recorded by whole cell patch clamp; adenovirus carrying miR-26 was constructed to in vivo over express miR-26; and endogenous miR-26 was knocked down by LNA knockdown oligo. Results We first found miR-26 being significantly down-regulated by >50% in AF. We subsequently verified KCNJ2 encoding Kir2.1 protein for I K1 as a cognate target for miR-26. Transfection of miR-26 reduced the Kir2.1 protein level, whereas knocking down miR-26 by its antisense oligomers (AMO-26) caused a robust increase in the Kir2.1 level. Accordingly, miR-26 depressed whilst AMO-26 enhanced, I K1 current density. We further characterized the core promoter regions of miR-26 genes and identified NFAT, a well-implicated transcription factor in AF, as a transcriptional repressor of miR-26 genes. More interestingly, application of in vivo knockdown oligo against miR-26 to mice greatly enhanced the AF vulnerability while overexpressing miR-26 largely reduced AF inducibility. Conclusion Our study revealed that miRNAs are critically involved in AF and the associated atrial electrical remodelling and may serve as therapeutic target for AF treatment. # Left ventricular systolic and diastolic function in paediatric patients with definitive pacemaker {#article-title-4} Background Chronic right ventricular pacing is associated with deleterious effects in left ventricular form and function. Alternative sites of stimulation could avoid the cardiac remodelling and ventricular dysfunction. Objective Compare the effects of chronic right and left ventricular pacing in children with structurally normal hearts. Methods Echocardiographic data were retrospectively and prospectively obtained from patients with structurally normal hearts that required the implantation of definitive pacemakers at right ventricle epicardium ( n = 7), at right ventricle endocardium ( n = 30), and at left ventricle epicardium ( n = 18), between 1 January2000 and 31 December 2009. Fractional shortening and fractional ejection were calculated as a measure of left ventricular systolic function, Doppler mitral inflow, pulmonary vein flow, and tissue Doppler were estimated to diastolic function analysis; left ventricular end-diastolic diameter and left ventricular end-systolic diameter were also determined. Echocardiographic evaluations were made before pacemaker implantation, immediately after and regularly during a medium-term follow-up. Results Left ventricular diastolic function did not change in any site pacing during the follow-up; left ventricular fractional shortening and fractional ejection were higher in left ventricular epicardium pacing (42 ± 8, 73 ± 6) than in right ventricular endocardium paced group (36 ± 4, 66 ± 5) and right ventricular epicardium paced patients (34 ± 6, 64 ± 5). Left ventricular end-diastolic and end-systolic diameters were increased as a result of depressed systolic function in both right ventricular pacing. Conclusion Left ventricular form and function are better in paediatric patients with structurally normal hearts and chronic left ventricular pacing when compared with children with chronic right ventricular pacing. # Radiologically observed lead placement complications seen after pacemaker implantation in 526 subjects: a compelling case for leadless pacing systems {#article-title-5} Background Despite technical advances, cardiac pacing is still associated with problems attributable to poor lead placement. Lead problems may be caused by operator experience, technical performance, and logistics. Elimination of leads and the utilization of alternate energy are the only way to avoid these complications. In this QA analysis, we sought to define and quantify post-procedure lead X-ray configurations to assess the potential benefit of emerging leadless technology. Methods and results Post-procedure chest X-rays of 526 patients after pacemaker implantation were reviewed. Final atrial lead (AL) positions (433) were categorized according to quadrants in the frontal projection: A (right upper-ideal), B (right lower), C (left lower), and D (left upper). Final ventricular lead (VL) locations (526) were also analysed. Septal and epicardial leads were excluded. In 433 patients, AL were non-ideal in 29.3% (Quadrant B −10.6%, C −1.2%, D −18.7%). Atrial lead looping was recorded in 10.4% cases. Ventricular lead placements were considered suboptimal in 15%. Return to theatre for early lead revision occurred in

Doente do sexo masculino, 75 anos, com quadro arrastado de tosse. Fez Radiografia do torax que demonstra opacificacao paratraqueal direita. Fez TC Toracica ( e ) para melhor caracterizacao que mostrou a denominada Anomalia Vascular de Neuhauser com arco aortico direito e arteria lusoria, correspondendo a imagem descrita na radiografia. […] Disfagia Causada por Arco Aortico Direito e Arteria Lusoria