Duchenne muscular dystrophy (DMD), an X-linked recessive disease, is characterised by a systemic lack of dystrophin protein in the sarcolemma, affecting skeletal and cardiac muscles. Current therapies have increased life span in DMD patients, but this has led to the majority of surviving patients developing dilated cardiomyopathy. Recently, stem cell-based ...

AIMS: To estimate the degree of cardiac involvement regarding left ventricular ejection fraction, conduction abnormalities, arrhythmia, risk of sudden cardiac death (SCD) and the associations between cardiac involvement and cytosine-thymine-guanine (CTG)-repeat, neuromuscular involvement, age and gender in patients with myotonic dystrophy type 1 (MD1). METHODS AND RESULTS: A Pub-Med search ...

Background- Aortic stenosis (AS) and diabetes mellitus (DM) are frequent comorbidities in aging populations. In heart failure, DM worsens diastolic left ventricular (LV) dysfunction, thereby adversely affecting symptoms and prognosis. Effects of DM on diastolic LV function were therefore assessed in aortic stenosis, and underlying myocardial mechanisms were identified. Methods ...

Cardiac dysfunction is a frequent manifestation of Duchenne muscular dystrophy (DMD) and a common cause of death for individuals with this condition. Early diastolic dysfunction and focal fibrosis proceed to dilated cardiomyopathy (DCM), complicated by heart failure and arrhythmia in most patients. Improvements in the management of respiratory insufficiency in ...

Mutations in the LMNA gene, which encodes A-type nuclear lamins (intermediate filament proteins expressed in most differentiated somatic cells), cause a diverse range of diseases, called laminopathies, that selectively affect different tissues and organ systems. The most prevalent laminopathy is cardiomyopathy with or without different types of skeletal muscular dystrophy. ...

Muscular ventricular septal defects remain a challenge despite the progress in surgical and interventional closure of ventricular septal defects. Our hypothesis was inspired by the fact that more than two thirds of children with muscular ventricular septal defects experienced spontaneous closure. Therefore, we intend to induce the spontaneous closure of ...

We report on a 74-year-old woman who presented with embolic stroke of the brainstem and right cerebellum. She had undergone coronary bypass surgery and prophylactic ligation of the left atrial appendage in the past. On further investigations, a source of emboli was found to be an incompletely ligated left atrial ...

Cardiac involvement has been reported in carriers of dystrophin mutations giving rise to Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD). The progress of these abnormalities during long-term follow-up is unknown. We describe the long-term follow-up of dilated cardiomyopathy (DCM) in DMD/BMD carriers. A long-term follow-up study was performed ...

OBJECTIVE: The aim of the study was to identify, in addition to conduction defects, possible predictors of cardiac events and death in patients with myotonic dystrophy (DM1). METHODS AND DESIGN: A retrospective observational cohort study was undertaken. Baseline clinical and non-invasive cardiac and respiratory investigations were obtained from 107 DM1 ...

Control over cell engraftment, survival, and function remains critical for heart repair. We have established a tissue engineering platform for the delivery of human mesenchymal progenitor cells (MPCs) by a fully biological composite scaffold. Specifically, we developed a method for complete decellularization of human myocardium that leaves intact most elements ...

Young and old patients with acute myocardial infarction have different risk factor profiles, clinical presentation, angiographic findings and prognosis. In the present study we investigated the clinical profile of patients aged <46 years with acute myocardial infarction. Full English text available from: www.revespcardiol.org.

Autophagy is a conserved catabolic process for long-lived proteins and organelles and is primarily responsible for nonspecific degradation of redundant or faulty cell components. Although autophagy has been described as the cell's major adaptive strategy in response to metabolic challenges, its influence on the cell's energy profile is poorly understood. ...

Myotonic dystrophy type 1 (DM1) is the most frequent muscular dystrophy in adults. It is a multisystem disorder also affecting the heart with an increased incidence of sudden cardiac death. We present a young female patient with ventricular tachycardia (VT) who had no cardiac complaints previously. In this patient, the ...

Homeodomain only protein x (Hopx) is an unusual homeodomain protein that has diverse effects on cardiac growth. Manipulation of Hopx function in murine models is associated with cardiac hypertrophy, dilation and fibrosis. In the present study, we examined the expression profile of Hopx in various models of pathologic cardiac hypertrophy ...

Aging is associated with an increased incidence of heart failure, but the existence of an age-related cardiomyopathy remains controversial. Differences in strain, age and technique of measuring cardiac function differ between experiments, confounding the interpretation of these studies. Additionally, the structural and genetic profile at the onset of heart failure ...

Abstract Objectives: Low-level laser irradiation (LLLI) has the potential of exerting cardioprotective effect following myocardial infarction (MI). The authors hypothesized that LLLI could influence the expression of cardiac cytokines and contribute to the reversal of ventricular remodeling. Background: LLLI regulates the expression of cytokines after tissue damage. However, little is ...

Diabetic cardiac dysfunction is associated with decreased rates of myocardial glucose oxidation (GO) and increased fatty acid oxidation (FAO), a fuel shift that has been shown to sensitize the heart to ischemic insult and ventricular dysfunction. We sought to evaluate the metabolic and functional consequences of chronic suppression of GO ...

INTRODUCTION: Patients with Brugada syndrome (BS) often have spontaneous changes in their electrocardiogram (ECG). OBJECTIVE: To evaluate the significance of ECG alterations, we investigated the relationships between the ECG and the occurrence of ventricular fibrillation (VF) in both patients and an experimental model of BS. METHODS AND RESULTS: Study 1. ...

Atrial Remodeling in Doxorubicin Cardiomyopathy. Introduction: All preclinical studies of atrial remodeling in heart failure (HF) have been confined to a single model of rapid ventricular pacing. To evaluate whether the atrial changes were specific to the model or represented an end result of HF, this study aimed to characterize ...

The cardiac complications of certain neurologic diseases have been well recognized for over 50 years and are mostly evident for cerebrovascular accidents. Although these complications are frequent and in most circumstances benign, detrimental cardiac side effects, such as serious arrhythmias and myocardial infarctions, may occur. The link to most of ...

Left atrial appendage (LAA) occlusion is increasingly accepted to reduce the risk of stroke in patients with atrial arrhythmia who are unsuitable for routine anticoagulation. It is generally performed under general anesthesia, guided by transoesophageal echocardiography with accurate imaging being essential for correct deployment of the device. We present a ...

This article reviews the safety and efficacy of ibutilide for use in patients with atrial fibrillation and flutter. Ibutilide, a class III antiarrhythmic agent, is primarily used for conversion of atrial flutter and fibrillation and is a good alternative to electrical cardioversion. Ibutilide has a conversion rate of up to ...

Sinus node disease (SND) is a common clinical condition and is the most common indication for permanent pacemaker implantation. This review aims to revisit the complex sinus node anatomy, the evolving understanding of its pacemaking mechanisms, the atrial myopathy in SND and sinus node remodeling. Recent high-density noncontact mapping of ...

Two-dimensional speckle tracking echocardiography (STE) is a relatively new method to detect regional myocardial dysfunction. To assess left ventricular (LV) regional myocardial dysfunction using STE in Duchenne muscular dystrophy model dogs (CXMD(J)) without overt clinical signs of heart failure. Six affected dogs, 8 carrier dogs with CXMD(J), and 8 control ...

Limb girdle muscular dystrophies (LGMD) are inclusive of 7 autosomal dominant and 14 autosomal recessive disorders featuring progressive muscle weakness and atrophy. Studies of cardiac function have not yet been well-defined in deficiencies of dysferlin (LGMD2B) and fukutin related protein (LGMD2I). In this study of patients with these two forms ...

We report on a trauma victim without history of or risk factors for cardiac disease, who suffered coronary artery dissection caused by blunt chest injury (BCI). Myocardial ischaemia was detected by multislice computed tomography (MSCT) promptly after trauma centre admission and managed by immediate revascularisation. Thoracic trauma may cause myocardial ...

AIMS: Long-term outcome of patients after myocardial infarction (MI) largely depends on the extent of post-infarct remodelling. To explore the molecular mechanism of remodelling, comparative proteomic analysis was undertaken to identify differential myocardial proteome profiles expressed in the border zone of the post-MI heart. METHODS AND RESULTS: Two-dimensional gel electrophoresis ...

Bepridil is effective for conversion of atrial fibrillation to sinus rhythm and in the treatment of drug-refractory ventricular tachyarrhythmias. We investigated the effects of bepridil on electrophysiological properties and spiral-wave (SW) reentry in a 2-dimensional ventricular muscle layer of isolated rabbit hearts by optical mapping. Ventricular tachycardia (VT) induced in ...

Atrial remodeling is an important part of cardiac remodeling after acute myocardial infarction (AMI). The aim of this study was to evaluate the effect of spironolactone on atria in patients with preserved left ventricular (LV) functions after AMI by using two-dimensional and tissue Doppler imaging techniques (TDI). The study consisted ...

We report the use of the hybrid procedure (bilateral pulmonary artery banding, ductal stenting followed by balloon atrial septostomy) in the first week of life for the treatment of two neonates with large left ventricular fibromas resulting in univentricular physiology. At 6 months of age, second-stage palliation was accomplished and ...

Dietary n-3 polyunsaturated fatty acids (PUFA) reduce coronary heart disease (CHD) complications, such as chronic arrhythmia and sudden cardiac death. Improved myocardial resistance to ischemia-reperfusion injury results in smaller myocardial infarction, which is a major factor in the occurrence of CHD complications. We hypothesized that a specific dietary fatty acid ...

We report a patient who presented with new onset atrial fibrillation in the setting of inferior ST-segment elevation myocardial infarction (STEMI). Cardiac catheterization revealed proximal occlusion of the left circumflex artery. Balloon angioplasty and stenting restored flow to a large left atrial branch, with prompt conversion of the rhythm to ...

Current fetal cardiac intervention for restrictive atrial septum is invasive and technically demanding. We investigated the feasibility of computer-assisted high-intensity focused ultrasound (HIFU) for cardiac intervention on the atrial septum of a beating heart. To create an interatrial communication in the beating heart of nine anesthetized rabbits, the heart was ...

A 71 year-old female presented with a large left aortic sinus to right atrial fistula causing severe congestive heart failure and atrial fibrillation. Successful percutaneous closure of the defect was achieved using a 20/18 duct occluder with dramatic improvement in symptoms.

The aim of the study was to assess the relationship between P-wave duration on the surface electrocardiogram (ECG) and echocardiographic parameters of atrial electromechanical delay (EMD), as well as contraction synchrony during different atrial pacing modalities. In 57 patients with sinus node disease and prolonged sinus P-wave duration treated with ...

Peri-mitral atrial flutter, which is a type of macroreentrant atrial tachycardia circulating around the mitral annulus, was terminated with the guidance of the high-density mapping catheter. A 72-year-old woman with atrial fibrillation and atrial tachycardia presented with symptoms of congestive heart failure. The recurrent peri-mitral atrial flutter following the initial ...

We sought to confirm our hypothesis that the human sinoatrial node (SAN) is functionally insulated from the surrounding atrial myocardium except for several exit pathways that electrically bridge the nodal tissue and atrial myocardium. The site of origin and pattern of excitation within the human SAN has not been directly ...

Electrophysiological properties of the atrial endocardium compared to epicardium are not well understood. The purpose of this study was to compare the electrophysiological properties and vulnerability to arrhythmia induction from these regions. Transseptal endocardial and percutaneous epicardial mapping were performed in a porcine model (n = 7). Two opposing 4-mm ...

PEA Recorded in the Atrium. Introduction: Preliminary studies have revealed a high correlation between peak endocardial acceleration (PEA) measured with a sensor embedded in a ventricular lead and human cardiac contractility. In this study we assessed (1) the contributions made by measurements of PEA1, an index of ventricular systolic contraction, from ...

The main objective of this study was to characterize morphological differences between sinus and retrograde atrial waves. We collected intracardiac atrial signals through a DDDR pacemaker to characterize their morphologies and discriminate retrograde from sinus atrial waves off-line. Intracardiac unipolar and bipolar signals were collected at an 800-Hz sampling rate ...

Ectopic atrial tachycardia (EAT) often resists medical therapy, making radiofrequency catheter ablation (RFCA) the preferred treatment. This study reviewed the records of 35 patients who underwent electrophysiologic studies (EPS) and 39 RFCA procedures for EAT during a 10-year period. Of the 35 patients, 10 (28%) presented with decreased ventricular function ...

Achieving transmural tissue ablation might be necessary for successful treatment of atrial fibrillation. The purpose of this study was to evaluate the reproducibility of transmural left atrial ablation using a high-intensity focused ultrasound energy system in a calf model. Nine heparinized bovines underwent a beating-heart left atrial ablation with a ...

LAA Activity in Surface ECG. Introduction: Interatrial frequency gradient is used to guide catheter ablation of atrial fibrillation (AF). Lead V1 adequately reflects right atrial activity, but reliable tools for noninvasive estimation of right versus left fibrillatory frequency are lacking. In this study, patients with dissociated left and right atrial ...

The authors describe the case of a 79-year-old man with prior mitral valve repair and a maze procedure who developed recurrent atrial fibrillation, in whom transesophageal echocardiography revealed an accessory lobe of the left atrial appendage in sinus rhythm when the remaining body of the left atrial appendage was in ...

We report a case of focal atrial tachycardia (AT) originating from prior superior vena cava isolation line. The P-wave morphology in lead aVL during the AT differed from that during sinus rhythm although their foci were in close proximity to each other. We discuss the mechanism based on the activation ...

Atrial septal aneurysm (ASA) is a saccular deformity located in the atrial septum. Atrial arrhythmias are common in patients with ASA. Atrial electromechanical delay (AEMD) can be used to evaluate development of atrial arrhythmias in various settings. The aim of the study was to investigate the relationship between ASA, cardiac ...

Left atrial enlargement is an important predictor of cardiovascular events such as atrial fibrillation, stroke, heart failure and mortality. A number of methods of left atrial size assessment by echocardiography have been reported, from the simple antero-posterior diameter in the parasternal long axis view to the more complex ellipsoid, area-length ...

Infants with hypoplastic left heart syndrome and intact or highly restrictive atrial septum require emergent therapy to relieve pulmonary congestion. Transcatheter stenting has become, in most large centers, the mainstay of therapy for relief of left atrial hypertension. Normally, this procedure is performed with the assistance of transesophageal echocardiographic guidance. ...

PURPOSE: Comprehensive treatment of atrial fibrillation requires both compartmentalization of the posterior left atrium and connecting ablations to the mitral annulus to interrupt perimitral macro reentry and to isolate the arrhythmogenic left atrial substrate. We present a new procedure that compartmentalizes virtually all arrhythmogenic anatomy etiologic in atrial fibrillation, enabling ...

This study assessed the cardiac electrophysiological and hemodynamic effects of an intravenous infusion of the combined ion channel blocker AZD1305. After successful ablation of atrial flutter, patients were randomized to receive placebo (n = 12) or AZD1305 (n = 38) in 4 ascending dose groups. Electrophysiological and hemodynamic measurements were ...