Electrocardiographic Characteristics and Their Correlation with Echocardiographic Alterations in Fabry Disease.

Fabry disease (FD) is an X-linked disorder with α-galactosidase A deficiency. Males (>30 years) and females (>40 years) often present with cardiac manifestations, predominantly left ventricular hypertrophy (LVH). The aim of this study was to evaluate electrocardiographic (ECG) characteristics within FD patients to identify gender related differences, and to additionally explore the association of ECG parameters with structural and functional alterations on transthoracic echocardiography (TTE).

Basal Segmental Longitudinal Strain: A Marker of Subclinical Myocardial Involvement in Anderson-Fabry Disease.

Background: Cardiac involvement in Anderson-Fabry disease (AFD) is associated with increased left ventricular (LV) wall thickness. The aim of this study was to evaluate if two-dimensional global and regional strain in patients with AFD can identify early myocardial involvement (when LV wall thickness and function are normal). Additionally, the association of altered strain with adverse cardiovascular events was evaluated.

Principles of transthoracic echocardiographic evaluation.

Transthoracic echocardiography is the most widely used imaging test in cardiology. Although completely noninvasive, transthoracic echocardiography has a well-established role in the diagnosis of numerous cardiovascular diseases, and also provides critical qualitative and quantitative information on their prognosis and pathophysiological processes. The aim of this Review is to outline the broad principles of transthoracic echocardiography, including the traditional techniques of two-dimensional, colour, and spectral Doppler echocardiography, and newly developed advances including tissue Doppler, myocardial deformation imaging, torsion, stress echocardiography, contrast and three-dimensional echocardiography.

Left atrial volumes: two-dimensional, three-dimensional, cardiac magnetic resonance and computed tomography measurements.

Purpose Of Review: Evaluation of left atrial volume is important, as it is a biomarker of cardiovascular disease and outcomes and correlates with diastolic dysfunction severity. Left atrial volume measurements by different imaging modalities, including 2D and 3D echocardiography (2DE and 3DE), cardiac magnetic resonance (CMR) and computed tomography (CT), are reviewed in regard to recent advances, methodology, prognostic value and limitations.

Recent Findings: Left atrial volume assessments correlate well between the different imaging modalities; however, 2DE significantly underestimates left atrial measurements.

Left atrial enlargement and reduced atrial compliance occurs early in Fabry cardiomyopathy.

Background: Fabry disease is associated with left ventricular hypertrophy (LVH) and myocardial fibrosis. The aim of this study was to evaluate left atrial (LA) size and function using tissue Doppler-derived strain in patients with Fabry disease.

Methods: Echocardiography was performed in 33 Fabry patients (14 without LVH, 19 with LVH) before commencement of enzyme replacement therapy, and results were compared with those from age-matched and gender-matched controls (n=28 and n=38, respectively).

Hypertension accelerates the 'normal' aging process with a premature increase in left atrial volume.

Hypertension (HT) is associated with left ventricular (LV) diastolic dysfunction and consequent left atrial (LA) dilatation. We investigated changes in LA size and phasic function by decade in patients with HT. Patients with mild or moderate HT (n = 122) were compared with a case controlled normal cohort (blood pressure <140/90 mm Hg).

Left ventricular mass predicts left atrial appendage thrombus in persistent atrial fibrillation.

Aims: Atrial fibrillation (AF) can result in the development of left atrial appendage (LAA) thrombi. We sought to examine demographic and echocardiographic predictors of LAA thrombus in patients with persistent AF.

Methods And Results: One hundred and sixty-five patients in persistent AF (36 with LAA thrombus and 129 without thrombus) were studied.

Atrial strain rate is a sensitive measure of alterations in atrial phasic function in healthy ageing.

Objective: Strain and strain rate measure local deformation of the myocardium and have been used to evaluate phasic atrial function in various disease states. The aim of this study was to define normal values for tissue Doppler-derived atrial strain measurements and examine age-related changes by decade in healthy individuals.

Methods: Transthoracic echocardiograms were performed on 188 healthy subjects.

Atrial dilation and altered function are mediated by age and diastolic function but not before the eighth decade.

Objectives: This study investigated changes in left atrial (LA) volumes and phasic atrial function, by deciles, with normal aging.

Background: LA volume increase is a sensitive independent marker for cardiovascular disease and adverse outcomes. To use this variable more effectively as a marker of pathology and a gauge of outcome, physiological changes due to aging alone need to be quantitated.

Left atrial enlargement and phasic function in patients following non-ST elevation myocardial infarction.

Background: Changes in left atrial (LA) volumes after ST elevation myocardial infarction are reported but have not been well described following non-ST elevation myocardial infarction (NSTEMI).

Methods: Seventy-five patients with NSTEMIs were studied within 48 hours of presentation and in follow-up at 6 and 12 months; they were compared with age-matched normal controls (n = 100). Biplane indexed LA volumes were measured, and phasic LA volumes (conduit, passive, and active emptying) were calculated.

Segmental atrial function following percutaneous closure of atrial septum using occluder device.

Objectives: The aim of this study was to quantify segmental atrial function in patients 6 months after the insertion of atrial septal occluder (ASO) devices.

Methods: Patients with ASO devices (n = 23) were followed up for 6 months after device insertion and compared with a normal age-matched cohort (n = 30). A subgroup of 13 patients were studied before, immediately after, and 6 months after device insertion.

Differential recovery of regional atrial contraction after restoration of sinus rhythm after intraoperative linear radiofrequency ablation for atrial fibrillation.

The aim of this study was to quantitate regional atrial contractility in patients with atrial fibrillation (AF) maintained in sinus rhythm after creating lines of block by intraoperative linear radiofrequency ablation for AF. We hypothesized that left atrial regional and global function remains impaired after radiofrequency ablation, despite restoration of sinus rhythm in this cohort. Patients with chronic AF maintained in sinus rhythm > or =6 months after radiofrequency ablation (n = 28) were studied and compared with a chronic AF group who, after standard electrical transthoracic cardioversion, were maintained in sinus rhythm for 6 months (n = 32) and a normal cohort (n = 32).

Segmental atrial contraction in patients restored to sinus rhythm after cardioversion for chronic atrial fibrillation: a colour Doppler tissue imaging study.

Aims: There is little known about segmental atrial function in patients with atrial arrhythmias. We evaluated segmental atrial contractility using colour Doppler tissue imaging (CDTI) in patients with chronic atrial fibrillation (CAF) who were successfully restored and maintained in sinus rhythm (SR).

Methods And Results: We compared the segmental atrial contractility in 39 CAF patients who were successfully cardioverted and maintained in SR for 6 months.

Automated ventricular substrate mapping--evaluation in an ovine chronic myocardial infarction model.

Introduction: We hypothesized that automated electrogram analysis might enable rapid localization of ventricular scar. This would allow the delivery of interventions such as radiofrequency ablation or therapeutic agents to critical areas within the scar and scar periphery.

Methods: Substrate mapping was performed on seven sheep 36.

Cooled needle catheter ablation creates deeper and wider lesions than irrigated tip catheter ablation.

Objectives: To design and test a catheter that could create deeper ablation lesions.

Background: Endocardial radiofrequency (RF) ablation is unable to reliably create transmural ventricular lesions. We designed an intramural needle ablation catheter with an internally cooled 1.

Value of noncontact mapping for identifying left ventricular scar in an ovine model.

Background: We assessed the hypothesis that "virtual electrograms" from a noncontact mapping system (EnSite 3000) could be used to localize myocardial scar.

Methods And Results: Myocardial infarctions were induced in sheep by inflating an angioplasty balloon in the left anterior descending coronary artery for 3 hours. Scar mapping was performed on 8 sheep without inducible ventricular tachycardia by use of the noncontact mapping system and a 256-channel contact mapping system.

Cooled intramural needle catheter ablation creates deeper lesions than irrigated tip catheter ablation.

Endocardial radiofrequency ablation of the left ventricle does not create transmural lesions reliably even with active electrode cooling. The authors developed a prototype catheter with an internally cooled needle electrode that could be advanced an adjustable distance into the myocardium. Freshly excised hearts from eight male sheep were perfused and superfused using oxygenated ovine blood.

A comparison of open irrigated and non-irrigated tip catheter ablation for pulmonary vein isolation.

Aims: The relative efficacy and safety of open irrigated tip catheters compared with conventional non-irrigated catheters for pulmonary vein isolation (PVI) is unknown.

Methods: Forty-eight patients undergoing PVI using an open irrigated tip ablation catheter (Group 1) were compared with a group of 31 historical controls (Group 2). The control group underwent similar procedures using a standard, 4 mm tip, temperature controlled ablation catheter.

Noncontact mapping of the left ventricle: insights from validation with transmural contact mapping.

It is not clear whether the noncontact electrograms obtained using the EnSite system in the left ventricle resemble most closely endocardial, intramural, or epicardial contact electrograms or a summation of transmural electrograms. This study compared unipolar virtual electrograms from the EnSite system with unipolar contact electrograms from transmural plunge needle electrodes using a 256-channel mapping system. The study also evaluated the effects of differing activation sites (endocardial, intramural, or epicardial).

Comparison of epicardial and endocardial linear ablation using handheld probes.

Background: The optimal technique for producing linear radiofrequency thermal lesions in myocardial tissue is unclear. We compared epicardial ablation on the beating heart with endocardial ablation after cardioplegia.

Methods: Radiofrequency lesions were produced using a multielectrode malleable handheld probe in ovine myocardium with three wall thicknesses.