Arrhythmogenic Cardiomyopathy and Skeletal Muscle Dystrophies: Shared Histopathological Features and Pathogenic Mechanisms.

Arrhythmogenic cardiomyopathy (ACM) is a heritable cardiac disease characterized by fibrotic or fibrofatty myocardial replacement, associated with an increased risk of ventricular arrhythmias and sudden cardiac death. Originally described as a disease of the right ventricle, ACM is currently recognized as a biventricular entity, due to the increasing numbers of reports of predominant left ventricular or biventricular involvement. Research over the last 20 years has significantly advanced our knowledge of the etiology and pathogenesis of ACM.

Left Ventricular Mass in Hypertrophic Cardiomyopathy Assessed by 2D-Echocardiography: Validation with Magnetic Resonance Imaging.

We aim to validate echocardiographic left ventricular (LV) mass (echoLVM) in sixty-one patients with hypertrophic cardiomyopathy (HCM), using cardiac magnetic resonance measures (cmrLVM) as gold standard. cmrLVM was calculated using LV short-axis images, from base to apex, whereas echoLVM by LV epicardial minus LV endocardial volumes in 4 and 2 chamber views, using Simpson disk summation; trabeculae and papillary muscle were excluded in both cmrLVM and echoLVM. cmrLVM and echoLVM were not different by paired t test (145 ± 66 vs 147 ± 61; p = 0.

Left ventricular diastolic dysfunction in type I Gaucher disease: an echo Doppler study.

Type I Gaucher disease (GD1) is an autosomal recessive lysosomal storage disease characterized by multiorgan damage. Left ventricular (LV) involvement has been rarely reported. Accordingly, the aim of the study was to evaluate LV geometry and function in a series of patients with GD1.

Speckle-tracking analysis based on 2D echocardiography does not reliably measure left ventricular torsion.

Purpose: Worldwide left ventricular (LV) twist is measured by 2D speckle tracking acquiring apical short axis at a LV level where papillary muscles are no longer visible; however, we hypothesized that this methodological recommendation is not enough accurate to obtain a reliable measurement of apical rotation.

Methods: We measured twist and untwist rate in 30 healthy subjects by following the earlier method. By 3D echocardiography, we identified two LV apex levels: (i) 3D Apex, defined as the last apical slice at which LV cavity was visible; (ii) 2D Apex, defined as the level where diameters are equal to those of apical LV short axis used for twist analysis in the same subject.

Percutaneous treatment of patients with heart diseases: selection, guidance and follow-up. A review.

Aortic stenosis and mitral regurgitation, patent foramen ovale, interatrial septal defect, atrial fibrillation and perivalvular leak, are now amenable to percutaneous treatment. These percutaneous procedures require the use of Transthoracic (TTE), Transesophageal (TEE) and/or Intracardiac echocardiography (ICE). This paper provides an overview of the different percutaneous interventions, trying to provide a systematic and comprehensive approach for selection, guidance and follow-up of patients undergoing these procedures, illustrating the key role of 2D echocardiography.