Prevalence of papillary muscle hypertrophy in fabry disease.

Background And Aims: Fabry disease (FD) is an X-linked genetic lysosomal disease, in which a deficit in the alpha-galactosidase A enzyme results in lysosomal build-up of globotriaosylceramide in several organs, causing cardiac, renal and cerebrovascular complications. The aim of this study was to assess the prevalence of papillary muscle hypertrophy (PMH) in patients with FD.

Methods: A group of 63 patients with FD and a positive genetic diagnosis were studied and were divided into two groups: one included 24 patients with FD and LVH and another group included 39 patients with FD and without LVH.

Use of tissue doppler imaging for the early detection of myocardial dysfunction in patients with the indeterminate form of Chagas disease.

Introduction: Chagas disease is one of the most common diseases in Latin America and heart involvement is the main cause of death. This study aimed to determine differences in tissue Doppler imaging (TDI) parameters in the assessment left and right ventricular function in patients with the indeterminate form of Chagas disease compared to those in healthy controls.

Methods: We compared 194 patients with the indeterminate form of Chagas disease to 72 age-matched healthy individuals.

Speckle tracking echocardiography to assess regional ventricular function in patients with apical hypertrophic cardiomyopathy.

Aim: To explore regional systolic strain of midwall and endocardial segments using speckle tracking echocardiography in patients with apical hypertrophic cardiomyopathy (HCM).

Methods: We prospectively assessed 20 patients (mean age 53 ± 16 years, range: 18-81 years, 10 were male), with apical HCM. We measured global longitudinal peak systolic strain (GLPSS) in the midwall and endocardium of the left ventricle.

Echocardiographic evaluation of mitral valve repair by modified "edge to edge" technique.

The "edge to edge" or "double-orifice" technique is an alternative surgical option of mitral valve repair to treat mitral regurgitation. Echocardiography is very useful to evaluate the postoperative valve function, but since this technique is not frequently used, there is little experience about its echocardiographic features, which are different from those of the classic mitral valve repair. In this report, we present a patient who underwent this repair with a modified approach called "triple-orifice technique" and was evaluated by transthoracic and transesophageal echocardiography.