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.

Hypothyroid cardiomyopathy: A reversible phenocopy of hypertrophic cardiomyopathy.

We present the case of a 46-year-old patient with hypothyroidism secondary to Hashimoto's thyroiditis who was admitted with decompensation in the form of myxedema. A 2-D echocardiogram shows a septal asymmetric hypertrophy, with low-voltage QRS complex in the ECG and a bull's-eye map of longitudinal strain with preserved apical strain with reduction of mid and basal strain that results in "cherry on the top" pattern, similar to the most frequent phenocopy of hypertrophic cardiomyopathy, as is the cardiac amyloidosis, and that, unlike this pathology, reverted after the patient reached the euthyroid state.

Rapidly progressive aortic stenosis treated with transcatheter aortic valve implantation in a patient with Fabry disease: a case report.

Background: Fabry disease (FD) is a rare lysosomal storage disease, caused by mutations in the gene encoding the enzyme α-galactosidase A (α-Gal A). Cardiac involvement is one of the main causes of death and it is characterized by progressive concentric left ventricular hypertrophy (LVH), which in most cases is symmetric. Mild thickening of the left-sided valves is seen in as many as a quarter of patients.

Speckle Tracking Imaging in Patients with Pulmonary Hypertension.

Background: Right ventricular (RV) systolic dysfunction is a strong predictor of mortality in pulmonary hypertension (PH). The goal of this study was to investigate whether right atrium (RA) and RV myocardial strain related to PH using speckle tracking echocardiography provide a superior estimation of RV systolic function than 2-dimensional (2D)-echo.

Methods: This cross-sectional study analyzed 22 patients with a diagnosis of PH stratified by right heart catheterization, and they were compared to a control group of 22 age- and sex-matched healthy subjects.

Speckle tracking echocardiography in the indeterminate form of Chagas disease.

Background: Chagas disease is one of the most common diseases in Latin-America, and cardiac involvement is a significant cause of death. Assessment of myocardial strain may detect early myocardial damage.

Objectives: To determine differences in longitudinal strain using speckle tracking to assess regional and global left ventricular function in patients with the indeterminate form of Chagas disease, in comparison with a control group.

Mechanical dispersion in Fabry disease assessed with speckle tracking echocardiography.

Background: Fabry disease (FD) is a rare X-linked storage disorder caused by deficiency of the lysosomal enzyme α-galactosidase A, and it typically causes multiorgan dysfunction. The main cause of death is heart disease resulting from left ventricular (LV) diastolic dysfunction, LV systolic dysfunction, severe LV hypertrophy (LVH), and sudden death. In several cardiac disorders, LV systolic dysfunction and ventricular arrhythmias are associated with mechanical dispersion (MD).

Twenty Years of Clinical Experience with Cardiac Myxomas: Diagnosis, Treatment, and Follow Up.

Background: Cardiac myxomas are the most frequent cardiac tumors. Although histologically benign, in some cases myxomas may be lethal, due to impairment of cardiac dynamics and their thromboembolic potential. The study aimed to assess the clinical presentation of cardiac myxomas and their correlation with echocardiographic features and to describe the perioperative results and long-term outcome of surgically treated patients.

Detection of angiographically significant coronary obstruction using resting transthoracic coronary Doppler echocardiography.

Background: Transesophageal Doppler echocardiography has shown that significant stenosis can be detected based on the presence of aliasing with color Doppler in the stenotic area. The study aimed to assess the detection of angiographically significant coronary stenosis (ASCS) by analyzing the characteristics and velocities of resting coronary artery flow (RCF) using transthoracic coronary Doppler echocardiography (TCDE).

Methods: TCDE was performed before diagnostic coronary angiography (CA).

Bone cement cardiac and pulmonary embolism.

This case describes a complication of bone cement use. A 65-year-old male patient with back spine trauma caused by a fall, underwent a percutaneous vertebroplasty. Five years later, he consulted for palpitations, and the electrocardiogram showed supraventricular arrhythmia.

Papillary fibroelastoma: clinical and echocardiographic features and initial approach in 54 cases.

Introduction: Papillary fibroelastoma (PFE) is a benign cardiac tumor that is currently detected more often due to the technological improvements in echocardiography.

Objectives: To describe the echocardiographic features of PFE and correlate them with the clinical presentation and initial treatment.

Materials And Methods: A prospective analysis of patients with a diagnosis of PFE was conducted between 2000 and 2015.

Pulmonary Edema Assessed by Ultrasound: Impact in Cardiology and Intensive Care Practice.

Pulmonary edema is a frequent condition found in adult patients hospitalized in cardiology wards and intensive care units. Ultrasonography is a diagnostic modality with a high sensitivity for the detection of extravascular lung water, visualized as B lines, and usually caused by cardiogenic or noncardiogenic pulmonary edema. This paper highlights a simple method for the assessment of patients with pulmonary edema, which allows for a differential diagnosis of its possible mechanism and contributes to therapeutic intervention guiding and monitoring.

Apical left ventricular hypertrophy and mid-ventricular obstruction in fabry disease.

We report the case of a rare cardiac presentation of Fabry disease. Although concentric left ventricular hypertrophy is a major cardiac finding in Fabry disease, there is no case report of dynamic obstruction at mid-left ventricular level. We describe a 59-year-old-woman suffering from a severe form of Fabry disease, mimicking an apical hypertrophic cardiomyopathy with mid-ventricular obstruction.

CoreValve prosthesis causes anterior mitral leaflet perforation resulting in severe mitral regurgitation.

Percutaneous transcatheter aortic valve replacement (TAVR) has become an alternative to surgical therapy for patients with severe aortic stenosis and high operative risk, but it is associated with specific complications. We report the case of a 72-year-old man who underwent the procedure without complications; however, 45 days after the procedure, he was admitted to the hospital with symptoms of heart failure secondary to severe mitral regurgitation. Necropsy findings showed prosthesis malposition and perforation of the anterior mitral leaflet caused by the contact of the stent of the CoreValve prosthesis (Medtronic, Minneapolis, MN).

Severe mitral regurgitation after radiotherapy.

We present the case of a 69-year-old patient with a history of gynecological neoplasia and a pulmonary metastasis, who in 1996 underwent chemotherapy and mediastinal radiotherapy followed by cancer remission. Ten years later she presented with heart failure and her Doppler echocardiogram showed severe mitral regurgitation with pulmonary hypertension. In 2011, she underwent a mitral valve replacement with a biological prosthesis and the pathology exam revealed valve damage consistent with radiotherapy-induced changes.

Complete regression of myocardial involvement associated with lymphoma following chemotherapy.

Cardiac involvement as an initial presentation of malignant lymphoma is a rare occurrence. We describe the case of a 26 year old man who had initially been diagnosed with myocardial infiltration on an echocardiogram, presenting with a testicular mass and unilateral peripheral facial paralysis. On admission, electrocardiograms (ECG) revealed negative T-waves in all leads and ST-segment elevation in the inferior leads.

Esophageal carcinoma and transthoracic echocardiography.

We report a rare case of a patient with esophageal carcinoma diagnosed using transthoracic echocardiography. This examination proved to be useful in the identification of a paracardiac mediastinal mass. Images of the esophageal carcinoma, of the stent in the esophagus, and the bubbles inside the stent generated with the ingestion of a carbonated beverage, have not been previously published.

Assessment of coronary flow with transthoracic color Doppler echocardiography in patients with hypertrophic cardiomyopathy.

Background: Recent advances in echocardiography have allowed assessment of flow velocity in the epicardial coronary arteries of patients with ischemic heart disease, using transthoracic color Doppler echocardiography (TTDE). However, few data are available regarding coronary blood flow in patients with hypertrophic cardiomyopathy (HCM).

Objective: To assess the epicardial coronary arteries of patients with HCM.

Two-dimensional speckle tracking echocardiography for early detection of myocardial damage in young patients with Fabry disease.

Fabry disease (FD) is characterized by left ventricular hypertrophy (LVH). Conventional echocardiography is not sensitive enough to perform the preclinical diagnosis To assess whether longitudinal myocardial strain of the left ventricle (LV), using speckle tracking, is useful to detect early myocardial involvement in FD. Forty-four patients with FD who were diagnosed with genetic testing were prospectively included and were compared to a sex-matched control group.

Two-dimensional speckle tracking echocardiography for the assessment of atrial function.

Echocardiography is the most common diagnostic method for assessing atrial function but the technique has some limitations. Traditionally, assessment of left atrial function has been performed by measuring volumes with 2D echocardiography. Additionally, it can be assessed with transmitral Doppler and pulmonary vein Doppler.

Left ventricular thrombus mimicking primary cardiac tumor in a patient with primary antiphospholipid syndrome and recurrent systemic embolism.

Primary antiphospholipid syndrome (APS) is a well-defined entity characterized by spontaneous and recurrent abortion, thrombocytopenia and recurrent vascular thromboses (arterial and venous). Left ventricular thrombus mimicking primary cardiac tumor with recurrent systemic embolism has not been previously reported. In this report we describe a 39 year-old man admitted to hospital presenting with left hemiparesis and a peripheral embolism.

Impaired myocardial function in hypertrophic cardiomyopathy.

Background: Tissue Doppler imaging (TDI) parameters of peak myocardial velocities (S', E', and A') has been employed to assess the regional left ventricular myocardial function. The global function index (GFI) derived from TDI has been recently employed to distinguish the different etiologies of left ventricular hypertrophy.

Objective: To analyze whether the GFI or individual TDI parameters of peak myocardial velocities (S', E', and A') allows detecting different degrees of regional myocardial dysfunction in the most frequent forms of hypertrophic cardiomyopathy (HCM).