The Subclinical Cardiomyopathy of Friedreich's Ataxia in a Pediatric Population.

Background: Identification of a subclinical cardiomyopathy in pediatric patients with Friedreich's ataxia (FA) has not been well-described.

Methods: We performed echocardiography (Echo), cardiac magnetic resonance imaging (cMRI), and neurologic assessment in a cross-sectional analysis of 48 genetically confirmed FA subjects aged 9-17 years with moderate neurologic impairment but without a cardiovascular history. Echo- and cMRI-determined left ventricular mass were indexed (LVMI) to height in grams/m.

A Summary of the American Society of Echocardiography Foundation Value-Based Healthcare: Summit 2014: The Role of Cardiovascular Ultrasound in the New Paradigm.

Value-Based Healthcare: Summit 2014 clearly achieved the three goals set forth at the beginning of this document. First, the live event informed and educated attendees through a discussion of the evolving value-based healthcare environment, including a collaborative effort to define the important role of cardiovascular ultrasound in that environment. Second, publication of these Summit proceedings in the Journal of the American Society of Echocardiography will inform a wider audience of the important insights gathered.

Device closure of paravalvular defects following transcatheter aortic valve replacement with the Edwards Sapien valve.

Paravalvular aortic insufficiency (AI) is observed in the majority of patients undergoing transcatheter aortic valve replacement (TAVR). While paravalvular AI is most commonly modest following TAVR, moderate or severe AI was seen in 10.5% and 6.

Percutaneous closure of a mitral perivalvular leak using three dimensional real time and color flow imaging.

The role of echocardiography, including three-dimensional (3D) echocardiography, during interventional procedures in the cardiac catheterization laboratory is continuing to expand as interventional cardiologists perform more catheter-based interventions. Echocardiography often complements angiographic imaging of cardiac structures and sometimes provides additional information not available by angiography and fluoroscopy. The closure of perivalvular leaks using catheter-based techniques is one of the areas in which 3D echocardiography can be helpful.

Assessment of the right ventricle by echocardiography: a primer for cardiac sonographers.

The assessment of right ventricular (RV) structure and function by echocardiography has been improved by advancements that include Doppler tissue imaging, strain imaging, and three-dimensional imaging. Doppler tissue imaging and strain imaging can be useful for the assessment of regional RV systolic and diastolic function. Three-dimensional imaging has been reported to determine RV volumes and ejection fraction, which have previously been cumbersome to measure with conventional two-dimensional echocardiography.

Casq2 deletion causes sarcoplasmic reticulum volume increase, premature Ca2+ release, and catecholaminergic polymorphic ventricular tachycardia.

Cardiac calsequestrin (Casq2) is thought to be the key sarcoplasmic reticulum (SR) Ca2+ storage protein essential for SR Ca2+ release in mammalian heart. Human CASQ2 mutations are associated with catecholaminergic ventricular tachycardia. However, homozygous mutation carriers presumably lacking functional Casq2 display surprisingly normal cardiac contractility.