2 results
A monthly publication of the Brazilian Society of Cardiology and considered the main channel for the promotion of Brazilian cardiovascular research.
ISSN 0066-782X
eISSN 1678-4170
Magalhães et al. assessed atrial flutter’s clinical, electrocardiographic, and electrophysiologic characteristics in patients with and without PRKAG2 cardiomyopathy. Although genetic sequencing was not carried out in their control patients, the absence of clinical features of this pathology is acceptable evidence of a non-carrier state. In humans, mutations in the PRKAG2 gene result in a highly penetrant phenotype. It is dominated by the cardiac features of left ventricular hypertrophy, ventricular pre-excitation, atrial tachyarrhythmia, cardiac conduction disease, and myocardial glycogen storage. It […]
Keywords: Arrhythmias, Cardiac; Atrial Flutter; Atrioventricular Block; Cardiac Arrythmias; Cardiomyopathy, Hypertrophic; Glycogen Storage Disease; Hypertrophy, Left Ventricular; Left Ventricular Hypertrophy
Eduardo Faria Soares de Magalhães
, Luiz Pereira de Magalhães
, Jussara de Oliveira Pinheiro, Alex Teixeira Guabiru, Roque Aras
Eduardo Faria Soares de Magalhães
, Luiz Pereira de Magalhães
, Jussara de Oliveira Pinheiro, Alex Teixeira Guabiru, Roque Aras
Abstract Background PRKAG2 syndrome is a rare autosomal dominant disease, a phenocopy of hypertrophic cardiomyopathy characterized by intracellular glycogen accumulation. Clinical manifestations include ventricular preexcitation, cardiac conduction disorder, ventricular hypertrophy, and atrial arrhythmias. Objective To compare the clinical and electrophysiological characteristics observed in patients with atrial flutter, with and without PRKAG2 syndrome. Methods An observational study comparing patients with atrial flutter: group A consisted of five patients with PRKAG2 syndrome from a family, and group B consisted of 25 patients […]
Keywords: Atrial Flutter; Atrioventricular Block; Cardiac Arrhythmias; Cardiomyopathy, Hypertrophic; Glycogen Storage Disease; Hypertrophy, Left Ventricular
Abstract Background PRKAG2 syndrome is a rare autosomal dominant disease, a phenocopy of hypertrophic cardiomyopathy characterized by intracellular glycogen accumulation. Clinical manifestations include ventricular preexcitation, cardiac conduction disorder, ventricular hypertrophy, and atrial arrhythmias. Objective To compare the clinical and electrophysiological characteristics observed in patients with atrial flutter, with and without PRKAG2 syndrome. Methods An observational study comparing patients with atrial flutter: group A consisted of five patients with PRKAG2 syndrome from a family, and group B consisted of 25 patients […]
Keywords: Arrhythmias, Cardiac; Atrial Flutter; Atrioventricular Block; Cardiac Arrythmias; Cardiomyopathy, Hypertrophic; Glycogen Storage Disease; Hypertrophy, Left Ventricular; Left Ventricular Hypertrophy