Atrial Flutter in PRKAG2 Syndrome: Clinical and Electrophysiological Characteristics.

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.

Prevalence and prognostic value of ventricular dyssynchrony in Chagas cardiomyopathy.

Background: Chagas cardiomyopathy is one important cause of heart failure in Latin America. Ventricular dyssynchrony may be a factor of decompensation in the course of this disease, but there are no data on its prevalence and its main prognostic implications yet.

Objective: Describe prevalence and prognostic value of ventricular dyssynchrony in Chagas cardiomyopathy.

Delayed right ventricular perforation in patient with implantable cardioverter - defibrillator.

We describe the case of a 62-year-old patient who returned for evaluation nine months after receiving an implantable cardioverter-defibrillator (ICD) with signs of delayed right ventricular (RV) perforation. The clinical signs that allowed the diagnosis of this late presentation to be achieved are discussed herein, as well as the conduct and the frequency of this complication in the literature.

Pharmacological treatment of electrical storm in cathecolaminergic polymorphic ventricular tachycardia.

We present a case of a young boy who had an implantable cadrioverter defibrillator implanted for refractory syncope and cathecolaminergic polymorphic ventricular tachycardia (CPVT). The patient underwent an electrical storm with multiple shocks and serious compromise of quality of life and risk. The pharmacological options are discussed as well as the strategies of managing ventricular arrhythmias in CPVT.

Atrial fibrillation ablation in Brazil: results of the registry of the Brazilian Society of Cardiac Arrhythmias.

Background: Aiming to define the profile of curative atrial fibrillation (AF) ablation in Brazil, the Brazilian Cardiac Arrhythmia Society [Sociedade Brasileira de Arritmias Cardíacas] (SOBRAC) created the Brazilian Registry of AF Ablation [Registro Brasileiro de Ablação da FA].

Objective: To describe the results of this registry.

Methods: A questionnaire was sent to SOBRAC members asking about data on patients submitted to AF ablation between September 2005 and November, 2006.

Familial pseudo-Wolff-Parkinson-White syndrome.

Introduction: PRKAG2 plays a role in regulating metabolic pathways, and mutations in this gene are associated with familial ventricular preexcitation, hypertrophic cardiomyopathy, and atrioventricular conduction disturbances. Clinico-pathologic and experimental data suggest the hypothesis of a glycogen storage disease.

Objective: To report a unique pattern of clinical features observed in individuals with a mutant PRKAG2 from two unrelated families.

Decrease of plasma triglycerides during the acute phase of unstable angina or non-ST elevation myocardial infarction is a marker of recurrent ischemia.

Both increase and decrease of plasma triglycerides during acute coronary syndromes (ACS) are reported, however, a clinical relevance for these distinct metabolic responses is unclear. To test the association between distinct responses of lipid metabolism and cardiovascular risk, 39 subjects admitted with non-ST elevation ACS within 48 h of presentation had plasma lipids measured on the first and sixth days of hospitalization, and continuous electrocardiogram was performed during the first 2 days to quantify recurrent ischemia and heart rate variability. No lipid-lowering therapy was offered to the patients.

Anti-inflammatory effect of atorvastatin (80 mg) in unstable angina pectoris and non-Q-wave acute myocardial infarction.

In this randomized trial, C-reactive protein increased during the first 5 days of an acute coronary syndrome in patients treated with placebo, but this phenomenon was not observed in those randomized to atorvastatin 80 mg/day. This suggests that short-term statin therapy inhibits inflammation in patients with non-ST-elevation acute coronary syndromes.