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Brugada syndrome was first described in 1992 as right precordial ST-segment elevation in patients with structurally normal hearts and sudden cardiac death. Brugada Syndrome is one of the most common reasons for sudden cardiac death (4-12%) and is a hereditary disease with an autosomal dominant pattern of transmission with nearly 300 pathogenic variants in 19 responsible genes published. The present review focuses on the diagnosis, genetics, risk stratification, and management of patients with Brugada Syndrome.

Pacemaker telemetry reflects algorithm-dependent interpretations about the cardiac rhythm. For these data to be useful, it is necessary to recognize the limitations of each algorithm and its interactions with pacing and sensing parameters. We report on a case in which pacemaker Holter recording showed several episodes of nonsustained supraventricular tachycardia which were erroneously classified as ventricular tachycardia.

Left ventricular (LV) noncompaction is a congenital dysfunction of endomyocardial morphogenesis characterized by excessively prominent trabeculations and deep intratrabecular recesses of the myocardium. Its association with Ebstein's anomaly is very rare. A 13-year-old boy presented to the outpatient clinic for a cardiac evaluation before enrolling in a sports activity. On physical examination, there was a grade 1/6 systolic murmur at the tricuspid valve location. His electrocardiogram was normal. Transthoracic echocardiography revealed numerous large trabeculations and deep intratrabecular recesses at the apex, lateral wall, and the apical part of the interventricular septum. Color flow Doppler examination confirmed the presence of blood flow within the trabeculae. The size and ejection fraction of the LV was normal (65%). The attached margins of the septal and inferior leaflets of the tricuspid valve were apically displaced and there was mild tricuspid regurgitation. The distance between the mitral and tricuspid annuli was 20 mm. The right ventricle showed no dilatation and had normal systolic function, but the right atrium was moderately enlarged. As the patient was asymptomatic and the LV systolic function was preserved, he was scheduled for regular follow-up without medication except for low-dose aspirin (100 mg/day). After five years of follow-up, he was still asymptomatic with normal size and ejection fraction of both ventricles.

Objectives: This study sought to investigate the role of left atrial diameter in predicting adverse clinical events in patients with hypertrophic cardiomyopathy (HCM)., Study Design: The study prospectively included 83 consecutive patients (45 males, 38 females; mean age 47+/-17 years) with HCM. Admission transthoracic echocardiograms were obtained. Left atrial diameter was measured at end-systole from the parasternal long-axis view and left atrial diameter index (LADI) was derived (left atrial diameter/body surface area). Clinical endpoints were defined as cardiovascular death and/or worsening heart failure symptoms. The mean follow-up period was 622+/-366 days (range 14 to 1,142 days)., Results: Adverse clinical events were seen in 27 patients (32.5%), including two deaths and deterioration in NYHA functional class (n=25). Based on the median LADI of 2.5 cm/m2, patients whose LADI was =or>2.5 cm/m2 had a higher value of log NT-proBNP, increased maximal wall thickness, and higher rates of adverse events, advanced NYHA functional class, significant mitral regurgitation, and left ventricular outflow tract obstruction. In ROC analysis, the cutoff value of 2.2 cm/m2 for LADI predicted adverse events with 89% sensitivity, 34% specificity, 84% negative and 38% positive predictive values. Kaplan-Meier analysis showed that patients with LADI p<2.2 cm/m2 had a significantly lower rate of adverse events. In binary logistic regression, predictors of increased LADI were female sex, advanced NYHA functional class, log NT-proBNP, maximal wall thickness, significant mitral regurgitation, and left ventricular outflow tract obstruction. However, log NT-proBNP was the only independent predictor of increased LADI in multivariate regression analysis (OR=2.1; 95% CI: 1.3-3.4; p=0.004)., Conclusion: Our data suggest that LADI may be used as a powerful predictor of adverse clinical events in patients with HCM.

Objectives: Traditional risk factors may underestimate the burden of subclinical atherosclerosis in women. Recently, multislice computed tomography (MSCT) has become widely available in detecting early coronary artery disease (CAD). We sought the prevalence of CAD in low to moderate-risk asymptomatic women by MSCT coronary artery calcium (CAC) scoring and coronary angiography., Study Design: The study included 185 women (mean age 57+/-12 years) without known CAD and diabetes, with low or moderate risk for CAD based on traditional risk scoring. Coronary artery calcium scoring and coronary angiography were performed by MSCT, which included a segment-based plaque detection and characterization of calcification. The plaques were classified based on the luminal stenotic effect (>50%). Patients with = or >1 stenotic plaque were classified as having obstructive CAD. Angiographic findings were compared with calcium scores., Results: Coronary artery calcium scoring and coronary angiography detected CAD in 63 (34.1%) and 100 (54.1%) women, respectively. In both groups, women were significantly older and had higher prevalences of hypertension and dyslipidemia. Coronary angiography showed CAD in 41 women (41%; 14.6% were obstructive) without CAC. These women were significantly younger than those with a positive CAC score (p<0.01). Age (p<0.02) and hypertension (p<0.05) were found as independent predictors of CAD detected by coronary angiography., Conclusion: Multislice computed tomography identified a subset of low-risk women who might be at higher risk than that suggested by current risk stratification strategies. Women, especially having hypertension and dyslipidemia may be potential candidates for further risk stratification by MSCT coronary angiography.

Objectives: The Irx4 gene is predominantly expressed in cardiac ventricles. It has been demonstrated in animal studies that disruption of the Irx4 gene causes inhibition of chamber-specific expression of myosin heavy chain genes, resulting in abnormal ventricular gene expression and cardiac hypertrophy. In this study, we aimed to investigate a possible association between mutations in the Irx4 gene and hypertrophic cardiomyopathy (HC)., Study Design: The study included 68 patients (32 females, 36 males; mean age 49 years; range 17 to 74 years) with HC and 67 healthy controls (33 females, 34 males; mean age 45 years; range 20 to 88 years). All the patients were evaluated with a detailed history, physical examination, 12-lead electrocardiography, and transthoracic echocardiography. DNA samples of all the subjects were extracted. Genomic DNA fragments were amplified by polymerase chain reaction and screened by single-strand conformation polymorphism analysis. DNA sequences were determined through an automated sequencing system., Results: All exons in the Irx4 gene were examined. No mutations were detected associated with HC. Four polymorphisms were identified including G355>A, A381>G, G1203>A, and C1431>T. Compared with patients having the GA and GG genotyes, patients with the AA genotype of A381>G polymorphism were found to have a higher maximal left ventricle outflow tract gradient (p=0.03), prolonged corrected QT dispersion (p=0.05), and albeit not statistically significant, increased septal thickness (p=0.07)., Conclusion: This is the first human study investigating the association between the Irx4 gene and HC. Polymorphism A381>G of the Irx4 gene may have a modifier effect on septal thickness, resulting in increased corrected QT dispersion and higher outflow gradients.