Narumol Chaosuwannakit

SCN5A Genetic Polymorphisms Associated With Increased Defibrillator Shocks in Brugada Syndrome

Background Brugada syndrome (BrS) is an inherited primary arrhythmia disorder leading to sudden cardiac arrest. SCN5A, encoding the α‐subunit of the cardiac sodium channel (Nav1.5), is the most common pathogenic gene of BrS. An implantable cardioverter defibrillator (ICD) is the standard treatment for secondary prevention. This study aimed to evaluate association of the SCN5A variant with this cardiac conduction disturbance and appropriate ICD shock therapy in Thai symptomatic BrS patients with ICD implants. Methods and Results Symptomatic BrS patients diagnosed at university hospital were enrolled from 2008 to 2011. The primary outcome of the study was an appropriate ICD shock defined as having non‐pacing‐associated ICD shock after the occurrence of ventricular tachycardia or ventricular fibrillation. Associations between SCN5A polymorphisms, cardiac conduction disturbance, and potential confounding factors associated with appropriate ICD shock therapy were analyzed. All 40 symptomatic BrS patients (median age, 43 years) with ICD implantations were followed for 24 months. There were 16 patients (40%) who had the appropriate ICD shock therapy after ICD treatment. An independent factor associated with appropriate ICD shock therapy was SCN5A‐R1193Q with an adjusted hazard ratio of 10.550 (95% CI, 1.631–68.232). Conclusions SCN5A‐R1193Q is associated with cardiac conduction disturbances. It may be a genetic marker associated with ventricular arrhythmia leading to appropriate ICD shock therapy in symptomatic BrS patients with ICD treatment. Because of the small sample size of study population and the appropriate ICD shock outcome, further large studies are needed to confirm the results of this study.

Malignant mesothelioma of the pericardium: a report of two different presentations.

Malignant mesothelioma of the pericardium is a rare and fatal condition that clinicians should be aware of due to its variability of clinical manifestation. The diagnosis may be delayed as a result of delayed treatment. Here, we report two cases of malignant pericardial mesothelioma with two different clinical aspects: cardiac tamponade and mimic tuberculous pericarditis. Both patients: may have indirect exposure to asbestos. Despite chemotherapy, both patients died at 2 weeks and 3 months after the diagnosis. Malignant mesothelioma of the pericardium is fatal, has a variety of presentation, and may not be related to asbestosis exposure.

Prevalence and associated factors of early repolarization pattern in healthy young northeastern Thai men: A correlation study with Brugada electrocardiography

Early repolarization pattern (ERP) is characterized by J‐point elevation with QRS notching or slurring in the terminal portion of the QRS complex. It may be associated with sudden death. Brugada syndrome (BS) is a genetic and fatal disease commonly found in northeastern Thai men. Data on the rate and predictors of ERP in Asian populations are limited. In addition, the correlation between ERP and BS has never been studied in an endemic area of BS. This study aimed to evaluate the prevalence of ERP and its associated factors in young, healthy male Asian subjects.

Value of cardiac magnetic resonance imaging in systemic sclerosis

Objectives To assess the prevalence and patterns of cardiac abnormalities as detected by cardiac magnetic resonance imaging (MRI) in systemic sclerosis. Material and methods Twenty-six consecutive patients with systemic sclerosis underwent cardiac MRI to determine morphological, functional, perfusion at rest, and delayed enhancement abnormalities. Results At least one abnormality on cardiac MRI was observed in 19/26 (73%) patients. Increased myocardial signal intensity in T2 was observed in 10 patients (38.5%), thinning of the left ventricular myocardium in 1 patient (3.5%), and pericardial effusion in 12 patients (46%). Left and right ventricular ejection fractions were altered in 10 patients (38.5%) and 11 patients (42%), respectively. Myocardial delayed contrast enhancement was found in 11 (42%) patients. No perfusion defects at rest were found. Patients with limited systemic sclerosis had similar cardiac MRI abnormalities to patients with diffuse systemic sclerosis. Four out of 11 patients (36.4%) without pulmonary arterial hypertension had right ventricular dilatation. Conclusions The present study shows that cardiac MRI is an accurate and reliable technique to diagnose cardiac involvement in systemic sclerosis and to analyze precisely its mechanisms, including inflammatory, microvascular and fibrotic components. As it is non-invasive, quantitative and highly sensitive, cardiac MRI appears to be a method of choice to determine the natural history of untreated patients or to accurately monitor the effects of treatment. Moreover, it could provide powerful prognostic factors in both groups. Compared to echocardiography, cardiac MRI appears to provide additional information by visualizing myocardial fibrosis and inflammation. Finally, the present study has shown that RV dilatation is not specific for pulmonary arterial hypertension and could correspond to a specific heart involvement in systemic sclerosis.

Reduction of radiation dose for coronary computed tomography angiography using prospective electrocardiography-triggered high-pitch acquisition in clinical routine

Purpose To evaluate the image quality, radiation exposure, and means of application in a group of patients who underwent coronary computed tomography angiography (CCTA) performed with low-dose prospective electrocardiography (ECG)-triggered acquisition in which a standard sequence was added if the low-dose sequence did not allow reliable exclusion of coronary stenosis with respect to image quality. Material and methods The present study was approved by the Ethics Committee of the Faculty of Medicine, and informed consent was obtained from all patients. The authors performed a retrospective review of 256 consecutive patients referred for CCTA using dual-source CT scanner (Definition FLASH, Siemens, Germany). CCTA was performed using prospective ECG-triggered high-pitch acquisition. In patients with higher heart rates (> 65 bpm) or in whom irregular heart rates were noted prior to the scan, a subsequent CCTA was performed immediately (double flash protocol). The effective radiation dose was calculated for each patient. All images were evaluated by two independent observers for quality on a four-point scale with 1 being non-diagnostic image quality and 4 being excellent. Results Mean effective whole-body dose of CCTA was 1.6 ± 0.4 mSv (range, 0.4-5.4) for the entire cardiac examination and 0.9 ± 0.3 mSv (range, 0.4-2.8) for individual prospective ECG-triggered high-pitch CCTAs. In 27 of these patients with higher heart rates or occasional premature ventricular contractions or atrial fibrillation, subsequent CCTAs were performed immediately. The average image quality score was good to excellent with less than 1% unevaluable coronary segments. The double flash protocol resulted in a fully diagnostic CCTA in all cases. Conclusions The prospective ECG-triggered high-pitch CCTA technique is feasible and promising in clinical routine with good to excellent image quality and minimal radiation dose. The double flash protocol might become a more robust tool in patients with higher heart rates or arrhythmia.

Anatomical variants and coronary anomalies detected by dual-source coronary computed tomography angiography in North-eastern Thailand

Purpose Congenital coronary anomalies are uncommon, with an incidence ranging from 0.17% in autopsy cases to 1.2% in angiographically evaluated cases. The recent development of dual-source coronary computed tomography angiography (coronary CTA) allows accurate and noninvasive depiction of coronary artery anomalies. Material and methods A retrospective study included a total of 924 patients who underwent coronary CTA because of known or suspected coronary artery disease. In each study, coronary artery anomalies (CAs) were investigated. Results A total of 924 patients (mean age 51.2 ± 12.8 years), who underwent dual-source coronary CTA, were studied. The overall prevalence of CAs in our study was 3.7%, with the following distribution: four single coronary artery, 14 anomalous origin from opposite sinus of Valsalva, three absent left main, four high take-off coronary artery, three anomalous left coronary artery from pulmonary artery, and eight coronary artery fistulas. Conclusions The present study supports the use of coronary CTA as a reliable noninvasive tool for defining anomalous coronary arteries in an appropriate clinical setting and provides detailed three-dimensional anatomic information that may be difficult to obtain with invasive coronary angiography.

The value of dual-source multidetector-row computed tomography in determining pulmonary blood supply in patients with pulmonary atresia with ventricular septal defect

BACKGROUND Primary evaluation of patients with pulmonary atresia with ventricular septal defect (PA-VSD) traditionally relies upon echocardiography and conventional cardiac angiography (CCA). Cardiac angiography is considered the gold standard for delineation of anatomy in children with PA-VSD. Data comparing CCA and dual-source multidetector-row computed tomography angiography (MDCT) in PA-VSD patients is limited. The objective of this study was to test the hypothesis that MDCT is equivalent to CCA for anatomic delineation in these patients. MATERIALS AND METHODS Twenty-eight patients with PA-VSD underwent CCA and MDCT in close proximity to each other without interval therapy. A retrospective review of these 28 patients was performed. All MDCT data of pulmonary artery morphology, major aortopulmonary collateral arteries (MAPCAs) and type of blood supply (dual vs. single supply) were evaluated by blinded experts and results were compared with CCA. RESULTS Twenty-eight patients had adequate size right and left pulmonary arteries (21 confluent and 7 non-confluent). Seven patients had complete absence of native pulmonary artery and 3 patients had stenosis of distal branches of pulmonary arteries; all had MAPCAs from descending thoracic aorta and/or subclavian arteries. Sensitivity, specificity, positive and negative predictive value of MDCT for detecting confluent of pulmonary arteries, absence of native pulmonary artery and stenosis of pulmonary arteries were all 100%. Moreover, accuracy of detecting MAPCAs was excellent. CONCLUSIONS These results suggest that MDCT and CCA are equivalent in their ability to delineate pulmonary artery anatomy and MAPCAs. Dual source MDCT provides high diagnostic accuracy in evaluation of pulmonary blood supply in patients with PA-VSD and allows precise characterisation of the condition of pulmonary arteries and MAPCAs which is of paramount importance in managing patients with PA-VSD. (Folia Morphol 2018; 77, 1: 116-122).

Outcomes of symptomatic Brugada syndrome patients with implanted cardioverter defibrillators: A report from an endemic area

In Thailand and Southeast Asia, Brugada syndrome (BS) is a common inheritable cause of sudden cardiac arrest (SCA) due to polymorphic ventricular tachycardia (VT) and ventricular fibrillation (VF). Currently, an implantable cardioverter defibrillator (ICD) is the recommended therapy for high‐risk patients. This study aimed to report the outcomes of symptomatic BS patients with implanted ICD in an area in which BS is endemic.