Predicting arrhythmic event score in Brugada syndrome: Worldwide pooled analysis with internal and external validation.

BACKGROUND: Brugada syndrome is an inherited arrhythmic disease associated with major arrhythmic events (MAE). Risk predictive scores were previously developed with various performances. OBJECTIVE: The purpose of this study was to create a novel score-Predicting Arrhythmic evenT (PAT)-with internal and external validation. METHODS: A systematic review was performed to identify risk factors for MAE. The odds ratios (ORs) of each factor were pooled across studies. The PAT scoring scheme was developed based on pooled ORs. The PAT score was internally validated with published 105 Asian patients (follow-up 8.0 ± 4.1 [SD] years) and externally validated with unpublished 164 multiracial patients (82.3% White, 14.6% Asian, 3.2% Black; mean follow-up 8.0 ± 6.9 years) with Brugada syndrome. Performances were assessed and compared with previous scores using receiver operating characteristic curve (ROC) analysis. RESULTS: Sixty-seven studies published between 2002 and 2022 from 26 countries (7358 patients) were included. Pooled ORs were estimated, indicating that 15 of 23 risk factors were significant. The PAT score was then developed accordingly. The PAT score had significantly better discrimination (ROC 0.9671) than the BRUGADA-RISK score (ROC 0.7210; P = .006), Shanghai Score System (ROC 0.7079; P = .003), and Sieira et al score (ROC 0.8174; P = .026) in an external validation cohort. PAT score ≥ 10 predicted the first MAE with 95.5% sensitivity and 89.1% specificity (ROC 0.9460) and the recurrent MAE (ROC 0.7061) with 15.4% sensitivity and 93.3% specificity. CONCLUSION: The PAT score was shown to be useful in predicting MAE for primary prevention in patients with Brugada syndrome.

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.

Modified mallampati classification in determining the success of unsedated transesophageal echocardiography procedure in patients with heart disease: simple but efficient.

BACKGROUND: The transesophageal echocardiograhpy (TEE) has been studied worldwide. However, identifying additional factors on top of operator's experience and patient's cooperation which could influence the success of the procedure in unsedated patients with heart disease  is not well documented. METHODS: Under the cross-sectional descriptive design, 85 target patients were fulfilling the criteria: being Thai national at the age of at least 20-year-old, being performed TEE by the study participant's cardiologists, being able to communicate verbally. Seven outcomes were recorded, including gag reflex, insertion attempt, insertion time, vital signs (heart rate, oxygen saturation and mean arterial blood pressure), visible blood on TEE probe tip, and oropharyngeal pain at 1 h and 24-h. RESULTS: There were 85 eligible patients during June 2013 to June 2014 [corrected]. The major participants were male (46, 54 %) and the mean age was 51.2 ± 12.5 years. The MMC class III was mostly found (33, 38.80 %). TEE probe insertion time and gag reflex were indicated statistical significance (P < 0.05). Linear regression revealed that MMC class III (b 3.718; SD ± 1.077; P = 0.001) and class IV (b 5.15; SD ± 1.286; P = 0.000) were statistically associated with TEE probe insertion time, whereas MMC class II was no statistically significant (b 2.348; SD ± 1.405; P = 0.099) according to constant value in MMC class I (5.318 s). Similarly, logistic regression indicated that the patients with high grade MMC were more likely to have gagging than the low grade MMC patients (MMC 2 OR 0.567, 95 % CI 0.09-3.42, P = 0.536; MMC 3 OR 5.231, 95 % CI 1.55-17.67, P = 0.008; MMC 4 OR 3.4, 95 % CI 0.84-13.76, P = 0.086). CONCLUSIONS: Modified Mallampati Classification is one of determining factors in the success of unsedated TEE procedure in patients with heart disease, especially for assessment of gagging and successful TEE probe insertion time.

Fragmented QRS as a Predictor of Appropriate Implantable Cardioverter-defibrillator Therapy.

BACKGROUND: Fragmented QRS (fQRS) has been shown to be a marker of local myocardial conduction abnormalities and a predictor of cardiac events in selected populations. We hypothesized that the presence of a fQRS might predict arrhythmic events in patients who received an implantable cardioverter-defibrillator (ICD), regardless of the indications for implantation. METHODS AND RESULTS: A cohort of 107 consecutive patients (mean age, 53 years; 82% male) who underwent an ICD implantation was studied. We defined fQRS, on a routine 12-lead ECG, as the presence of an additional R wave or notching in the nadir of the S wave in 2 consecutive leads corresponding to a major coronary artery territory. In the presence of bundle branch block, more than 2 notches in the R or S waves in 2 consecutive leads were required to characterize fQRS. Patients were followed for 21.3±23 months for appropriate ICD therapy (antitachycardia pacing and/or shock). ICDs were implanted predominantly in patients with ischemic cardiomyopathy (N=45, 42.1%), followed by Brugada syndrome (N=26, 24.3%). fQRS presented in 42 patients (39.3%). During follow-up, patients with fQRS received more appropriate ICD therapy than those without fQRS (45.2% vs. 10.8%, P<0.0001). After adjustment for covariates, fQRS remained an independent predictor for appropriate ICD therapy (hazard ratio=5.32, 95% confidence interval=2.11-13.37, P<0.0001). CONCLUSION: The presence of fQRS appeared to be directly associated with appropriate ICD therapy.

Cardiac troponin-T in pre-end stage kidney disease.

BACKGROUND: Cardiac troponin T level (cTnT) is commonly increased in end stage chronic kidney disease (CKD) in the absence of acute myocardial infarction. There are few data available on serum cTnT concentration in patients with pre-end stage CKD. OBJECTIVE: To evaluate the correlation of cTnT level and severity of kidney disease in patients with CKD stage 3 and 4 and to evaluate whether there is a relationship between left ventricular mass index and cTnT level. MATERIAL AND METHOD: Patients (103) with CKD stage 3-4 between 26 and 85 years of age (mean 60.0 +/- 11.9) entered the present study. Serum cTnT determined using a third-generation electrochemiluminescent immunoassay on an Elecsys 2010 analyzer (Roche Diagnostics Ltd.). All patients underwent echocardiography. Left ventricular hypertrophy (LVH) was considered when LV mass index exceeded 125 g/m2. RESULTS: Overall, 28 patients (28.2%) had cTnT > 0.01 micro/L and two patients (1.8%) had cTnT > 0.1 microg/L cTnT concentration was commonly increased in more severe CKD (9 patients in stage 3 and 20 patients in stage 4). LVH was not associated with increased cTnT (p=0.105). CONCLUSION: The present study demonstrated that the elevated cTnT > 0.01 microg/L is relatively common in patients with CKD stage 3-4 who do not require dialysis treatment, however serum cTnT level above > 0.1 microg/L is uncommon in this population. Increased serum cTnT is associated with decreased renal clearance but not LVH.