Pediatric and Familial Genetic Arrhythmia Syndromes: Evaluation of Bidirectional Ventricular Tachycardia-Differential Diagnosis.

Bidirectional ventricular tachycardia is a unique arrhythmia that can herald lethal arrhythmia syndromes. Using cases based on real patient stories, this article examines 3 different presentations to help clinicians learn the differential diagnosis associated with this condition. Each associated genetic disorder will be briefly discussed, and valuable tips for distinguishing them from each other will be provided.

Trans-2,3-enoyl-CoA reductase-like-related catecholaminergic polymorphic ventricular tachycardia with regular ventricular tachycardia and response to flecainide.

INTRODUCTION: We describe a unique case of TECRL-CPVT presented with cardiac arrest. METHODS: Post resuscitation, the patient developed regular ventricular tachycardia featuring a left purkinje system morphology. RESULTS: There was clear suppression of arrhythmia with the addition of flecainide and isolated ventricular ectopy causing secondary T-wave changes. CONCLUSION: A high index of suspicion was required to eventually make the diagnosis through whole exome sequencing.

Surgical Outcomes of Congenital Heart Disease in Down Syndrome: Tertiary Center Experience-Focus on the Electrical Conduction System.

To document outcomes of cardiac surgical repair in Down syndrome (DS) patients with specific focus on the associated electrical conduction morbidities, ultimately leading to a higher incidence of pacemaker implantation (PMI). A retrospective study conducted between 2011 and 2020. A total of 167 DS patients undergoing 204 surgeries were included. The mean gestational age (GA) and mean weight were 37.3 weeks and 5.5 kg, respectively. Complete atrioventricular septal defect (AVSD) was the most common diagnosis. Pre-operative ECG revealed superior axis deviation (SAD) in 92 and 32% of patients with AVSD and isolated perimembranous ventricular septal defect (VSD), respectively (p < 0.01). Postoperative right bundle branch block (RBBB) was observed in 83 and 55% of patients with AVSD and following perimembranous VSD repair, respectively (p = 0.04). Ten patients underwent post-operative pacemaker implantation (PMI). Reintervention rate was around 8.9%. Three mortalities were encountered throughout the study period, 2 of which were in-hospital deaths. Low mortality was observed, however, a higher rate of PMI requirements noted with risk factors including lower age and weight.

A Comparative Study Utilizing the Voice Handicap Index-10 (VHI-10) in Teachers and the General Population of Kuwait.

OBJECTIVES: The objective of this study was to determine the prevalence of voice handicap perception of teachers in Kuwait and compare them within the general population by using the validated Arabic version of the voice handicap index-10 (VHI-10). Moreover, we explored variations within the teaching profession itself with regards to specific characteristics. METHODS: The study was a cross-sectional survey design, in which participants from all governates of Kuwait (1820 teachers and 755 controls) completed the validated Arabic VHI-10, health-related, and demographic questions. The questionnaires were distributed among a random sample of elementary, middle, and high school teachers of both genders, and a random sample of the control population. RESULTS: The mean VHI-10 of teachers was significantly higher than nonteachers (mean VHI-10 of teachers = 5.7, mean VHI-10 of nonteachers = 3.7, P < 0.001). Furthermore, there was a significant difference between teachers and controls in those who scored >11 in the VHI-10 (>11 = 17.6%, 10.2% respectively, P = <0.001). Female teachers scored a higher mean VHI-10 than male teachers (B = 0.66, P < 0.001). There was no difference in the mean VHI-10 among the different teaching class levels. However, elementary school teachers were the most group to exceed the cut-off point (VHI-10 >11) (Odds Ratio = 1.38, P = 0.04). With regards to smoking, we found no difference in the mean VHI-10 and scoring >11 in the questionnaire. Art and science teachers had the highest mean VHI-10 (P = 0.005 and 0.03, respectively). CONCLUSION: The results of the study revealed a higher perception of voice handicap in teachers than nonteachers. Therefore, emphasis should be on teachers in order to prevent voice damage.

Electrocardiogram interpretation among pediatricians: Assessing knowledge, attitudes, and practice.

OBJECTIVES: This study assesses the competency of pediatricians in interpreting electrocardiograms (ECGs). METHODS: A cross-sectional study involving 125 pediatricians comprised of 71 general pediatricians, 15 pediatric cardiologists, and 39 other subspecialists recruited from all public hospitals and two specialty centers. Participants completed a questionnaire that included 10 ECGs and questions regarding backgrounds, attitudes, and practices. The ECGs were graded to obtain a knowledge score out of 30 points. Mann-Whitney U test and Kruskal-Wallis test with post hoc analysis and Bonferroni adjustment were used to compare groups. RESULTS: The mean knowledge score ranged from 47.7% to 69.7% among various pediatric specialties (P = 0.006). Age, increasing years of experience, confidence level, number of cardiology referrals, and perceived importance of having good ECG interpretation skills were significantly related to the knowledge score (P ≤ 0.05). Accuracy was highest in identifying normal ECGs (76.8%), supraventricular tachycardia (64.8%), along with long QT interval (58.4%), and was lowest for right bundle branch block (RBBB) (10.4%), 2:1 atrioventricular conduction (10.4%), and atrial tachycardia (AT) (4.8%). Accuracy among pediatric cardiologists was highest for long QT interval (100%), normal ECG (80%), as well as Wolff-Parkinson-White syndrome (80%), and lowest for RBBB (13.3%) and AT (0%). Most pediatricians believe that ECGs are "useful" (78.4%) and that having good interpretation skill is "important" (80.6%). CONCLUSIONS: Pediatricians recognize the importance of ECGs. However, their skill and level of accuracy at interpretation is suboptimal, including cardiologists, and may affect patient care. Thus, efforts should be made to improve ECG understanding to provide better service to patients.

Pacemaker implantation post congenital heart disease surgical repair: tertiary center experience.

This was a retrospective study documenting all pacemaker implantations (PMIs) secondary to postoperative atrioventricular block. A total of 26 patients were included between 2011 and 2020. The incidence rate was 1.8%, with a median follow-up time of 4.5 years. At the time of the initial PMI, the median weight was 5 kg, and the median generator longevity was 45 months. Mean cardiopulmonary bypass and aortic clamp times were significantly longer among surgeries complicated with PMI (P≤ 0.05). Trisomy 21 patients were 4 times more likely to need a PMI (95% CI 1.8-9, P < 0.001). The mean Risk Adjustment in Congenital Heart Surgery and Society of Thoracic Surgery scores were higher in patients with PMI. All initial PMIs were epicardial (18 single chamber). Most patients underwent ventricular septal defect closure (isolated or complex), except for 5 patients who underwent left-sided surgery. Pacing-induced dilated cardiomyopathy occurred in 3 patients. All implanted leads were functional except for 2 leads with high thresholds and another biventricular system infection. There was a 31% rate of pacing reintervention.Conclusion: PMI resulted in significant morbidity but without mortality. The highest risk for PMI was left ventricular outflow tract repair, trisomy 21, prolonged cardiopulmonary bypass, and aortic cross times. What is Known: •Incidence rate for postoperative atrioventricular block requiring pacemaker was at 1.8%, similar to previously published reports. •Longer cardiopulmonary bypass and aortic cross-clamp times were associated with higher risk for developing postoperative persistent atrioventricular block. What is New: •Incidence for persistent atrioventricular block requiring pacemaker was highest among left ventricular outflow tract surgery at 8.6%. •Following all intracardiac repair, Down syndrome patients were 4 times more likely to need a pacemaker implantation compared to the non-syndromic group.

Insights on Atrial Fibrillation in Congenital Heart Disease.

Patients with congenital heart disease (CHD) have been surviving late into adulthood, with atrial arrhythmias being the most common long-term complication. In recent reports, atrial fibrillation (AF) tended to be the most common form of arrhythmias among groups of patients with adult CHD (ACHD) older than 50 years of age. When compared with their adult counterparts without CHD, AF in patients with ACHD has been characterized by a higher incidence and prevalence, younger age of onset, and a greater risk of progression to persistent AF. Risk factors for the development of AF are not well known but include older age, left atrial dilation, systemic hypertension, and multiple cardiac surgeries. Data on management options such as optimal antiarrhythmic drug therapy, indications for anticoagulation, and efficacy and safety of catheter ablation are limited. There is a crucial need for further research exploring management, prevention, and monitoring strategies for the growing ACHD patient population with AF. This report will provide a contemporary review of the epidemiology, pathophysiology, and management options for AF in this complex patient population.