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.

The effect of a course of selected corrective exercises on posture, scapula-humeral rhythm and performance of adolescent volleyball players with upper cross syndrome.

BACKGROUND: This study aims to investigate the effect of a course of selected corrective exercises on posture, scapula-humeral rhythm and performance of adolescent volleyball players. METHODS: 30 adolescent volleyball players with upper cross syndrome were purposefully selected and assigned into 2 control and training groups. The degree of back curvature was evaluated using a flexible ruler, forward head and forward shoulder size by photographic method, scapula-humeral rhythm by Lateral Scapular Slide Test (LSST), and performance by closed kinetic chain test. The training group performed the exercises for 10 weeks. After the exercises, the post-test was administered. To analyze the data, analysis of co-variance tests and paired t-test at the level of 0.05 were employed. RESULTS: The research results showed that corrective exercises have a significant effect on abnormalities of forward head, forward shoulder, kyphosis, scapula-humeral rhythm and performance. CONCLUSIONS: Corrective exercises can be effective in reducing shoulder girdle and spine abnormalities and improving scapula- humeral rhythm and performance of volleyball players.

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.

Endoscopic Assessment of the Isthmus Tympanicum and Tensor Tympani Fold and their Relationship with Mastoid Pneumatization in Chronic Otitis Media.

OBJECTIVES: To endoscopically evaluate the patency of the isthmus tympanicum and integrity of the tensor tympani fold as routes of ventilation of the attic and mastoid in chronic otitis media (COM) and to assess their effects on mastoid pneumatization. MATERIALS AND METHODS: Sixty patients with COM were categorized into two groups: (1) Group A: 36 patients with tympanic membrane perforation (2) Group B: 24 patients with limited attic disease of whom 14 patients had attic retraction pockets and 10 with limited attic cholesteatoma. A multislice computed tomography scan of the temporal bone was performed for each patient to assess the degree of mastoid pneumatization. Notably, either myringoplasty or tympanomastoid surgery was performed in all patients. An endoscope was inserted into the middle ear for evaluation of the isthmus tympanicum and tensor fold area. RESULTS: The isthmus tympanicum was patent in most ears (83.3%) of group A, whereas it was blocked in most ears (83.3%) of group B. The tensor fold was complete in 77.8% of ears in group A and 83.3% of ears in group B. It was observed that 94.1% of ears with patent isthmus in both groups had normal mastoid pneumatization and 5.9% of ears had poorly pneumatized mastoid. By contrast, 7.7% of ears with blocked isthmus tympanicum had normal mastoid pneumatization and 92.3% had poor mastoid pneumatization. Normal mastoid pneumatization was observed in 50% of ears in both groups with complete tensor fold, and 83.3% of ears with an incomplete tensor fold. CONCLUSION: A significant correlation was observed between COM with limited attic disease and obstruction of the isthmus tympanicum. Obstruction of isthmus tympanicum was associated with poor mastoid pneumatization. Furthermore, an incomplete tensor fold was associated with well pneumatized mastoid.

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.

Safety threshold of intravitreal clonidine in rabbit's eyes.

AIM: To determine the safe dose of intravitreal clonidine (IVC), a potential drug for neuroprotection and angiogenesis inhibition in rabbits. METHODS: A total of 28 rabbits were divided into four groups. Three groups received IVC with concentrations of 15 (Group A), 25 (Group B), and 50 (Group C) µg/0.1 mL and the control group (Group D) received 0.1 mL balanced salt solution (BSS). To investigate IVC safety, electroretinography (ERG) was performed at baseline, then at 1, 4 and 8wk after injection. After last ERG, all rabbits were euthanized, their eyes were enucleated and subjected to routine histopathological evaluation, immunohistochemistry for glial fibrillary acidic protein (GFAP) and terminal deoxynucleotidyl transferase dUTP nick end labeling (TUNEL) test. RESULTS: Based on ERG, histopathology, GFAP and TUNEL assay findings, 15 µg IVC was determined as the safe dose in rabbit eyes. While, the results of routine histopathology and TUNEL assay were unremarkable in all groups, toxic effects attributed to 25 and 50 µg IVC were demonstrated by ERG and GFAP tests. CONCLUSION: Totally 15 µg clonidine is determined as the safe dose for intravitreal injection in rabbits. Contribution of IVC in neuroprotection and inhibition of angiogenesis deserve more studies.

Intraoperative Three-Dimensional Mapping of Supraventricular Tachycardia in a Young Fontan Patient.

Preoperative tachycardia is uncommon before an initial Fontan procedure. Catheter intervention can risk complications in small patients. A patient with fetal tachycardia and double inlet left ventricle underwent Norwood and bidirectional Glenn procedures. She had persistent tachycardia. A three-dimensional electroanatomic mapping system was modified for the operative procedure; it was used to identify the arrhythmia substrate and cryoablate an atrioventricular nodal tachycardia. Mapping added 20 minutes to the operation. She is arrhythmia-free and off medication after 4 months. This three-dimensional system was successfully adapted for an intraoperative SVT ablation in this small patient, and it may be useful in other settings.

Left main coronary artery stenting in a 3.6 kg infant after arterial switch operation for transposition of the great arteries.

The patient presented with flash pulmonary edema related to severe left ventricular diastolic dysfunction several weeks after arterial switch operation (ASO) for D-transposition of the great arteries. Long segment, critical left main coronary artery stenosis in this 3.6 kilogram infant was successfully stented and resulted in resolution of the clinical findings. At 15-month follow-up, the patient remains asymptomatic and thriving.

Bronchoscopic guidance of endovascular stenting limits airway compression.

OBJECTIVE: Bronchial compression as a result of pulmonary artery and aortic arch stenting may cause significant respiratory distress. We set out to limit airway narrowing by endovascular stenting, by using simultaneous flexible bronchoscopy and graduated balloon stent dilatation, or balloon angioplasty to determine maximum safe stent diameter. METHODS: Between August 2010 and August 2013, patients with suspected airway compression by adjacent vascular structures, underwent CT or a 3D rotational angiogram to evaluate the relationship between the airway and the blood vessels. If these studies showed close proximity of the stenosed vessel and the airway, simultaneous bronchoscopy and graduated stent re-dilation or graduated balloon angioplasty were performed. RESULTS: Five simultaneous bronchoscopy and interventional catheterization procedures were performed in four patients. Median age/weight was 33 (range 9-49) months and 14 (range 7.6-24) kg, respectively. Three had hypoplastic left heart syndrome, and one had coarctation of the aorta (CoA). All had confirmed or suspected left main stem bronchial compression. In three procedures, serial balloon dilatation of a previously placed stent in the CoA was performed and bronchoscopy was used to determine the safest largest diameter. In the other two procedures, balloon testing with simultaneous bronchoscopy was performed to determine the stent size that would limit compression of the adjacent airway. In all cases, simultaneous bronchoscopy allowed selection of an ideal caliber of the stent that optimized vessel diameter while minimizing compression of the adjacent airway. CONCLUSION: In cases at risk for airway compromise, flexible bronchoscopy is a useful tool to guide endovascular stenting. Maximum safe stent diameter can be determined without risking catastrophic airway compression.

Intravitreal Topotecan Inhibits Laser-induced Choroidal Neovascularization in a Rat Model.

PURPOSE: A two-phase preclinical study was designed to determine the safe dose of intravitreal topotecan and its inhibitory effect on experimental choroidal neovascularization (CNV) in a rat model. METHODS: In phase I, 42 rats were categorized into 6 groups, 5 of which received intravitreal topotecan injections of 0.125 µg, 0.25 µg, 0.5 µg, 0.75 µg, and 1.0 µg/5 µl, respectively; the control group received an injection of normal saline. Ophthalmic examination and electroretinography (ERG) were performed on days 7 and 28, and enucleated globes were processed for histopathology and immunostaining for glial fibrillary acidic protein. In phase II, CNV was induced via laser burns in 20 rats and the animals were divided into 2 groups. One group received topotecan and the other received normal saline intravitreally. Four weeks later, mean scores of fluorescein leakage on fluorescein angiography as well as mean CNV areas on histology sections were compared. RESULTS: In phase I, clinical, ERG and histopathologic results were unremarkable in terms of retinal toxicity in all groups. Based on the results of phase I, a dose of 1 µg/5 µl topotecan was chosen for phase II. Leakage scores obtained from late-phase fluorescein angiography were significantly lower in topotecan-treated than control eyes (P < 0.01) four weeks after induction of CNV. Compared to control eyes, topotecan-treated eyes showed a significantly lower incidence of fibrovascular proliferation (8.7% vs. 96.2%) and significantly smaller areas of CNV (P < 0.01). CONCLUSION: Intravitreal injection of topotecan at a dose of 1 µg/5 µl is safe and may be a promising treatment for CNV.

Wavefront - guided laser - assisted subepithelial keratectomy in low myopia, myopic astigmatism and high myopia / 国际眼科杂志(Guoji Yanke Zazhi)

To compare the safety, efficacy, predictability, stability and complications of wavefront-guided laser-assisted subepithelial keratectomy ( LASEK ) in low myopia, myopic astigmatism and high myopia correction.METHODS: A retrospective analysis of 416 eyes were assigned to 3 groups:159 eyes with low myopia ( LM) and mean refractive spherical equivalent (MRSE) of-3. 68±1. 33 dioptre ( D ); 161 eyes with myopic astigmatism ( MA ) and MRSE of -5. 99 ± 2. 24D and mean cylinder of 2. 41 ± 1. 07D;and 96 eyes with high myopia (HM) and MRSE of- 7. 41 ± 0. 80D. After an epithelial flap creation, a wavefront-based excimer laser ablation was performed. Safety, efficacy, predictability and stability were evaluated at day 10, 2, 6 and 12mo postoperatively.RESULTS:At 12mo, the MRSE was -0. 36 ± 0. 31D in LM group, 0. 15 ± 0. 41D in MA group and 0. 58 ± 0. 68D in HM group. The uncorrected visual acuity ( UCVA) was 20/20 in 90. 60% of patients in LM group, 78. 90% in MA group and 67% in HM group. Efficacy indices were 0. 98, 1. 04 and 0. 92 in LM, MA and HM groups, respectively. Safety indices were 1. 00, 1. 07 and 1. 05 in LM, MA and HM respectively. Five eyes (3. 1%) in the LM group gained 1 line. Forty-four eyes (27. 3%) in MA gained 1-3 lines and eighteen eyes (19. 2%) of HM group gained 1-2 lines of BSCVA. Only 2 eyes in LM group developed corneal haze. There were not statistically significant differences in efficacy and safety indices amongst three groups.CONCLUSlON:Wavefront-guided LASEK is an effective and safe procedure for the treatment of LM, MA, and HM. although in myopic astigmatism the predictability, efficacy and safety indices had been better.