Search

Introduction: Williams syndrome (WS) cases have been reported to have with 25–100 times greater increased risk of sudden cardiac death (SCD). SCD has been reported in cases without any evidence of structural cardiovascular anomalies. Wolff–Parkinson–White (WPW) syndrome is characterized by short PR interval and delta wave. Ventricular preexcitations can develop paroxysmal reentrant tachycardia through Kent bundle or less frequent atrial fibrillation and in some cases with accessory pathway effective refractory period (APERP) under 250 ms considered as risky and may lead to SCD. WS associated with WPW has not been reported before. Case Report: An 11‐year‐old male who had been followed up with WS was referred to pediatric cardiology outpatient clinic with the complaint of palpitation. Electrocardiographic examination showed short PR interval and delta wave in the ECG consistent with WPW. He underwent electrophysiological study (EPS). Basic measurements were performed, and APERP was found at 280 ms cycle atrial pacing. RF energy was delivered using a 4 mm tip nonirrigated radiofrequency (RF) ablation catheter where the best ventriculoatrial (VA) signals were received and the AP was abolished within few seconds. Discussion and Conclusions: Although, WPW cases are usually asymptomatic or related to SVT, the risk of SCD should not be ignored. Thus, all patients with WPW deserve an EPS for assessing the AP conduction properties. Due to the increased risk of SCD in patients with WS compared to general population, in the presence of concomitant WPW, these patients should be evaluated with EPS even if they do not have symptoms. [ABSTRACT FROM AUTHOR]

Objective: Kawasaki Disease (KD) is a type of systemic vasculitis mediated by an abnormal immune response, similar to autoinflammatory disorders, due to increased release of proinflammatory cytokines. This cross-sectional study aims to evaluate the relationship between the frequency and severity of KD and the presence of Mediterranean Fever (MEFV) gene variants. Materials and Methods: Thirty-eight children diagnosed with KD were included in the study. Demographic and clinical data such as age at diagnosis, gender, and duration of fever prior to intravenous immunoglobulin (IVIG) treatment were recorded. Laboratory and echocardiographic data at the time of diagnosis were also retrospectively reviewed. MEFV gene analysis was performed for all patients. Results: The study comprised 38 patients, including 25 boys (65.8%) and 13 girls (34.2%), with a median age of 3.4 years (range 1.8-6.3 years). Coronary arterial lesions were observed in 11 patients (28.9%), and 13 patients (34.2%) were resistant to initial IVIG treatment. Twenty-two patients (57.9%) had at least one allele with MEFV variants. Of these, eight patients (36% of MEFV-positive patients) had at least one exon-10 variant, while 14 patients had only exon-2 variants. No statistically significant differences were found in clinical, demographic, or echocardiographic parameters between MEFV-positive and MEFV-negative patients. Conclusion: This study found that the presence of MEFV gene variants in KD patients is almost three times higher compared to the general Turkish population. This relationship warrants further clarification through nationwide studies, especially in countries where Familial Mediterranean Fever (FMF) is a significant concern. [ABSTRACT FROM AUTHOR]

Objectives: In this study, a comparison of cardiac magnetic resonance imaging (MRI) and simultaneous transthoracic echocardiography data were obtained from patients with great artery transposition (TGA), who underwent arterial switch operation (ASO) surgery. In to discover the most effective and optimal viewing method during long-term follow-ups. Materials and Methods: This retrospective cohort included 20 TGA patients (16 male, 4 female), which had ASO surgery. Along with cardiac MRI and transthoracic echocardiography data were obtained from the images. The mean age was 93.00±29.82 months (60-144). Seventeen patients had TGA only. However, 3 patients with TGA included an existing ventricular septal defect. Results: We showed a meaningful correlation between echocardiographic variables (left ventricle dilatation of the left ventricle function of the aorta failure, myocardial performance index) and MRI parameters (ejection fraction of left ventricle dilatation of the left ventricle function of the aorta insufficiency). Conclusion: TGA patients require careful pre and post operative evaluation, anatomical and a functional use of cardiac indicators. For this purpose, both echocardiography and MRI are useful, safe, and trustworthy methods of diagnosis. Choosing the optimal imaging technique and lifetime reoccurring assessments of the left ventricle function is of vital importance in foreseeing complications, preventing morbidities, and creating a protocol. In clinical practice, the myocardium performance index provides values similar to that of the MRI about the left ventricle dilatation and left ventricle dysfunction. These results provide awareness about the use of specific parameters and the use of information based on quantitative data. [ABSTRACT FROM AUTHOR]

Ventricular septal defect (VSD) is the most common congenital cardiac lesion. However, acquired VSDs are rare and typically associated with blunt chest injuries. The clinical symptoms and timing of presentation can vary, making the diagnosis difficult and sometimes delayed. Closure of traumatic VSDs depends on factors such as heart failure symptoms, hemodynamics, and the size of the defect. Percutaneous closure has emerged as a viable alternative to surgical repair. In this case report, we present the case of a 3-year-old girl who presented with a traumatic ventricular septal defect (VSD) after being struck by a minibus. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Turkish Society for Rheumatology is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)