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Introduction: This study aims to investigate the clinical characteristics of pediatric patients diagnosed with supraventricular tachycardia (SVT) and assess their shortterm prognosis. Materials and Methods: Data from 213 patients diagnosed with SVT between 2010 and 2015 at the Department of Pediatric Cardiology, Bursa Uludag University Faculty of Medicine, were retrospectively reviewed. Results: The ratio of male to female patients was 1: 1.25. Regarding treatment response and prognosis, the recurrence rate of SVT attacks was higher in males. The most common complaint at the time of diagnosis was palpitation. Furthermore, 17.8% of patients were diagnosed due to tachycardia detected during routine check-ups; almost all were under one year of age. In 56.3% of patients, the initial diagnosis was made at the pediatric emergency clinic. In acute treatment, 30% of patients were treated with vagal maneuvers; 61% had their attacks terminated, while 41% received drug therapy. Adenosine was the most used drug, and it successfully terminated attacks in 79% of patients receiving it. In the evaluation of patients' follow-ups after the initial attack, 56.3% of patients experienced recurrences, and 71% of second attacks occurred within the first three months. Prophylactic drug therapy was initiated in 94.4% of patients, with an average duration of 2.5±1.6 years. After treatment discontinuation, 75.4% of patients remained symptom-free, while 24.6% experienced SVT attacks again. Electrophysiological studies were performed in 16.9% of patients at another centers, and ablation was applied to 15.5%. Conclusion: Patients with SVT may be asymptomatic during infancy and vagal stimulation and adenosine response was quite good in acute treatment. Prophylactic medical treatment was effective in children whose weight is <15 kg and the recurrences were low after discontinuation especially in infants. [ABSTRACT FROM AUTHOR]

Introduction: Holter monitoring (HM) is usually used in patients with syncope when etiology cannot be explained with history, physical examination and electrocardiography (ECG). In this study the objective was to evaluate the diagnostic value of HM in children with syncope. Materials and Methods: Databases were collected retrospectively by analyzing the HM results of 3.122 pediatric patients between 2010-2014. Gender, age at diagnosis, detailed clinical history, physical examination, 12-lead electrocardiographic and echocardiographic results were noted using standardized form. Results: The study included 323 patients with syncope with a mean age of 13.21±3.67. There were 199 female and 124 male patients in this study. Among all patients 284 (87.9%) had normal HM results, while 11 (3.4%) patients had abnormal Holter studies that consider to explain as syncope. Three of 11 patients with abnormal Holter results diagnosed through ECG before HM, hence, the diagnostic value of HM was calculated as 2.4%. In contrast, diagnostic value of HM in patients with positive family history was found to be 16.6%. In this study, 7 patients were considered to have long QT syndrome according to their HM findings. Conclusions: Detailed clinical history has a great value in children with syncope. As a result, HM has low diagnostic value if the patients are not in high risk group. However, HM was considered to be important because of concealed long QT syndrome especially if the patient had positive family history and exercise related syncope. [ABSTRACT FROM AUTHOR]

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Introduction: Costello syndrome is probably an autosomal dominant inherited disorder that is characterized by postnatal growth retardation, developmental delay, coarse facies, loose skin, nonprogressive cardiomyopathy and friendly behavior. The aim of this study was to evaluate a variety of clinical findings and cardiac involvement of six patients diagnosed as Costello syndrome. Materials and Methods: Six cases that had a typical features of Costello syndrome admitted to pediatric cardiology department due to cardiac murmur between 2002-2013 years were evaluated. Their complaints, clinical findings, treatments and clinical courses were examined. Results: Three of the patients were male and the mean age of patients was 34±12 months (7 months to 11 years). All of the cases had coarse faces, deep palmar and plantar creases, hypertelorism, epicanthal folds, depressed nasal bridges and cardiac murmur at auscultation. Congenital heart disease was found in all patients. Five patients had pulmonary stenosis and balloon pulmonary valvuloplasty was performed in 3 of these patients. Atrial septal defect was detected in 3 patients and 1 of the patient had hypertrophic cardiomyopathy. Mental retardation was found in 2 patients. Conclusions: Costello syndrome must be keep in mind in all patients with psychomotor retardation, coarse faces, sparse hairs, deep palmar and plantar creases, phenotypical features resembling a storage disease especially in association with pulmonary stenosis and hypertrophic cardiomyopathy. [ABSTRACT FROM AUTHOR]