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Objective: Long QT syndrome (LQTS) is an inherited primary arrhythmia syndrome associated with life-threatening ventricular arrhythmias and sudden death. This study aimed to report the clinical and genetic characteristics and outcomes of children diagnosed as having LQTS in a tertiary pediatric cardiology center in Turkey. Methods: This was a retrospective review of pediatric patients diagnosed as having LQTS at our center from January 2011 to April 2020. Results: A total of 145 patients (76 males) were included, with a mean age of 9.2±4.5 years and a mean weight of 35.7±18.5 kg; 38 (26.2%) were identified as having LQTS during family screening, whereas a significant proportion of patients were asymptomatic at presentation, 15 patients (10.3%) were diagnosed after previous cardiac arrest, and 26 patients (18%) had syncope. The mean Schwartz score was 4.5 points (range, 3-7.5 points). Furthermore, 107 patients (82%) were confirmed to have a pathogenic mutation for LQTS genes. All patients received beta-blockers. Implantable cardioverter-defibrillator insertion was performed in 34 patients (23.4%). Left or bilateral cardiac sympathetic denervation was performed in 9 patients (6.2%). Median follow-up time was 35.6±25.8 months. Five (3.4%) patients died during the follow-up. Statistical analyses of risk factors for major cardiac events revealed that the QTc was >500 ms and that T wave alternans, high Schwartz score, and Jervell and Lange-Nielsen syndrome were strong and significant predictors of cardiac events. Conclusion: LQTS has a variety of clinical manifestations. Patients' symptoms ranged between asymptomatic and sudden cardiac death (SCD). By raising the awareness of physicians regarding the disease, SCD might be prevented in the early period. [ABSTRACT FROM AUTHOR]

The article focuses on an approach which seeks to expand the use of pediatric extracorporeal life support (ECLS) systems after open heart surgery in Turkey. It provides an overview of the venoarterial ECLS and venovenous ECLS, which is widely used in pediatric intensive care units (ICU). Information regarding the ECLS outcomes from pediatric cardiac centers and pediatric ICUs is provided.

Extracorporeal life support (ECLS) is instituted for the management of life threatening pulmonary or cardiac failure (or both), when no other form of treatment has been or is likely to be successful. It has being used in developed countries for many years. ECLS has come into practice for a while in Turkey, in order to ECLS recommendations. However, most of them are postcardiotomy cases. However, this therapeutic option has more indications including its reason that brought it into practice. All equipment of the system has being changed with the developing technology. The aim of this paper is to point out this modality, to introduce equipment, and to give information about organization and management to bring this therapeutic option in Turkey in order. [ABSTRACT FROM AUTHOR]

Information about several papers discussed at a symposium held December 17, 2011 at the American Hospital in Istanbul, Turkey on the topic minimizing adverse effects of cardiopulmonary bypass procedures in neonates and pediatric cardiac patients is presented. Topics includes development of pediatric cardiac surgery, neonatal and pediatric cardiopulmonary bypass (CPB) and latest techniques and circuits for pediatric cardiac surgery.

Extracorporeal life support (ECLS) is instituted for the management of life threatening pulmonary or cardiac failure (or both), when no other form of treatment has been or is likely to be successful. It has being used in developed countries for many years. ECLS has come into practice for a while in Turkey, in order to ECLS recommendations. However, most of them are postcardiotomy cases. However, this therapeutic option has more indications including its reason that brought it into practice. All equipment of the system has being changed with the developing technology. The aim of this paper is to point out this modality, to introduce equipment, and to give information about organization and management to bring this therapeutic option in Turkey in order.