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Copyright of Ümraniye Pediatri Dergisi is the property of KARE Publishing 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.)

The article reports that the isolated congenital complete atrioventricular block is an uncommon disease linked to SSA/Ro and SSB/La autoantibodies that come from the mother. Topics include examines several patients are diagnosed prenatally or in the first month have a congenital heart condition and the diagnosis is considered to be childhood CAVB.

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 presents a case study of a 12-year-old male patient with a murmur and pectus carinatum and dysmorphic features of dolichocephaly, palpebral fissures and scoliosis. It mentions a computed tomography evaluation which revealed aneurysmatic dilatation from the aortic root to the right truncal brachiocephalic branch. It also states detection of mutation of heterozygous missense in the TGFBR2 gene.

Objective: Extracorporeal membrane oxygenation (ECMO) is a lifesaving intervention for pediatric patients with respiratory and/or cardiovascular failure. In this study, we evaluated the cardiac catheterization results of pediatric patients on ECMO support. Methods: Between January 2012 and October 2016, 98 patients (5.2% of all surgery patients) needed ECMO support during perioperative cardiac surgery. We retrospectively reviewed the clinical data of 16 patients who underwent cardiac catheterization under ECMO support. Results: The median age at catheterization was 6.5 months (range, 3.3-60 months), and the median weight was 6.0 kg (range, 3.7-16 kg). Eight of the catheterizations were diagnostic, and the remaining eight were interventional. Five out of these eight patients underwent surgical palliation after diagnostic catheterization. Right pulmonary artery (RPA) stenting, right ventricular outflow tract (RVOT) stenting, combined left pulmonary artery (LPA) and RVOT stenting, combined LPA and modified Blalock-Taussig shunt stenting, bilateral pulmonary artery balloon angioplasty, and bilateral pulmonary artery stenting were each performed once, whereas LPA stenting was performed in two different patients. In one patient undergoing RVOT stenting, a complete atrioventricular block developed, resulting in hypotension; however, this was overcome with an ECMO flow increase. In another patient, the ECMO tubing disconnected from the arterial line. Minor vascular complications were seen in three patients. Twelve patients (75%) were successfully weaned from ECMO after the procedure and ten (63%) were discharged. Conclusion: Diagnostic and interventional cardiac catheterization can be safely and effectively performed in patients on ECMO. If the patient cannot be weaned from ECMO support, clinicians should consider performing an early angiogram either to treat or clarify the underlying problem. [ABSTRACT FROM AUTHOR]

Copyright of Archives of the Turkish Society of Cardiology / Türk Kardiyoloji Derneği Arşivi is the property of KARE Publishing 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.)

The article presents a case study of a 06-month-old boy with recoarctation of aorta (CoA). Topics include two-dimensional transthoracic echocardiography revealing severe aortic regurgitation and aortic cusp prolapsus; and computed tomography angiography (CTA) showing multiple aneurysms in different parts of the ascending and descending aorta.

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]

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]

The article presents a case stud on the hybrid transcatheter pulmonary valve implantation (PVI). It highlights the case of three patients who had severe pulmonary valve regurgitation (PVR) after the transannular patch repaired of tetralogy of Fallot (TOF). It also provides discussion of the result which indicates that hybrid PVI plays a significant role in patients who are not suitable for percutaneous PVI.

The article presents the case study of 4.5-month-old boy who underwent hybrid Norwood stage 1, bilateral pulmonary artery banding and ductal stenting with the diagnosis of hypoplastic left heart syndrome (HLHS) who underwent stent implantation into the atrial septum.