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Karagöz salnamesi, dördüncü sene (1331 / 1329 / 1913)

Eserde "Marmara Üniversitesi Fen-Edebiyat Fakültesi Kütüphanesi" Mührü Vardır

Endovascular stent implantation for coarctation of the aorta in children and young adults: intermediate follow-up results from Turkey

The aim of this study was to report the intermediate follow-up results of stent implantation for coarctation of the aorta in children from Turkey. Patients with native or postoperative coarctation, who had abnormal flow at the descending aorta exceeding diastolic phase on Doppler echocardiography or upper extremity hypertension, underwent endovascular stent implantation. Ten patients with a mean age of 12.4 +/- 5.5 years (6-23 years) underwent stent implantation between January 2001 and September 2006. Of these, three had previous surgical repair, and the remainder were native coarctation. Palmaz, Genesis and Numed CP stents were used. All the implantations were successful and there was no major complication during the procedure. The follow-up period was 8-72 months and only one patient needed re-dilatation during this period. Stent implantation may be a feasible alternative to balloon angioplasty and surgery for adolescents. Patients who have previous surgical repair, high surgical risk, unfavorable anatomy, or refuse consent for surgical intervention are the best candidates for stent implantation. However, longer follow-up and new studies are necessary especially in younger patients

Analysis of Gene Copy Number Variations in Patients with Congenital Heart Disease Using Multiplex Ligation-Dependent Probe Amplification

Objective: At the molecular and cellular levels, heart development entails the precise orchestration of genetic events such as the interplay of master transcriptional regulators, signaling pathways, and chromatin remodeling. Recent studies among patients with congenital heart disease (CHD) have shown the importance of recurrent copy number variations (CNVs) in a significant number of patients. Recurrent CNVs that span several genes may affect other important organs, besides the heart. Because CHD may be the first presenting symptom in such patients, the analysis of recurrent CNVs in the genomic regions containing genes associated with CHD in patients referring to cardiology clinics may lead to an early diagnosis and the treatment of extracardiac symptoms in these patients. In this study, we aimed to screen CNVs of genomic regions including GATA4, NKX2-5, TBX5, BMP4, and CRELD1 genes and to analyse the 22q11.2 chromosomal region in apparently nonsyndromic patients with cardiac septal defects. Methods: Genomic regions including GATA4, NKX2-5, TBX5, BMP4, and CRELD1 genes and the 22q11.2 chromosomal region were analyzed in apparently nonsyndromic 45 patients with cardiac septal defects using the MLPA P-311 A2 Congenital Heart Disease kit. Multiplex ligation-dependent probe amplification (MLPA) is an established technique for the detection of known CNVs. MLPA is substantially less expensive than array CGH and is relatively simple to use for clinicians without specific expertise in genomic technology; thus, MLPA could be used as a first-tier screening assay. Results: We screened 45 patients with cardiac septal defects for CNVs using the MLPA P-311 A2 kit. We identified three CNVs (n=3/45, 6.66%) and three 22q11 deletions. The CNVs were confirmed using fluorescence in situ hybridization. Conclusion: Our study confirmed that the analysis of recurrent CNVs using the MLPA assay within pediatric cardiology clinics can led to an early syndrome diagnosis in nonsyndromic patients with CHD.PubMedWoSScopu

Subcutaneous Defibrillator Implantation in Pediatric Patients

Objective: Although sudden cardiac death is rare in children, an intracardiac defibrillator system is indicated in children with various types of cardiomyopathy, primary electrical diseases, and after surgical repair of congenital heart defects. The use of transvenous defibrillator lead systems is limited in pediatric patients because of a small body size and/or limited vascular access. Subcutaneous array leads combined with an abdominally placed generator can enable implantation. Method: This is a retrospective study of 13 patients who underwent subcutaneous defibrillator implantation between September 2010 and March 2015. The subcutaneous system was preferred because patients were not amenable to transvenous lead placement. Results: The median patient age was 4.1 years, and the median patient weight was 12.1 kg. Diagnoses of patients were long-QT syndrome in 6, aborted cardiac arrest with left ventricular non-compaction in 3, hypertrophic cardiomyopathy with sustained ventricular tachycardia in 3, and arrythmogenic right ventricular cardiomyopathy in 1. Revision of the subcutaneous lead was required in 5 patients 2–26 months after the implantation. Appropriate shocks were observed in three patients. Inappropriate shock and lead fractures were observed in one patient during the follow-up period. The failure of therapy was observed in one patient. There were no perioperative complications and no early or late deaths. Conclusion: Subcutaneous defibrillator systems are safe and effective in pediatric patients when the transvenous method is risky and contraindicated. Because the high growth rate in this population leads to lead failures, a close follow-up of this population is essential. (Anatol J Cardiol 2016; 16: 630-4)PubMedWoSScopu

Electrical Storm In An Adolescent With Arrhythmogenic Right Ventricle Cardiomyopathy Treated With Cardiac Transplantation

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