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    A CASE OF SECKEL SYNDROME WITH TRICUSPID ATRESIA
    (MEDECINE ET HYGIENE, 2014) Arslan, D.; Cimen, D.; Guvenc, O.; Sert, A.; Oktay, A.; Oran, B.
    A case of Seckel syndrome with tricuspid atresia: Seckel syndrome is an autosomal recessive disease presenting with marked growth retardation, microcephalic dwarfism, some facial and skeletal abnormalities. Tricuspid atresia is a rare and life threatening cyanotic congenital heart diseases, with an incidence of 1% to 3%. It is feature of the anatomically normally related great arteries with a large ventricular septum defect and stenosis of right ventricular outflow tract. Tricuspid atresia has never been reported in patients with Seckel syndrome. Here we report a 15-day-old girl baby diagnosed as having Seckel syndrome with tricuspid atresia.
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    A CASE WITH 18q DELETION SYNDROME IDENTIFIED WITH B CELL ABSENCE AND CONGENITAL HEART DISEASE
    (MEDECINE ET HYGIENE, 2015) Guvenc, O.; Cimen, D.; Kaplan, M. B.; Aslan, E.; Artac, H.; Annagur, A.
    [Abstract not Available]
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    Prospective ECG-gated high-pitch dual-source cardiac CT angiography in the diagnosis of congenital cardiovascular abnormalities: Radiation dose and diagnostic efficacy in a pediatric population
    (ELSEVIER MASSON, CORP OFF, 2016) Koplay, M.; Kizilca, O.; Cimen, D.; Sivri, M.; Erdogan, H.; Guvenc, O.; Oc, M.
    Purpose: The goal of this study was to investigate the radiation dose and diagnostic efficacy of cardiac computed tomography angiography (CCTA) using prospective ECG-gated high-pitch dual-source computed tomography (DSCT) in the diagnosis of congenital cardiovascular abnormalities in pediatric population. Materials and methods: One hundred five pediatric patients who were clinically diagnosed with congenital heart disease with suspected extracardiac vascular abnormalities were included in the study. All CCTAs were performed on a 128 x 2-section DSCT scanner. CCTA findings were compared with surgical and/or conventional cardiac angiography findings. Dose-length product (DLP) and effective doses (ED) were calculated for each patient. Patients were divided into 4 groups by age, and ED and DLP values were compared among groups. The image quality was evaluated using a five-point scale. Results: CCTA showed 173 abnormalities in 105 patients. There were 2 patients with false positive and 3 with false negative findings. The sensitivity and specificity of CCTA were 98.3% and 99.9%, respectively. The positive predictive value and negative predictive value of CCT were 98.9% and 99.9%, respectively. The average DLP and ED values were 15.6 +/- 9.6 (SD) mGy.cm and 0.34 +/- 0.10 (SD) mSv, respectively. The mean image quality score was 4.8 +/- 0.5 (SD) in all patients. The inter-observer agreement for the image quality scores was good (kappa = 0.80). Conclusion: CCTA is an excellent imaging modality for evaluation of cardiovascular abnormalities and provides excellent image quality with very low radiation exposure when low-dose prospective ECG-triggered high-pitch DSCT is used. (C) 2016 Editions francaises de radiologie. Published by Elsevier Masson SAS. All rights reserved.