PO-0051 Zinc And Copper Disorders In Children With Diabetes Type 1

Abstract

Discussion Improvement of the recorded changes in the heart rate and LVEDD after VD therapy implements an important role of VD through its effect on the concentration of the extracellular calcium ion that could modify the strength of the myocardial contraction through excitation-contraction coupling. Conclusion These data strongly indicate that the maintenance of an optimal vitamin D status may be a promising approach for the prevention and/or therapy of myocardial diseases and in countries with high prevalence of VDD, vitamin D supplementation can prevent this risk of cardiac dysfunction. Background and aims The arterial switch operation (ASO) is the surgical standard of care for repair of transposition of the great arteries (D-TGA). Until recently, anatomical variations of the coronary arteries, especially the intramural course of a single coronary artery, were considered contraindications for ASO. Transfer of the coronary arteries may be a surgical challenge in these cases increasing the risk of (sub-)acute coronary artery occlusions. Methods We report our management of two exemplary cases of D-TGA with coronary artery anomalies: PO-0047(1) Single coronary ostium of RCA, Cx and LAD originating from aortic sinus II and an intramural course of the proximal LAD.(2) Side-by-side position of the great arteries, RCA and LAD originating from sinus I and Cx from sinus II. Results Both neonates successfully underwent ASO with transfer of the coronary arteries.During the post-operative period, patient 1 was diagnosed with a subacute anteroseptal ischemia and was then managed conservatively. Follow-up echocardiogram at 12 months demonstrated satisfactory left and good right ventricular function.The postsurgical course of patient 2 was uneventful with good biventricular function at follow-up. Conclusions Anatomical variations of the coronary arteries require adaptions of the surgical technique of coronary artery transfer. Nowadays, ASO is even possible in patients with D-TGA and complex coronary anomalies. The long-term management, however, has to be evaluated, e.g. regarding the need for coronary artery re-surgery. Background and aims Absent pulmonary valve (APV) is a rare congenital defect of the right ventricular outflow tract (RVOT). The genetics of APV are unknown. However, mutations in the NOTCH-signalling pathway have been associated with RVOT obstruction. Mutations in the JAG-1 gene cause a broad spectrum of symptoms, ranging from an isolated heart defect to the complete clinical features of Alagille syndrome. PO-0050We present the case of a 14 month-old girl with APV and a family history of Alagille syndrome. Methods Pulmonary stenosis and a large ventricular septal defect (VSD) had been diagnosed prenatally. Postnatal echocardiogram revealed an APV, pulmonary stenosis, a large sub-aortal VSD, and right ventricular hypertrophy.Genetic analysis of the JAG-1 gene showed a frame-shiftmutation in exon 12 of the JAG-1 gene that had not been described before.The patient underwent corrective heart surgery at 9 months o...