Aim: To assess heart rhythm disturbances (HRD) rate and structure in children with single ventricle (SV) at the stages of defect hemodynamic correction. Methods: 70 patients with CHD with SV that were performed total cavopulmonary connection (TCPC) with extracardiac conduit were examined. All the patients were performed ECG and Holter monitoring. To integrate data, all the patients were divided into 8 groups: 1 -sinus rhythm; 2 -non-sinus rhythm (atrial rate, nodal rhythm, AV-dissociation); 3 -bradyarrhythmia (sinus and atrial bradycardia); 4 -sinus tachycardia; 5 -supraventricular tachycardias (SVT) ( paroxysmal and incessant); 6 -extrasystole (supraventricular and ventricular); 7 -atrioventricular block (AVB) of I-II deg.; 8 -AV block, III deg. Results: Only one patient had 2 SVT paroxysms, broken cordarone bolus dosing, in early postoperative period at the first stage. At the second stage (Glenn operation) HRD was recorded in 14 (20%) pts, in 7 (10%) of them during admission. Non-sinus rhythm (86%) predominated in HRD structure. At the third stage (TCPC) recent HRD was recorded in 36 (51.4%) pts. After TCPC recent HRD was registered in 23 (32.9%) pts, in 13 of them HRD was preserved, and in 34 (48.6%) it was not recorded. Arrhythmias, connected with sinus node dysfunction (bradyarrhythmias, SVT, non-sinus rhythm), predominate in HRD structure in 43.9%. The negative correlation between Sat O2 in capillary blood and arrhythmias frequency was disclosed after TCPC, p ¼ 0,02. The older patient at TCPC and lower SV contractile function after it, the oftener arrhythmias, connected with sinus node dysfunction, appeared ( p ¼ 0,04). Conclusions: HRD appeared in patients with extracardiac Fontan at any stage of hemodynamic correction. Arrhythmias, connected with sinus node dysfunction, predominated in frequency of occurrence. Low Sat, low SV contractile function and the age of TCPC made their own contributions into HRD appearance after Fontan. Conflict of interest: none CLINICAL AND GENETIC PROFILE OF ARRHYTHMOGENIC CARDIOMYOPATHY WITH LEFT VENTRICULAR INVOLVEMENTErika López-Moreno, Pablo J. Sánchez-Millán, Manuel Molina Lerma, Isabel GallardoSánchez, Rosa Macias Ruíz, Juan Jiménez-Jáimez, Miguel Á Lvarez, and Luis Tercedor Granada, SpainBackground: The natural history of arrhythmogenic cardiomyopathy (AC) with left ventricular involvement appears to be different from the exclusive right ventricular disease. Our aim is to analyze the clinical and genotypic features in our series of subjects with left ventricular AC comparing to the patients with the exclusive right ventricular disease. Methods: We studied 30 patients with AC. Information of clinical variables, advanced cardiac imaging and genetic testing was collected. These variables were compared in both groups of patients with AC, who presented or not left ventricular involvement. Results: Biventricular involvement was predominant (60%). There were significant differences in the arrhythmic profile of the patients, as well as for the burden of sudden cardiac death in...

A 5-year-old boy referred to our service with suspected sinus node dysfunction. In addition to the arrhythmia, he had moderate mitral valve regurgitation and depressed ventricular function during a hypoglycemic episode. Cardiac abnormalities resolved with glucose infusion. We believe that hypoglycemia was responsible for the cardiac manifestations and it should be considered in unexplained rhythm disturbances or ischemia.