Radiofrequency ablation of accessory pathways in children with complex congenital cardiac lesions: a report of three cases

Catheter ablation is an accepted, highly effective modality of treatment for cardiac arrhythmias in children. The success rate depends on the operator's experience, especially in cases involving complex anatomies. We hereby report our recent experience of successful ablation of accessory pathways in three children with complex congenital heart diseases. The first case was a 7-year-old girl with tricuspid atresia and a previous Glenn shunt, in whom a sub-epicardial overt accessory pathway was successfully ablated via the coronary sinus. The second case, a 9-year-old girl, received accessory pathway ablation via the fenestration of an extracardiac Fontan pathway. The third case was a 14-year-old boy with dextrocardia, common atrium, common ventricle, and a previous extracardiac Fontan operation, in whom ablation of a concealed accessory pathway was carried out retrogradely from the aorta. All the ablations were done in Rajaie Cardiovascular, Medical and Research Center, Tehran, and all the patients were discharged from the hospital without any complication

Same genotype and different phenotypes in a family with PRKAG2 gene mutation / 中华心血管病杂志

<p><b>OBJECTIVE</b>The gamma(2) subunit of AMP-activated protein kinase (PRKAG2) located in chromosome 7 plays an important role in regulating metabolic pathways, and patients with PRKAG2 mutations are associated with familial ventricular pre-excitation, hypertrophic cardiomyopathy and AV block. We observed the difference on the phenotypes in a large family with same PRKAG2 mutation.</p><p><b>METHOD</b>Direct DNA sequence was performed to screen the exons and exon-intron boundaries of PRKAG2 gene in a large family with 13 affected persons detected by electrocardiography (ECG).</p><p><b>RESULTS</b>Sinus bradycardia, short PR interval, right bundle bunch block (RBBB), complete AV block, atrial flutter, atrial fibrillation and sudden cardiac death were identified in this family. Hypertrophic cardiomyopathy was found in one family member. Genetic analysis revealed a missense mutation (Arg302Glu) in all affected family members. This mutation was previous described in patients with Wolff-Parkinson-White (WPW) syndrome and hypertrophic cardiomyopathy.</p><p><b>CONCLUSIONS</b>Besides WPW syndrome and hypertrophic cardiomyopathy, PRKAG2 mutations are responsible also for a diverse phenotypes. PRKAG2 gene mutation should be suspected with familial occurrence of RBBB, sinus bradycardia, and short PR interval.</p>