心房颤动机制研究进展-细胞核孔复合物与跨核运输障碍故事的开始:常染色体隐性遗传房颤家系1 1-57 family members-32 males-25 females-5 living generations-Age:3 m-93 y-Autosomal recessive inheritance pattern-7 consanguineous marriagesAn Interesting FamilyCirculation,2004Oberti et al.Circulation,2004;110:3753-3759Proband V:11-Chronic AF-QTc=0.42 s-Delivered by caesarean section due to fetal tachycardia,HR of 250 bpm,atrial fibrillation and atrial flutter-Echo at day 2:marked dilatation of both atria,EF 52%-EP at 1 m detected AF,ablation of AV n