Walls J, Dipchand A, Sanatani S
Division of Cardiology, British Columibia Children's Hospital, Children's Heart Centre, 4480 Oak Street Vancouver, British Columbia, V6H 3V4, Canada.
Division of Cardiology, The Hospital for Sick Children, 555 University Ave, Toronto, ON, M5G 1X8, Canada.
Pediatr Cardiol. 2006 Jan-Feb;27(1):156-159. doi: 10.1007/s00246-005-1114-1.
Atrioventricular block has been described in association with cases of long QT syndrome and mortality is increased in this subgroup. We describe an infant with congenital QT prolongation and atrioventricular block with normal cardiac function, leading to the initial diagnosis of long QT syndrome. She subsequently developed dilated cardiomyopathy requiring cardiac transplantation. We postulate that the presenting electrocardiograph abnormalities were early manifestations of the myocardial disease, preceding the development of myocardial dysfunction by several months. The need for heightened surveillance in cases of QT prolongation with atrioventricular block is amplified by the possibility of an evolving cardiomyopathy.
房室传导阻滞已被描述与长QT综合征病例相关，且该亚组患者的死亡率会增加。我们描述了一名患有先天性QT延长和房室传导阻滞且心功能正常的婴儿，最初被诊断为长QT综合征。她随后发展为扩张型心肌病，需要进行心脏移植。我们推测，最初出现的心电图异常是心肌病的早期表现，比心肌功能障碍的出现早几个月。由于存在发展为心肌病的可能性，对于伴有房室传导阻滞的QT延长病例，加强监测的必要性更加突出。
