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[目的]探讨一个遗传性长QT综合征(LQTS)合并房颤家系的临床特点。[方法]对该家系包括先证者在内的三代44例家族成员进行常规临床检查、心电图、动态心电图、超声心动图等检查。[结果]先证者诊断LQTS合并阵发性房颤。6例临床确诊LQTS,8例疑诊LQTS。先证者母亲有晕厥和房颤病史,包括先证者在内2例LQTS合并房颤,另外1例为持续性房颤合并III度房室传导阻滞;阵发性房速2例,有可疑房颤症状2例。2例青少年家族成员心电图表现早期复极综合征,其中1例有晕厥,疑诊LQTS。[结论]LQTS合并房颤是一种少见的临床情况,同时又合并传导阻滞临床罕见。早期复极综合征可能不一定总是良性临床经过。遗传性疾病的家系研究是进一步基因学研究的基石。
[Objective] To investigate the clinical features of a hereditary long QT syndrome (LQTS) combined with atrial fibrillation pedigree. [Methods] A total of 44 family members of three generations of the family, including probands, underwent routine clinical examination, electrocardiogram, Holter, echocardiography and so on. [Results] The probands diagnosed LQTS complicated with paroxysmal atrial fibrillation. 6 cases of clinically confirmed LQTS, 8 cases of suspected LQTS. The proband’s mother had a history of syncope and atrial fibrillation, including probands, including 2 cases of LQTS with atrial fibrillation, another case of persistent atrial fibrillation with III degree atrioventricular block; paroxysmal atrial tachycardia in 2 cases, there Suspicious symptoms of atrial fibrillation in 2 cases. 2 cases of adolescent family members showed early repolarization ECG, including 1 case of syncope, suspected LQTS. [Conclusion] LQTS combined with atrial fibrillation is a rare clinical situation, while the combination of block and clinical block rare. Early repolarization syndrome may not always be benign clinical. Pedigree studies of hereditary diseases are the cornerstone of further genetic research.