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在先天性长QT综合征中威胁生命的室性心律失常是常见的。作者在门诊中发现一个112名成员的家族中有15人在年轻时睡眠中猝死。所有猝死成员既往健康,无心悸或晕厥史,且均能较好耐受运动,仅一人有显著的QT间期延长和异常T波。在存活的家族中未发现心脏病证据,反复作24小时动态心电图(AECG)未检测到室性心律失常,房室传导阻滞。无人耳聋,听力图检查皆正常。此项研究试图了解先天性心动过缓依赖性QT延长与晚电位的关系。
Life-threatening ventricular arrhythmias are common in congenital long QT syndromes. In the outpatient clinic, 15 of the 112 family members were found to have had sudden death during their young sleep. All sudden death patients had no prior history of heart failure, palpitations or syncope and were well tolerated. Only one person had significant QT prolongation and abnormal T wave. No evidence of heart disease was found in the surviving family, and ventricular arrhythmia and atrioventricular block were not detected in the 24-hour Holter assay (AECG). No one deaf, audiogram examination are normal. This study sought to understand the relationship between congenital bradycardia-dependent QT prolongation and late potentials.