Mehta A V, Chidambaram B, Garrett A
Department of Pediatrics, James H. Quillen College of Medicine, East Tennessee State University, Johnson City 37604, USA.
Pediatr Cardiol. 1995 Sep-Oct;16(5):231-4. doi: 10.1007/BF00795713.
Symptomatic sinus bradycardia, due to either sick sinus syndrome or vagotonia, can be familial, affecting several members of a family. We report an 18-year-old male patient with palpitations and limited exercise capacity who was noted to have severe sinus bradycardia. His resting heart rate was 40/min, with normal PR and corrected QT intervals, and sinus pauses up to 6 seconds during sleep. Exercise treadmill test and pharmacologic autonomic blockade during electrophysiologic studies abolished the bradycardia, suggestive of vagotonia rather than intrinsic sinus node dysfunction. This patient's father and a female cousin had a similar clinical history but associated with syncope and severe sinus bradycardia. The mode of transmission appeared to be autosomal dominant. All three have permanent demand pacemakers implanted and are asymptomatic.
由病态窦房结综合征或迷走神经张力亢进引起的症状性窦性心动过缓可能具有家族性，影响一个家族中的多个成员。我们报告了一名18岁男性患者，他有心悸症状且运动能力受限，被发现有严重的窦性心动过缓。他静息心率为40次/分钟，PR间期和校正QT间期正常，睡眠期间窦性停搏长达6秒。运动平板试验以及电生理研究期间的药物自主神经阻滞消除了心动过缓，提示为迷走神经张力亢进而非窦房结内在功能障碍。该患者的父亲和一名女性堂妹有类似的临床病史，但伴有晕厥和严重的窦性心动过缓。遗传方式似乎为常染色体显性遗传。三人都植入了永久性按需起搏器，且均无症状。
