- 期刊
Atrioventricular Block in Kearns-Sayre Syndrome: a Case Report
Kearns-Sayre症候群造成房室傳導阻滯：─病例報告
摘要
Kearns-Sayre(K-S)症候群包含有眼外肌麻痺、眼底色表沉著病變及心臟傳導阻滯等三項臨床表徵。一般認為，粒腺體病變是造成此症候群的主要原因。而其中又以心臟傳導阻滯為影響此症候群預後的最重要因素，常常因此而需要裝上心律調節器，以預防猝死。本篇報導一位34歲男子，他於15歲時，因眼瞼下垂而被診斷為K-S症候群。此次因頭暈、無力被送至醫院，結果發現心電圖為2:1房室傳導阻滯。於住院期間，也發現有間斷性完全房室傳導阻滯。心電氣生理檢查發現HV間隔延長（85ms）。因為有房室傳導阻滯，我們為他植入一永久性心律調節器，目前病人狀況穩定並繼續接受門診追蹤。
關鍵字
無資料並列摘要
The Kearns-Sayre (K-S) syndrome which includes the triad of progressive external ophthalmoplegia, pigment retinopathy, and disorder of cardiac conduction was first described in 1958. The mitochondria disorder is believed to be the cause of this syndrome. Involvement of the cardiac conduction system is the most importment prognostic factor in K-S syndrome. A 34-year-old male K-S syndrome patient, manifesting as ptosis and weakness of limbs since the age of 15 years, suffered from dizziness and weakness. Twelve-lead eletrocardiography (ECG) showed a 2:1 atrioventricular (AV) block with slow ventricular rate. Intermittent complete AV block, complete left bundle branch block and torsades de pointes were noted in Holter ECG. The electrophysiology study demonstrated prolonged HV interval (85 ms) on conduction best and infra-His black on non-conduction beat. A VVIR mode of permanent pacemaker was implanted and the patient's condition was stable during this period of follow-up.
延伸閱讀
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