Sudden Death and the Familial Occurrence of Mid‐Systolic Click, Late Systolic Murmur Syndrome
Sudden Death and the Familial Occurrence of Mid‐Systolic Click, Late Systolic Murmur Syndrome
复制标题
猝死和家族性中收缩期咔嗒声、收缩晚期杂音综合征
作者：
S. D. Shappell;C. Marshall;Ross E. Brown;T. A. Bruce
该男子是患有二尖瓣后叶气球样扩张综合征的六名家庭成员之一，在首次出现心脏骤停之前，曾经历过五次晕厥发作。在几乎完全恢复后进行的后续评估显示，尽管进行了广泛的心电图监测和最大运动测试，但 Q-Tc 略有延长，但没有出现心律失常。静脉注射普萘洛尔未能改变心导管插入术时的 Q-Tc 间期或二尖瓣后叶的气球扩张程度。口服 Dilantin 治疗达到临床毒性程度后，未能显着缩短 Q-Tc 间期。在口服普萘洛尔期间，患者在一次紧张的争吵中最终心脏骤停。进行了详细的尸检研究，并对以前未报道过的“粘液瘤”材料进行了组织化学鉴定。
The propositus, one of six family members with the ballooning posterior mitral leaflet syndrome, had experienced five syncopal episodes prior to initial presentation with cardiac arrest. Subsequent evaluation, after nearly complete recovery, revealed slight Q-Tc prolongation but no arrhythmia, despite extensive ECG monitoring and maximum exercise testing. Intravenous propranolol failed to alter the Q-Tc interval or the magnitude of ballooning of the posterior mitral leaflet at cardiac catheterization. Therapy with oral Dilantin to the point of clinical toxicity failed to significantly shorten the Q-Tc interval. While on oral propranolol, the patient, during a stressful argument, suffered her final cardiac arrest. Detailed post mortem studies were performed, with histochemical identification of the “myxomatous’ material not previously reported.