The surdo-cardiac syndrome and therapeutic observations
The surdo-cardiac syndrome and therapeutic observations
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DOI：
10.1136/hrt.32.4.467
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发表时间：
1970-07
影响因子：
--
通讯作者：
P. Olley;R. Fowler
中科院分区：
文献类型：
--
作者：
P. Olley;R. Fowler
1957年首次发现，该综合征包括先天性耳聋、QT间期延长、晕厥和猝死的高发病率。2例患者的血流动力学检查正常，但左室舒张期出现异常波，可能与左室舒张功能异常有关。晕厥发作是由于心律失常引起的：室颤和心搏停止均可发生。心脏的异常肾上腺素能刺激可能是原因。普萘洛尔似乎对预防晕厥发作有效。人工起搏引起心室颤动的一名患者，似乎禁忌。
First recognized in 1957, the surdo-cardiac syndrome includes congenital deafness, prolonged QT interval, and a high incidence of syncope and sudden death. Haemodynamic studies in two patients were normal except for an abnormal wave during left ventricular diastole probably related to abnormal left ventricular relaxation. The syncopal attacks are based on cardiac arrhythmias: both ventricular fibrillation and asystole may occur. Abnormal adrenergic stimulation of the heart is probably responsible. Propranolol appears to be effective in preventing the syncopal attacks. Artificial pacemaking provoked ventricular fibrillation in one patient and seems contraindicated.