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聋哑-心脏综合征及治疗观察

The surdo-cardiac syndrome and therapeutic observations.

作者信息

Olley P M, Fowler R S

出版信息

Br Heart J. 1970 Jul;32(4):467-71. doi: 10.1136/hrt.32.4.467.

Abstract

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.

摘要

1957年首次被发现,聋哑-心脏综合征包括先天性耳聋、QT间期延长,以及晕厥和猝死的高发生率。两名患者的血流动力学研究除左心室舒张期出现异常波(可能与左心室舒张异常有关)外均正常。晕厥发作基于心律失常:室颤和心搏停止都可能发生。心脏异常的肾上腺素能刺激可能是原因所在。普萘洛尔似乎对预防晕厥发作有效。人工起搏在一名患者中诱发了室颤,似乎是禁忌的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/927a/487355/b3a79a2fc183/brheartj00308-0048-a.jpg

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