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Effective management of the long QT syndrome with verapamil Yasuhiko Tomita 1 , Takashi Fukaya 1 , Masaru Yamakawa 1 , Kunizo Baba 1 , Seiichi Takao 2 1Department of Pediatrics, Kobe Central Municipal Hospital 2Department of Internal Medicine, Kobe Central Municipal Hospital pp.791-797
Published Date 1987/7/15
DOI https://doi.org/10.11477/mf.1404205096
  • Abstract
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An 11-year-old girl with congenital long QT syn-drome (Romano-Ward syndrome) was successfully treated with verapamil. She has a family history of sudden death (paternal aunt) and QT prolongation (sister). She had her first syncopal attack and con-vulsion at age of 6. She was controlled by propra-nolol (1 mg/kg) with initially good result followed by a recurrence of syncopal attacks after 4 years. She came to our hospital after fourth syncopal attack and convulsion with sustained chest pain and disturbance of consciousness persisting about 5 hours. She was in shock state with incessant form of torsade de pointes. Lidocaine and magnesium sulfate did pro-duce a transient improvement while additional large doses seemed to increase number of episodes. Atrial overdrive pacing and propranolol (0. 07 mg/kg ; bolus injection) also failed to control until verapamil (0. 1 mg/kg) dramatically produced sustained normal sinus rhythm after left stellate ganglion block. Following 9 attacks of short run of VT in every 3 hours were also eliminated perfectly with verapamil alone show-ing shortened QT interval. Chronic oral verapamil therapy was cautiously undertaken and she is now in stable state during 6 month follow-up period. We concluded that verapamil might be the safest and most effective drug in acute and chronic control of Romano-Ward syndrome.


Copyright © 1987, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1200 印刷版ISSN 0452-3458 医学書院

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