Romano-Ward症候群のTorsade de pointesに対してVerapamilが著効であった1例

Effective management of the long QT syndrome with verapamil

　Romano-Ward症候群のtorsade de pointesに対して左星状神経節ブロック1〜4），propranolol^1,2,4,7,8），lido—caine^5,6），rapid atrial pacing^7,8）などが有効とされ，その発作の予防にはpropranolol,1idocaine類似経口薬が有効との報告がみられる^5,6）。また最近torsade de poin—tesにMg^＋＋が効くとの報告もある^9）。しかしRomano—Ward症候群のtorsade de pointesに対して上記治療があまり効果なく，今まで報告のみられなかったvera—pamilが著効で，また予防的治療にもその経口投与が有効であった1例を経験したので報告する。

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.