日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Online ISSN: 2187-2988 Print ISSN: 0911-1794
特定非営利活動法人日本小児循環器学会 Japanese Society of Pediatric Cardiology and Cardiac Surgery
〒162-0801東京都新宿区山吹町358-5アカデミーセンター Japanese Society of Pediatric Cardiology and Cardiac Surgery Academy Center, 358-5 Yamabuki-cho, Shinju-ku, Tokyo 162-0801, Japan
Pediatric Cardiology and Cardiac Surgery 33(4): 326-331 (2017)
doi:10.9794/jspccs.33.326

症例報告Case Report

手掌多汗症状に対し胸腔鏡下胸部交感神経遮断術を施行したQT延長症候群の一例Long QT Syndrome with Palmar Hyperhidrosis Treated with Endoscopic Thoracic Sympathectomy

佐賀大学小児科Department of Pediatrics, Saga University Hospital ◇ Saga, Japan

受付日:2017年2月21日Received: February 21, 2017
受理日:2017年6月7日Accepted: June 7, 2017
発行日:2017年7月1日Published: July 1, 2017
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先天性QT延長症候群(LQTS)のTorsade de Pointes(TdP)発生において運動,精神的興奮,ストレスの関与が知られており,特にLQT1, LQT2では交感神経興奮が強く関与するといわれている.そのためTdP予防としてβ遮断薬が第一選択薬として挙げられるが,内服コンプライアンスの問題などにより治療継続に難渋することを時に経験する.症例は14歳女児.明らかなLQTS,突然死,失神の家族歴はなく,小学校入学後より失神発作を繰り返していた.学校健診でQT延長を指摘され当科を紹介され受診し,遺伝子検査の結果QT延長症候群:LQT2の診断に至った.発作予防ためβ遮断薬を開始し,失神発作を起こすことなく経過していたが,運動耐容能低下のため10歳時に治療を自己中断された.その後失神発作を繰り返したため13歳より通院再開となり,運動制限ならびにβ遮断薬内服を開始した.失神発作を認めなくなったが,精神的に不安定となり不登校となり,また以前より本人が両側の手掌多汗症状に苦痛を感じていたため14歳時に胸腔鏡下胸部交感神経遮断術(endoscopic thoracic sympathectomy: ETS)を施行した.術後は多汗症状の改善が得られ,明らかな合併症を認めることなく経過した.加えて安静時の補正QT時間が570 msから511 msと改善したので報告する.

It is well known that exercise, mental excitement, and stress induce torsade de pointes (TdP) in congenital long QT syndrome (LQTS). The excitability of the sympathetic neuron is believed to be deeply involved especially in LQT1 and LQT2. Therefore, beta blockers are the first-line drugs considered to prevent TdP, but in some cases, continuing medication is difficult because of internal medication compliance issues. The patient was a 14-year-old girl who had no apparent family history of LQTS, sudden death, or fainting, but had repeated syncopal attacks after starting elementary school. Long QT was identified in a school health examination, and the patient was referred to our hospital. She was diagnosed as having long QT syndrome (LQT2) on the basis of genetic screening results. Βeta blocker was administered to prevent attacks. Over time, no syncopal attacks occurred, but she suspended her own treatment at age 10 years because of reduced exercise tolerability. She returned to our hospital as an outpatient at age 13 years because of repeated syncopal attacks and received beta blocker and exercise restrictions. The syncopal attacks ceased, but the patient became mentally unstable and stopped attending school. Moreover, she had palmar hyperhidrosis in both hands. At age 14 years, she was performed endoscopic thoracic sympathectomy (ETS). The palmar hyperhidrosis improved, and no side effects such as compensatory hyperhidrosis or Horner’s syndrome were observed. Moreover, the corrected QT when resting improved from 570 to 511 ms.

Key words: long QT syndrome; torsade de pointes; endoscopic thoracic sympathectomy; palmar hyperhidrosis

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