An adult case of unrepaired pulmonary atresia and ventricular septal defects with main aortopulmonary collateral arteries

DOI
  • Horiuchi Kiwamu
    Division of Cardiovascular, Respiratory and Neurology, Department of Internal Medicine, Asahikawa Medical University
  • Tanabe Yasuko
    Division of Cardiovascular, Respiratory and Neurology, Department of Internal Medicine, Asahikawa Medical University
  • Takeuchi Toshiharu
    Division of Cardiovascular, Respiratory and Neurology, Department of Internal Medicine, Asahikawa Medical University
  • Date Ayumi
    Division of Cardiovascular, Respiratory and Neurology, Department of Internal Medicine, Asahikawa Medical University
  • Akasaka Kazumi
    Division of Cardiovascular, Respiratory and Neurology, Department of Internal Medicine, Asahikawa Medical University
  • Minoshima Akiho
    Division of Cardiovascular, Respiratory and Neurology, Department of Internal Medicine, Asahikawa Medical University
  • Sugiyama Eitaro
    Division of Cardiovascular, Respiratory and Neurology, Department of Internal Medicine, Asahikawa Medical University
  • Kitani Yuya
    Division of Cardiovascular, Respiratory and Neurology, Department of Internal Medicine, Asahikawa Medical University
  • Sakamoto Naka
    Division of Cardiovascular, Respiratory and Neurology, Department of Internal Medicine, Asahikawa Medical University
  • Kawamura Yuichiro
    Division of Cardiovascular, Respiratory and Neurology, Department of Internal Medicine, Asahikawa Medical University
  • Sato Nobuyuki
    Division of Cardiovascular, Respiratory and Neurology, Department of Internal Medicine, Asahikawa Medical University
  • Hasebe Naoyuki
    Division of Cardiovascular, Respiratory and Neurology, Department of Internal Medicine, Asahikawa Medical University

Bibliographic Information

Other Title
  • 主要体肺動脈側副血行路を伴う心室中隔欠損＋肺動脈閉鎖を認めた非手術成人の1例
Published
2017-12-15
DOI
  • 10.11281/shinzo.49.1261
Publisher
Japan Heart Foundation

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<p>症例は51歳，男性．6歳時に心疾患の指摘があり，学童期より激しい運動時にチアノーゼを認めることがあったが経過観察をしていた．数日前より咳嗽，発熱，呼吸困難が出現し，近医を受診したところ著明な低酸素血症を認め，胸部X線，CT検査より肺炎および心不全が疑われ同院に緊急入院した．心エコーを行ったところ心室中隔欠損（VSD）＋肺動脈閉鎖（PA）が認められ，体外式膜型人工肺（ECMO）で呼吸管理の下，精査治療を目的に当院へ搬送された．集中治療室にて集学的治療を行い，心不全および呼吸不全は順調に改善した．造影CTでは下行大動脈より右へ3本，左へ1本の主要体肺動脈側副血行路（MAPCAs）が認められ，心臓カテーテル検査にて圧測定を行ったところ，右中MAPCAの平均圧は36 mmHg，左MAPCAおよび右上と右下MAPCAは平均圧で28～29 mmHgであった．本症例におけるMAPCAの外科的統合は解剖学的に困難であり，また患者は手術を希望されず，在宅酸素療法を導入したところ症状の改善を認めたため，外来で経過観察する方針となった．MAPCAsを伴ったVSD＋PAの非手術成人例は稀であり報告する．</p>

Journal

  • Shinzo

    Shinzo 49 (12), 1261-1266, 2017-12-15

    Japan Heart Foundation

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