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 37(2): 78-87 (2021)
doi:10.9794/jspccs.37.78

ReviewReview

先天性気管狭窄症を合併した先天性心疾患とその周術期管理Clinical Features and Perioperative Management of Congenital Tracheal Stenosis in Patients with Congenital Heart Diseases

兵庫県立こども病院小児集中治療科Department of Pediatric Critical Care Medicine, Hyogo Prefectural Kobe Children’s Hospital ◇ Hyogo, Japan

発行日:2021年8月1日Published: August 1, 2021
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先天性気管狭窄症(CTS)は完全気管輪を伴った気管内腔の狭小化により重篤な呼吸障害を来し得る難治性の疾患である.スライド気管形成術の普及によりその治療成績は著しく向上したが,窒息などの急変対応,手術手技や周術期管理,術後フォローアップの難しさから,国内でCTSの管理や治療に取り組んでいる施設は限られている.一方,先天性心疾患(CHD)において呼吸と循環は相互に密接に関連しているため,気道病変の合併は病態をより複雑かつ重症化させ,診療や治療に難渋することが多い.CTSは稀な疾患であるがCHDの合併は比較的多く,小児循環器診療に携わる医療従事者にとってCTSに関する理解を深めることは重要である.本稿ではCTSについて概説し,CHD合併に焦点を当てながら外科的治療を含めた周術期管理の要点をまとめ,施設間ネットワークやチーム医療によるCTS管理の普及と発展を期待する.

Congenital tracheal stenosis (CTS), characterized by the narrowing of the tracheal tract due to complete tracheal rings of the cartilage, is a rare but potentially life-threatening disease that often leads to severe respiratory failure in children. CTS is often found in conjunction with congenital heart diseases (CHD). Although slide tracheoplasty has demonstrated huge improvement in clinical outcomes of treating CTS and has recently emerged as the dominant surgical technique, some hospitals in Japan are still challenged by the amount of work required to manage and treat CTS cases. Generally, CTS in patients with CHD makes the condition more complex and severe due to the sensitive and crucial link between the pulmonary and systemic circulations, such as cardiopulmonary interaction. Therefore, acquiring sufficient knowledge on CTS is highly essential for the attending medical staff in pediatric cardiology. This article reviews and summarizes the clinical presentation and perioperative management of CTS, emphasizing its association with the CHD.

Key words: congenital tracheal stenosis; congenital heart disease; slide tracheoplasty; perioperative management; cardiopulmonary interaction

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This page was last modified on 2021-08-03T20:08:36.000+09:00


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