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
Pediatric Cardiology and Cardiac Surgery 37(4): 283-294 (2021)
doi:10.9794/jspccs.37.283

ReviewReview

先天性心血管疾患に合併する先天性気管狭窄Congenital Tracheal Stenosis as a Complication of a Congenital Cardiovascular Defect

1平塚市民病院小児科Department of Pediatrics, Hiratsuka City Hospital ◇ Kanagawa, Japan

2東京都立小児総合医療センター循環器科Department of Cardiology, Tokyo Metropolitan Children’s Medical Center ◇ Tokyo, Japan

3慶應義塾大学医学部小児科学教室Department of Pediatrics, Keio University School of Medicine ◇ Tokyo, Japan

受付日:2021年4月9日Received: April 9, 2021
受理日:2021年6月4日Accepted: June 4, 2021
発行日:2021年12月1日Published: December 1, 2021
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先天性気管狭窄は,気管軟骨が気管全周にわたり完全輪状軟骨輪を形成し,狭窄所見を呈する疾患である.多くは新生児・乳児期早期から,呼吸器症状が出現する.半数以上の症例で先天性心血管疾患を合併し,特に肺動脈スリングが多い.小児循環器科医・小児心臓血管外科医が診断と管理を担う機会が多いが.呼吸状態と循環動態が相互に影響しながら病態を形成するため,小児外科医,小児呼吸器科医,麻酔科医,小児集中治療医,新生児科医との緊密な連携診療が必要である.本総説では,心血管疾患との合併を念頭に発生・解剖・病態を説明し,手術介入の要点を概説する.

Congenital tracheal stenosis is caused by complete cartilaginous rings and leads to obstructive respiratory disease. Most patients with this condition experience respiratory symptoms from the neonatal or infantile period. In more than half of these patients, the condition is associated with a congenital cardiovascular defect, especially a pulmonary artery sling. Cardiovascular procedures for treating this condition are performed often. However, its complicated pathology, involving interactions between the respiratory and circulatory systems, poses a challenge to the pediatric cardiologist and cardiovascular surgeon. Therefore, a multidisciplinary team-based approach based on cooperation between pulmonologists, pediatric surgeons, anesthesiologists, neonatologists, and intensivists is highly recommended. Thus, this review provides an outline of congenital tracheal stenosis from the embryological, anatomical, and pathophysiological perspectives, with special attention to possible complications of congenital cardiovascular defects. Furthermore, it provides some recommendations regarding surgical techniques.

Key words: congenital tracheal stenosis; complete cartilaginous rings; congenital cardiovascular defect; pulmonary artery sling; multidisciplinary team-based approach

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This page was last modified on 2022-03-08T10:54:07.000+09:00


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