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(2): 78-87 (2021)


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


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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