Clinical Features and Perioperative Management of Congenital Tracheal Stenosis in Patients with Congenital Heart Diseases

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 med-ical staff in pediatric cardiology. This article reviews and summarizes the clinical presentation and perioperative management of CTS, emphasizing its association with the CHD.