Background/aim: Airway compression (AC) by vascular structures is an important complication of congenital heart disease (CHD) that often goes unrecognized. It is not easy to identify whether CHD patients require additional invasive examinations or not. Therefore, the present study aims to develop an AC diagnostic algorithm for CHD patients. Materials and methods: CHD patients with persistent respiratory symptoms that were treated between January 2007 and December 2015 were retrospectively reviewed. The following data were recorded for all CHD patients with AC: age, cardiac anomalies, the compressed structure, the airway diameter ratio (ADR), the compressing structure(s), treatment, and follow-up.Results: During the 8-year study period, 62 of 253 CHD patients had persistent respiratory symptoms, of which 11 cases were diagnosed as AC via bronchoscopy and/or thoracic computed tomography angiography. The most frequently affected structures were the left main bronchus and trachea, and the most common compressing structure was the right pulmonary artery. The ADR was near total compression in 3 patients and >0.50 in 3 patients. During follow-up, 5 of the 11 patients with AC underwent surgery, 2 died, and 4 were followed clinically. Patients with ADR of >0.50 did not require surgery and were followed clinically. Conclusion: CHD patients with persistent respiratory symptoms associated with lower respiratory airway obstruction should be evaluated via invasive examination. An AC diagnostic algorithm for pediatric CHD patients was developed.