OBJECTIVES: Congenital heart disease (CHD) is known to be associated with congenital and acquired airway disorders such as bronchopulmonary dysplasia (BPD). Short-acting β2-agonists (SABA) are highly effective in the treatment of BPD. However, β-adrenoceptor agonists have inotropic and chronotropic effects that can increase arrhythmias or tachycardia. To our knowledge, among pediatric patients with CHD, the association of SABAs therapy with arrhythmia in pediatric patients with BPD have not been evaluated before. The focus of this study was to compare the occurrence of arrhythmia between SABA users and non-users among pediatric patients with BPD and CHD. METHODS:  Texas Medicaid database from 2008-2014 was used to conduct the retrospective cohort study. Patients aged ≤12 years who had a diagnosis of CHD (ICD-9-CM 745-747.xx, V151) and BPD (ICD-9-CM 770.7) were included in this study. SABAs included albuterol, ipratropium, levalbuterol, and tiotropium. Covariates adjusted for were demographic factors (age, gender, and race) and clinical factors (respiratory distress syndrome, asthma, cardiovascular condition, and use of anti-arrhythmia drugs). Propensity score matching, logistic regression, Cox proportional hazard regression model, and Kaplan–Meier plots were used to compare the occurrence of arrhythmia between the control and the test groups. RESULTS:  After 1:1 matching, 2,882 patients were identified. Logistic regression showed that SABA users were more likely to have arrhythmia compared to non-users (OR=2.17 (95% CI: 1.56-3.02), p<.0001). For individuals on SABA therapy, the number of supply days of SABA was significantly associated with the occurrence of arrhythmia (p<.001). A Cox proportional hazard model showed that the risk of arrhythmia for SABA users were significantly higher than non-users (HR=2.10 (95% CI: 1.53-2.90), p<.0001). CONCLUSIONS: Our analysis showed that SABA use in pediatrics patients with BPD and CHD may be associated with higher risk of arrhythmia. Phycisians should monitor patients for long-term SABA therapy to avoid potential risk of arrhythmia.