BACKGROUND The objective of this study was to investigate the changes and significance of microRNA-204 (miR-204) in children with congenital heart disease (CHD) complicated with pulmonary hypertension (PH). MATERIAL AND METHODS Fifty-two CHD patients with left-to-right shunt were divided into 3 groups according to preoperative pulmonary artery systolic pressure (PASP) detected by color Doppler echocardiography: a control group (PASP <30 mmHg), a mild PH group (PASP 30-49 mmHg), and a severe PH group (PASP >50 mmHg). Peripheral venous blood and supernatant were collected on an empty stomach at 1 h before surgery and 7 days after surgery. The expression of miR-204 in plasma was detected by RT-qPCR. RESULTS One hour before surgery and 7 days after surgery, plasma miR-204 expression was at a higher level than that in the mild PH group and higher than in the severe PH group. miR-204 expression in children in each group showed a decreasing trend after surgery. The mild PH and severe PH groups had lower plasma miR-204 expression and PASP after surgery than before surgery. In the mild PH and severe PH groups, plasma miR-204 expression was negatively correlated with PASP. In all 52 cases, plasma miR-204 expression was negatively correlated with PASP. CONCLUSIONS The plasma miR-204 expression in CHD children with PH was negatively correlated with the degree of PH, suggesting miR-204 may be involved in PH development, and miR-204 expression may be an indicator of PH severity.