Abstract: OBJECTIVE To explore the distribution and drug resistance of pathogens isolated from congenital heart disease patients with ventilator-associated pneumonia so as to provide guidance for reasonable use of antibiotics during the treatment. METHODS A total of 195 congenital heart disease patients with ventilator-associated pneumonia who were treated in the ICU and RICU of cardiac surgery department from Jan 2009 to Dec 2014 were enrolled in the study, then the pathogens that were isolated from the submitted specimens were identified by using the bacterial identification system of Zhuhai DL Biotech Co., Ltd and VITEK-2 Compact automatic microbial identification system of BioMérieux, France, the drug susceptibility testing was carried out by means of disk diffusion method, and the statistical analysis of data was performed with the use of SPSS 18.0 software. RESULTS Totally 212 strains of pathogens were isolated from the submitted specimens that were obtained from the 195 patients, including 153 (72.17%) strains of gram-negative bacteria, 39 (18.40%) strains of gram-positive bacteria, and 20 (9.43%) strains of fungi. The drug resistance rates of the Klebsiella pneumoniae and Pseudomonas aeruginosa to ampicillin, cefuroxime, and cefoxitin were more than 85.00%; the drug resistance rates of the coagulase-negative Staphylococcus to penicillin, cefazolin, amoxicillin, and ampicillin were more than 90.00%. CONCLUSION It is of great significance to investigate the distribution and drug resistance of the pathogens isolated from the congenital heart disease patients with ventilator-associated pneumonia so as to reasonably use antibiotics, shorten the disease course, and promote the rehabilitation.