In recent years, the number of cases of congenital heart disease combined with infective endocarditis has increased significantly due to the widespread use of antibiotics and invasive tests. The diagnosis is based on a combination of clinical manifestations and ancillary tests. The diagnosis of infective endocarditis is based on certain clinical manifestations such as fever, significantly increased white blood cells, bleeding spots on the skin, and splenomegaly with one of the following: (1) positive blood culture; (2) echocardiographic examination; and (3) intraoperative finding of superfluous organisms. The vast majority of patients had already undergone anti-inflammatory treatment before admission. Treatment principles: Patients had more than 3 blood cultures before surgery, and all intraoperative superfluous organisms were cultured for bacteria. The surgery was performed under moderate hypothermic extracorporeal circulation and cardiac arrest. Myocardial protection was induced by warm blood, with intermittent intraoperative cold blood supplemental perfusion and terminal warm blood resuscitation. The underlying primary cardiac abnormality was first corrected, including subaortic stenosis removal, ventricular septal defect repair, and arterial catheter ligation, and then valve replacement or angioplasty was performed according to the extent of lesion involvement. The procedure is performed with careful removal of redundant and inflammatory destructive tissue, followed by application of 10% iodophor to the affected area, and then application of a sensitive antibiotic solution to soak the prosthetic valve. High doses of sensitive antibiotics are applied for 4 to 8 weeks postoperatively. The current treatment shows that about 50% of patients have aortic valve infection, 25% have mitral valve infection, and 15% have double valve infection; the positive rate of intraoperative flaccid blood culture is only 10%-15%, and the total positive rate of preoperative blood culture and intraoperative flaccid bacterial culture is 20%-25%. The infecting bacteria were mainly streptococci, followed by staphylococci. The overall susceptibility of these organisms to vancomycin + amikacin was 100%. Surgery includes valvuloplasty and valve replacement with very satisfactory treatment results. In recent years, the incidence of infective endocarditis has been increasing year by year. And the proportion of rheumatic heart disease among the underlying causes has gradually decreased, while the proportion of congenital heart disease has increased year by year, which may be related to the informal application of antibiotics, increased bacterial resistance, and the wide availability of invasive tests. Due to the widespread use of antibiotics, the clinical manifestations of patients with infective endocarditis are mostly atypical, and the positive rate of blood culture is not high, which makes early diagnosis and treatment difficult, but a negative blood culture does not exclude the disease, and cardiac ultrasound is important for the diagnosis and treatment of infective endocarditis. Even though the positive rate of blood culture in preoperative examination is less than 20%, and the positive rate of blood culture in intraoperative superfluous organisms is less than 15%, the positive rate of preoperative cardiac ultrasound examination is as high as more than 85%. Therefore, any congenital heart disease with unexplained fever lasting more than 2 weeks with significantly elevated leukocytes should be highly suspected of combined infective endocarditis. Regarding the timing of surgery, it should depend on the status of the changes in cardiac function, and the time of application of antibiotic therapy can only be used as a reference factor. We believe that once the diagnosis of combined infective endocarditis is established, especially in the presence of moderate or higher valvular regurgitation, the decision to operate should be made as early as possible to avoid further deterioration of cardiac function and the occurrence of IE-related complications, without waiting for prolonged anti-inflammatory therapy. Anti-inflammatory therapy is more effective only after the primary disease has been eliminated and the infection site has been completely cleared. In cases where echocardiography shows aortic and/or mitral valve coarctation with severe insufficiency, emergency surgery is considered, even if the infection is not well controlled, to completely remove the infected valve and perform valve replacement surgery.