Due to the insidious nature of pediatric cardiac Behçet's disease (BD), misdiagnosis or missed diagnosis occurred frequently. We described a female pediatric patient with BD with cardiac valvular involvement diagnosed at the age of 4 years with clinical symptoms, including aphthous ulcers, fever, perianal ulcers, and erythema nodosum, as well as significantly elevated inflammatory markers. Echocardiography revealed that previously absent aortic valve lesions developed later and gradually worsened. After being diagnosed with BD with cardiovascular involvement, the patient was treated with glucocorticoids, immunosuppressants, biologics, diuretics, and aortic valvuloplasty. At the time of the follow up, the patient was stable. A review of 13 publications was conducted, including 14 cases of cardiac involvement in pediatric BD.
Keywords: Aortic valvular lesion; Behçet's disease; cardiac; case report; pediatric.