A very rare case of congenital adrenocortical insufficiency accompanied with ventricular septal defect, is presented. Surgical treatment together with glucocorticoid replacement therapy was performed. The patient, a 4-month-old girl with congenital adrenocortical insufficiency, had been treated with a long-term adrenocortical hormones replacement. Surgical treatment for ventricular septal defect was applied in order to reduce the risk of heart failure. The administration dose of glucocorticoid was determined according to the body surface area and chronologic change of serum cortisol. Following the surgical treatment, and with adequate glucocorticoid replacement, the patient showed a good clinical outcome. In conclusion, we showed a beneficial treatment protocol with adequate glucocorticoid replacement in open heart surgery for a case of congenital adrenocortical insufficiency.

Patients with Behçet disease often develop postoperative valve detachment and pseudoaneurysm as a potentially fatal complication following aortic valve surgery, necessitating re-operation in a few cases. A 37-year-old man underwent 5 aortic valve and aortic root surgeries for the management of valve detachment after initial aortic valve replacement. Evaluation during the course of his disease revealed incomplete Behçet disease. He presented with high fever and Staphylococcus epidermidis bacteremia during the introduction of immunosuppressive therapy with infliximab. Contrast computed tomography revealed a pseudoaneurysm around the aortic root, and an aortic root replacement was performed using an aortic homograft after administration of a 6-week course of vancomycin. The patient is being observed at our outpatient clinic and has demonstrated no complications after 5 years from his last surgery.